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Complex regional pain syndrome
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Some periarticular bursae communicate with the joint space. Effusions occur as a result of repetitive use trauma, direct blunt or penetrating trauma, crystal deposition, or infection. The history and examination combined with knowledge of the local anatomy are essential for diagnosis.
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Hypertrophic osteoarthropathy
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This is a periostitis of unknown cause with: (1) clubbing of the fingers, (2) new subperiosteal bone in the long bones, (3) swelling and pains in the joints, and (4) autonomic disturbances of the hands and feet, such as flushing, sweating, and blanching. The earliest sign is finger clubbing (see Clubbing). With progression, bone pain occurs while swelling and pain in the joints may become severe and tenderness is elicited by palpation over the distal forearms and legs. In advanced cases, sweating and flushing of the hands and feet may alternate with the Raynaud phenomenon. The condition may be congenital, with signs appearing in childhood, or acquired. Primary hereditary HOA (Marie-Bamberger syndrome) is an autosomal dominant syndrome, which is expressed much more commonly in males. It has clubbing, greasy thickening of the skin, especially noticeable on the face, and hyperhidrosis of the hands and feet. Acquired HOA is caused by systemic disease. CLINICAL OCCURRENCE: Congenital: Familial, cyanotic congenital heart disease, cystic fibrosis; Endocrine: Graves disease, pregnancy; Idiopathic: Emphysema, cirrhosis; Inflammatory/Immune: Ulcerative colitis, Crohn disease, chronic interstitial pneumonitis, sarcoidosis, dysproteinemia; Infectious: Lung or liver abscess, bronchiectasis, tuberculosis, bacterial endocarditis, dysentery; Metabolic/Toxic: Malabsorption, chronic hypoxemia; Neoplastic: Lung, pleural, and gastrointestinal cancer, metastatic disease to lung; Vascular: Aortic aneurysm.
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Necrotizing soft-tissue infection. Rapid expansion of infection in subcutaneous tissue planes produces local vascular thrombosis leading to ischemia of fat, connective tissue, skin and underlying muscle. Infection and necrosis spread proximally and distally along tissue planes and invade deeper structures along neurovascular bundles that penetrate these planes. When the muscle fascia is involved the term necrotizing fasciitis is applied. Pain and fever accompanied by signs of inflammation (erythema, warmth, tenderness) result from infection with group A streptococci or polymicrobial (mixed aerobic/anaerobic) soft-tissue infections. Systemic hypotension, tachycardia, and delirium develop rapidly, and outcome is fatal without complete surgical debridement. Predisposing factors include diabetes mellitus, immunosuppression, and minor puncture wounds. Urgent surgical debridement is mandatory to save limb and life [Hoadley DJ, Mark EJ. Weekly clincopathological exercises: Case 28–2002: a 35-year-old long-term traveler to the Caribbean with a rapidly progressive soft-tissue infection. N Engl J Med. 2002;347:831–837].
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Acromegaly and gigantism
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The hands enlarge from overgrowth of bone and soft tissues, stimulated by an excess of growth hormone, usually from an adenoma of the anterior pituitary gland. When the condition occurs before the epiphyses close, the enlarged skeleton is well proportioned and the condition is termed gigantism. When growth occurs after epiphyseal closure, the skeletal pattern is called acromegaly, in which the hands, feet, face, head, and soft tissues are thickened. Patients often have diffuse muscle or joint stiffness and may complain of headaches and back pain. Examination shows thickening of the soft tissues, particularly apparent in the face, hands, and feet. Widening without lengthening of the bones leads to prominence of the jaw, wide spacing of the teeth, prominent supraorbital ridge, and enlarged hands and feet. OA is common. In advanced disease with suprasellar extension of the tumor, signs of hypopituitarism and bitemporal hemianopsia may be present.
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This is a congenital disease resulting from mutations of the fibrillin-1 gene. It is frequently inherited as an autosomal dominant, but sporadic cases occur. It affects the development of bone, ligaments, tendons, arterial walls, and supporting structures in the heart and eyes. Although the complete syndrome is striking, many persons show only a few stigmas. The long, slender phalanges, spider fingers have given the name arachnodactyly to the disease (Plate 29); some patients lack this sign. The skull is long and narrow, and the palate is high and arched. The long bones are thin and elongated, so the finger-to-finger span with the outspread arms exceeds the body height. Thoracic deformities may be either pectus excavatum (funnel breast) or pectus carinatum (pigeon breast). The spine may exhibit fused vertebrae or spina bifida. Joint laxity permits hyperextension (double-jointedness), dislocations, kyphoscoliosis, pes planus, or pes cavus. The ears may be long and pointed, satyr ear. Weakness of the supporting structures of the eye produces elongation of the globe (myopia), retinal detachment, lens dislocation, and blue sclerae. Degeneration of the vascular elastic media leads to aneurysmal dilatation of the aorta or pulmonary artery. Aortic dissection and rupture are common causes of early death. Deformities of the cardiac valve cusps are sites for bacterial endocarditis. The foramen ovale may remain patent.
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Syndromes Primarily Affecting Joints
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Arthritis—inflammatory and noninflammatory joint disease
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The diagnosis of arthritis requires the examiner to confirm signs of acute or chronic joint inflammation: redness, warmth, tenderness, synovial thickening, effusion, bony enlargement, or erosive changes on X-ray. Careful attention during the physical examination is necessary to distinguish involvement of the joint (synovium and articular cartilage: arthritis) from inflammation of the periarticular tendon and ligament insertions into bone (the enthesis: enthesitis), the tendons and their sheaths (tendonitis and tenosynovitis) or the underlying bone (osteitis). When tendon inflammation is suspected, listen with the stethoscope for a rub over the painful site.
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The most diagnostically useful descriptive classification of joint diseases is based upon the history and physical findings. First, the number of joints actively involved is assessed as monarticular (1 joint), oligoarticular (2–4 joints), or polyarticular (>4 joints). For patients with oligo- or polyarthritis, note the pattern of joint involvement: (1) large more proximal joints or distal small joints, (2) axial joints and/or peripheral joints, and (3) whether involvement is symmetric or asymmetric. Widespread and symmetrical joint involvement increases the likelihood of a systemic inflammatory disease primarily involving the joints (e.g., RA and SLE).
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A second group of arthritis syndromes are classified as spondyloarthritis. These disorders have in common prominent enthesiopathy, involvement of the SI joints and spine, variable large joint disease (oligoarthritis), and associations with inflammatory and infectious diseases in other organs (gastrointestinal tract, genitourinary tract, skin, and eye).
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No classification system is perfect and the examiner must always be alert to evolution of the pattern over days, weeks, or years. Remember that polyarthritic diseases may initially present with single joint involvement. Correct rheumatologic diagnosis requires patience and an open mind.
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Monarticular arthritis
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Arthritis involving a single joint is likely caused by local mechanical, inflammatory, or infectious factors
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Less commonly, it is the initial manifestation of a systemic process that will involve other joints. Sequential involvement of single joints with intervening remissions suggests an underlying systemic disorder (congenital or acquired) with superimposed local precipitating events, for example, trauma in hemophilia. CLINICAL OCCURRENCE: Congenital: Hemophilia; Endocrine: Hyperparathyroidism, hypothyroidism; Idiopathic: OA; Inflammatory/Immune: Postinfectious reactive arthritis, psoriasis, RA (initial presentation), SLE, amyloidosis; Infectious: Acute septic arthritis (Staphylococcus aureus, gonococcemia, others), Lyme disease, syphilis, mycobacteria, osteomyelitis, viral (e.g., HIV, parvovirus, others); Metabolic/Toxic: Crystal-induced diseases (e.g., gout, calcium pyrophosphate deposition, calcium hydroxyapatite, calcium oxalate), scurvy; Mechanical/Traumatic: Blunt trauma, hemarthrosis, fracture, repetitive use/overuse; Neoplastic: Sarcoma (bone, synovium, or cartilage), metastases to bone, benign tumors (e.g., osteochondroma, osteoid osteoma, pigmented villonodular synovitis), leukemia; Neurologic: Neuropathy producing a Charcot joint; Vascular: Osteonecrosis.
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Direct extension of bacterial infection (most commonly with S. aureus) from skin, soft tissue, or periarticular bone or hematogenous spread (e.g., bacterial endocarditis, gonorrhea bacteremia) produces infection within the joint. Release of lysosomal enzymes can rapidly destroy the joint. Usually one joint is involved, often the knee, but in gonococcal arthritis, three-fourths of the patients have an initial transient (2–4 days) migratory oligoarthritis and/or tenosynovitis. Symptoms often begin suddenly with chills and fever. The joint swells rapidly, the overlying skin is red and warm, and the joint is painful and tender to touch. The swelling becomes fluctuant, indicating fluid in the synovial cavity. In immunosuppressed patients signs of infection are frequently absent. Joint aspiration discloses purulent fluid that must be cultured to identify the causative organism [Margaretten ME, Kohlwes J, Moore D, Bent S. Does this adult patient have septic arthritis? JAMA. 2007;297:1478–1488]. CLINICAL OCCURRENCE: In addition to S. aureus and gonorrhea, less-common organisms to consider are streptococci, meningococci, Haemophilus influenzae (especially in unimmunized infants and children), and, rarely, brucellosis, typhoid fever, glanders, blastomycosis, granuloma inguinale, tuberculosis, fungi, and others.
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Uric acid accumulates in tissue and extracellular fluid as a result of genetic (primary gout) or acquired (secondary gout) causes of uric acid overproduction or decreased excretion. When the fluid becomes supersaturated, crystals form in the tissue. When crystals are shed into the joint fluid and phagocytosed by polymorphonuclear neutrophils, acute inflammation results [Choi HK, Mount DB, Reginato AM. Pathogenesis of gout. Ann Intern Med. 2005;143:499–516]. The initial attack presents the chief diagnostic problem because a history of recurrent stereotypic episodes is very suggestive of gout. Frequently, the patient is awakened from sleep by severe burning pain, tingling, numbness, or warmth in a joint. The joint rapidly swells and becomes excruciatingly tender, intolerant to the pressure of the bedclothes. Typically, the overlying skin becomes red or violaceous. There may be malaise, headache, fever, and tachycardia. Untreated, the attack lasts for 1 to 2 weeks. In more than half the cases, the MTP joint of the great toe is affected initially (podagra) (Plate 30). Other sites are the midfoot (instep), ankle, knee, elbow, or wrist. Acute gout in the midfoot resembles cellulitis. The attacks may be triggered by trauma, surgery, acidosis, infection, exposure to cold, changes in atmospheric pressure, overindulgence in alcoholic beverages, or any acute illness. Large tissue deposits of uric acid (tophi) occurring around joints over bony prominences are not usually inflamed.
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Calcium pyrophosphate dihydrate deposition disease (CPPDD)–pseudogout, chondrocalcinosis
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Calcium pyrophosphate dihydrate, shed from articular cartilage, crystalizes within the joint into small, rhomboidal, weakly positive birefringent crystals that trigger inflammation. Articular fibrocartilage may calcify and be detected by X-ray (chondrocalcinosis) in the knee, pelvis (hip joints and symphysis pubis) or wrist. This disorder is quite similar to true gout in both its acute and chronic forms. The attack begins abruptly with painful swelling and heat, usually in a single joint, occasionally in two or more joints. The knee, ankle, and wrist are most commonly affected. Untreated, the pain and tenderness are intense for 2 to 4 days, then gradually subside during the next 1 to 2 weeks. It is associated with increasing age and OA. It may be a clue to hyperparathyroidism or hemochromatosis (approximately 5% of CPPDD).
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Absence of pain or proprioceptive sensation in a joint leads to loss of joint integrity. Repeated injuries cause successively three stages of articular damage: swelling, joint degeneration, and formation of new bone. In the initial stage, erythema and swelling are the only findings. The course is progressive with hypermobility, traumatic osteophyte formation, and subluxation leading to painless deformities and crepitus on movement. In any stage, the diagnostic clue is the absence of the expected pain with movement and loss of pain sensation and proprioception in the involved limb. A single joint may be affected, or, commonly, all the joints in an anatomic region (e.g., the midfoot joints) are involved. Physical examination should try to determine if the neuropathy is part of a local or systemic neuropathic process. Classic clinical conditions, causing Charcot joints, include tabes dorsalis (knee most commonly involved; hip, ankle, lower spine, less frequently involved) (Fig. 13-21C), diabetes mellitus (tarsal and metatarsal joints most commonly, ankle occasionally, knee rarely), syringomyelia (usually joints of the upper limbs), and leprosy [Hartemann-Heurtier A, Van GH, Grimaldi A. The Charcot foot. Lancet. 2002;360:1776–1779].
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Mechanical trauma as a result of falls, sports, or other activities causes bleeding (hemarthrosis) or effusion with pain as a consequence of damage to the joint capsule, intra- or periarticular ligaments, cartilage, or bone. The knee and ankle are commonly affected, but any joint can be injured. The exact mechanism of injury, time course of pain (immediate versus delayed), and physical examination will assist in suggesting the type and degree of injury. Guidelines for the proper use of radiologic examination for injuries to the ankle and knee have been developed.
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There is chronic swelling of a single joint with only moderate pain. Joint effusion may be present with thickening of the synovium. Most commonly affected are the hips, knees, and spine. Previous intraarticular or oral corticosteroids and immunosuppressive drugs are major risk factors. Cultures of aspirated joint fluid or synovial biopsy yield Mycobacterium tuberculosis.
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Recurrent painless knee effusion
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The patient experiences episodes of painless swelling and joint effusion in one or both knees, with no constitutional symptoms over a number of years, with an average duration of 3 to 5 days. The cause is unknown, though, occasionally, the condition presages the onset of RA.
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Oligoarthritis and polyarthritis
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Arthritis involving several joints, either simultaneously or sequentially, is termed oligoarthritis (2–4 joints) and polyarthritis (>4 joints).
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The pattern of involvement can be useful in helping to identify the exact diagnosis. Generally, oligoarthritis involves larger joints and is asymmetric whereas the classic polyarthritis syndromes (RA, SLE) involve symmetric joints, including the small joints of the hands and feet. These are only guidelines and must not be interpreted as hard-and-fast rules. CLINICAL OCCURRENCE: Congenital: Hemophilia, familial Mediterranean fever, hemochromatosis, sickle cell disease, alkaptonuria/ochronosis; Endocrine: Hyperparathyroidism, hypothyroidism, acromegaly; Idiopathic: Inflammatory and noninflammatory OA; Inflammatory/Immune: Reactive arthritis, psoriasis, RA, systemic erythematosus, RF, ankylosing spondylitis, systemic sclerosis (scleroderma), polymyositis/dermatomyositis, antisynthetase syndrome, Still disease, Behçet syndrome, relapsing polychondritis, amyloidosis, sarcoidosis, erythema multiforme, erythema nodosum, drug reactions, serum sickness; Infectious: Septic arthritis, Lyme disease, viral (e.g., HIV, parvovirus, rubella, mumps, others), Whipple disease, mycoses (coccidioidomycosis, histoplasmosis, blastomycosis, cryptococcosis), actinomycosis, secondary syphilis, brucellosis, typhoid fever; Metabolic/Toxic: Crystal-induced diseases (e.g., gout, calcium pyrophosphate deposition, calcium hydroxyapatite, calcium oxalate), ochronosis, scurvy; Neoplastic: Sarcoma (bone, synovium, or cartilage), metastases to bone, benign tumors (e.g., osteochondroma, osteoid osteoma, pigmented villonodular synovitis); Vascular: Osteonecrosis, systemic vasculitis, HOA.
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RA—deforming symmetrical distal polyarthritis
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RA is characterized by proliferation of inflamed synovial tissue (pannus) that enters the joint cavity in tongue-like projections. The pannus erodes cartilage, periarticular bone, and soft tissues, including tendons and ligaments. Untreated, the result is destruction of the joint surfaces and supporting structures producing subluxation, deformity, and loss of joint function. The onset may be insidious with morning stiffness and pain followed by swelling and tenderness of the joints, proceeding over weeks to months into a small and large joint polyarthritis. Less commonly, the onset is sudden with pain and swelling occurring simultaneously in several joints accompanied by fever and prostration. Smaller joints of the hands (MCP, PIP), feet (MTP), wrists, and ankles are typically involved early and symmetrically; onset in a single larger joint, usually a knee, is not rare. Interphalangeal joints become fusiform from joint effusion (fluctuant) or thickening of the joint capsule (nonfluctuant). DIP joints are invariably spared. Tenderness is confined to the region of the capsule. Joint motion is initially limited by pain or effusion, later by fibrosis or muscle shortening. Muscle weakness and wasting may be rapid and disproportionate to the degree of disuse. Although remissions may occur, the disease is usually progressive over a period of years. Joint contracture and subluxations are frequent, with subluxation of the MCP joints producing characteristic ulnar deviation of the fingers. Tenosynovitis is manifest as swelling of the tendon sheaths. In 20% to 35% of cases, subcutaneous rheumatoid nodules develop over bony prominences and tendon sheaths. They are painless, firm, and freely movable over bones, similar to those in RF. A serious late complication is instability of the cervical spine from subluxation of C1 on C2 that may present as neck pain or upper motor neuron signs. Diagnosis is based upon clinical and laboratory criteria. Frequently, the patient must be observed for many months before the diagnosis is secure. DDX: RA frequently involves the temporomandibular joint unlike RF. In RF, arthritis is migratory, whereas it is persistent in RA. Reactive arthritis is usually oligoarticular and mainly affects large joints, especially ankles and knees. An initial monarthritis may suggest an infectious or crystalline arthritis; however, aspiration of joint fluid excludes these possibilities.
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Felty syndrome is the triad of RA, splenomegaly, and leukopenia. Palindromic rheumatism presents as multiple afebrile attacks of mono or oligoarthritis lasting for only 2 or 3 days, leaving no residua. Secondary Sjögren syndrome is diagnosed when keratoconjunctivitis sicca and xerostomia accompany RA. Vasculitis may accompany RA and involve the skin (necrosis and nodules), nervous system, and lung. Juvenile idiopathic arthritis is a group of disorders which may overlap with the adult disease [Hashkes PJ, Laxer RM. Medical treatment of juvenile idiopathic arthritis. JAMA. 2005;294:1671–1684]. See pediatric texts for descriptions and details.
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SLE—nondeforming symmetrical distal polyarthritis
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The cause is unknown, but the disease involves inflammation of multiple tissues and organs accompanied by antibodies to specific nuclear antigens usually degraded in the nucleosome. These autoantibodies are the hallmark of SLE, but their role in its pathogenesis is unclear. SLE is a chronic inflammatory multisystem disease, more common in women than men. No one symptom or sign is pathognomonic of SLE; rather, the diagnosis is established by clinical criteria. The most common symptoms and signs are fatigue, malaise, or fever (90% of cases), arthritis or arthralgias (90%), and skin rashes (50–60%). The arthralgias, myalgias, and joint inflammation resemble mild RA, but deformities usually do not develop. The malar (“butterfly”) rash is a macular to maculopapular, sometimes scaly, erythematous process forming the “wings” of the butterfly on each malar prominence, with the “trunk” on the bridge of the nose. It may be more intense after exposure to sunlight. In addition, there may be skin atrophy, telangiectasia, and mucosal ulcers. Serositis is common, presenting as pleurisy (with effusion and/or pleural rubs), abdominal pain, or pericarditis. Nonbacterial endocarditis with valvular insufficiency (usually mitral), central nervous system disease (with personality change, psychosis, or seizures), and glomerulonephritis can occur at any time and may be the presenting syndrome. Other signs are recurrent urticaria, mononeuritis multiplex and lymphadenopathy. Fetal wastage and thromboembolic disease are associated with antiphospholipid antibodies [D’Cruz DP, Khamashta MA, Hughes GRV. Systemic lupus erythematosus. Lancet. 2007;369:587–596; Caro I, Zembowicz A. Case 5–2003: a 16-year-old girl with a rash and chest pain. N Engl J Med. 2003;348:630–637].
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Rheumatic Fever—migratory polyarthritis
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This disease is a delayed inflammatory reaction following infection with specific Lancefield groups of group A beta-hemolytic streptococci. Tissues affected include the heart, joints, skin, and central nervous system. The disease is protean in its clinical manifestations. The classic presentation begins from 1 to 4 weeks after streptococcal pharyngitis, with gradual onset of malaise, fatigue, anorexia, and fever. A single large joint becomes painful, tender, and swollen, and the overlying skin is red and hot. An effusion forms that is turbid and sterile. Although the fever and other signs of illness persist, the joint inflammation spontaneously subsides in a few days, only to reappear in another joint, and later in another joint—a migratory polyarthritis. Joint involvement may be so mild that pain is unaccompanied by physical signs of inflammation. Months of observation may be required to distinguish it from RA; involvement of the temporomandibular joint often occurs in RA, practically never in RF. RF leaves no residual joint deformity. At onset, the inflammation of a single joint with effusion requires distinction from suppurative arthritis. Carditis is manifest as tachycardia, muffled heart sounds, heart enlargement, valvular insufficiency murmurs, pericardial friction rub, or gallop rhythm on examination. The electrocardiogram may show PR prolongation. Two distinct types of skin lesions may be associated with RF, although neither is pathognomonic. Erythema marginatum or circinatum is characterized by coalescing circular erythematous areas over the trunk and extremities, migratory and transitory, changing within the hour. With chronic disease, subcutaneous rheumatic nodules may appear as firm, nontender masses over the joint prominences and tendon sheaths of the limbs, scalp, and spine. They are loosely attached to the underlying tissue; when numerous, their distribution tends to be symmetrical. Sydenham chorea may appear several months after onset. There are no diagnostic laboratory tests, but the full clinical picture is fairly distinctive. Diagnosis is based upon the Jones criteria of major and minor manifestations.
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See Gout. Tophi are masses of monosodium urate crystals deposited in the tissues often over bony prominences and around joints where they erode bone. Acting as foreign bodies, they stimulate low-grade inflammation that may extrude the tophi through the skin. The asymmetrical nodular swellings and cartilage degeneration may impair joint function. Clinical signs of inflammation are variable, from mild to moderately severe. The olecranon, bunion and prepatellar bursae, and hands are most frequently affected.
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OA, degenerative joint disease (DJD)—noninflammatory polyarthritis of large and small joints
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This is a disease of articular cartilage with degradation of the proteoglycan matrix leading to fissuring, thinning, and loss of articular cartilage, and secondary thickening of subchondral bone. In late stages, the bone ends rub directly on each other so their surfaces become worn and polished. The joint capsules are little affected, so adhesions are not formed, and although joint motion is restricted, ankylosis does not result. The bony margins proliferate to form spurs, lipping, and exostoses. Genetic and acquired factors (trauma, surgery, obesity, excessive use) contribute to the pathogenesis. Symptoms are usually first noticed in the weight-bearing joints after age 40 and signs of inflammation are relatively slight. Symptoms correlate poorly with the objective extent of joint disease. The most common symptom is pain with use that disappears with rest. The patient may note grating during motion. Initially, the range of motion is normal, with a gradual decrease occurring as the disease progresses. Enlargement of the DIP and PIP joints of the fingers (Heberden and Bouchard nodes, respectively), is frequently encountered. Painless knee effusion is frequent as is asymmetric loss of knee cartilage leading to valgus or varus deformity.
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Gonococcal arthritis—migratory oligoarthritis
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Neisseria gonorrhoeae infects the urethra or uterine cervix and then disseminates hematogenously to synovial membranes and skin, causing local inflammation. Infectious arthritis, tenosynovitis, and skin lesions are the most common extragenital complications of gonorrhea. One to four weeks after the onset of urethritis, inflammation may suddenly occur in the knees, wrists, and ankles, although other joints may be affected. The most common pattern is a migratory oligoarthritis and tenosynovitis. Small amounts of thin fluid may accumulate in joint cavities. In other cases, suppurative arthritis develops, with inflammation of a single joint and production of purulent exudate. Tenosynovitis in the hands, wrists, or ankle is more common in gonorrhea than in arthritis from any other cause. The diagnosis may not be easy because the gonococcus is difficult to culture from the joints. Pustular skin lesions on an erythematous base are seen with gonococcal bacteremia and help to distinguish gonococcal arthritis from other conditions. Genital, rectal, and throat specimens for culture or polymerase chain reaction testing of urine for gonococcus and chlamydia should be obtained. Reiter syndrome (nonspecific urethritis, arthritis, and conjunctivitis) following nongonococcal urethritis may cause confusion, because conjunctival infection is present in up to 10% of patients with gonorrhea.
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The second and third MCP joints and the radiocarpal joint of the wrist are most commonly affected. Chondrocalcinosis is present. Symmetric noninflammatory arthritis of the MCP joints should initiate a search for an iron-storage disorder.
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Relapsing symmetrical seronegative synovitis with pitting edema
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This condition of unknown cause presents with recurrent episodes of symmetrical synovitis of the hands and wrists with pitting edema and erythema of the dorsum of the hands. Pain is relatively mild and the condition usually remits after several days to a couple of weeks. When RS3PE occurs as a paraneoplastic condition, it may be more chronic.
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Inflammation at the insertion of ligaments and tendons into bone (the enthesis, enthesitis) leads to joint and tendon sheath effusions and ossification of periarticular structures.
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Genes (HLA-B27) and acquired illness (inflammatory bowel disease, infectious colitis, nongonococcal urethritis, and psoriasis) predispose to these disorders. Asymmetric oligoarthritides of the large joints with prominent involvement of the spine and SI joints and negative tests for rheumatoid factor are described as the seronegative spondyloarthritides. Patients present with back pain and stiffness, and occasionally with fever, malaise, and weight loss. Look for extraarticular disease, such as genitourinary or gut symptoms, eye involvement (uveitis), and skin disease. [Khan MA. Update on spondyloarthropathies. Ann Intern Med. 2002;136:896–907]. CLINICAL OCCURRENCE: Ochronosis, disk disease, DISH, ankylosing spondylitis, enteropathic arthritis (inflammatory bowel disease), psoriasis, reactive arthritis after diarrheal and genitourinary infections.
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Ankylosing spondylitis
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Inflammation of the ligamentous attachments to the vertebrae (enthesitis), the SI joints and the junction of the annulus fibrosis and the vertebral end plates leads to new bone formation and bridging resulting in ankylosis. This chronic, progressive arthritis begins with SI involvement and progresses proximally often leading to severe ankylosis. Nonspecific symptoms often begin in adolescence and occur intermittently for 5 or 10 years. Pain and morning stiffness are felt in the lumbar region, buttocks, and SI region. Fatigue, fever, and weight loss may occur. Decreased lumbar spinal motion is an early sign with the normal lumbar lordosis straightened, diminished anterior flexion, and spinal rotation and lateral bending impaired. Slowly, the process ascends the lumbar and thoracic spine, the cervical region may be involved late. Episodes of acute or subacute arthritis may involve hip, shoulder, sternoclavicular, or manubriosternal joints. Tenderness is found over the involved joints. Iridocyclitis occurs in one-fifth of the cases. Aortic regurgitation is a late complication in 3% of patients. Idiopathic ankylosing spondylitis needs to be distinguished from psoriatic arthritis and enteropathic associated spondyloarthritis (Crohn disease and ulcerative colitis), Whipple disease, and DISH.
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Following infection of the urethra (chlamydia) or gut (Shigella, Salmonella, Yersinia, and Campylobacter) an oligoarthritis, predominately of the lower extremities, develops. It may be accompanied by enthesitis of the hands, ankles, and feet, conjunctivitis, urethritis, and rash on the glans penis (circinate balanitis) and feet (keratoderma blenorrhagica). Sacroiliitis and spine involvement may occur. Eighty-five percent of patients are HLA-B27-positive.
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Enteropathic arthritis
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Inflammatory asymmetric arthritis predominately of the ankles and knees may occur in association with inflammatory bowel disease (ulcerative colitis and Crohn disease). Enthesitis is common, especially at the Achilles tendon insertion, and symmetrical SI and spinal involvement occurs. The arthritis may precede clinical manifestations of the inflammatory bowel disease.
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The joint disease is usually an asymmetrical oligo- or polyarthritis of small and large joints. It is occasionally a symmetrical polyarthritis resembling RA. Destructive arthritis of the DIP joints is seen in psoriatic arthritis but not in RA. Spondylitis and sacroiliitis occur in up to 25% of patients with psoriatic arthritis, especially those with HLA-B27. Enthesitis may predominate in some patients. The arthritis may precede, accompany, or follow onset of the skin rash. Physical examination should include a careful skin examination, especially of the scalp (the rash may be hidden by hair or dismissed as seborrhea) and fingernails (looking for pits and onycholysis).
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Conditions Primarily Affecting Bone
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Bone resorption exceeds bone formation, leading to decreased bone mass and decreased mechanical strength. Trabecular bone is affected more than cortical bone. Increased resorption of bone results from immobilization, inflammation, multiple myeloma, and hyperparathyroidism. Decreased bone formation results from gonadal hormone deficiency, glucocorticoid steroid excess, and advanced age. Trabecular bone deficiency is especially important in the vertebrae and pelvic bones, whereas cortical bone loss is predominant in long bones. Osteoporosis is asymptomatic until insufficiency fractures occur, usually in the thoracic or lumbar spine and pelvis. Thoracic kyphosis results from anterior wedging of thoracic vertebrae. Bone mineral density is assessed by dual X-ray absorptiometry (DEXA) scan. The bones are much more susceptible to fracture with minor trauma; hip and wrist fractures from falls should initiate evaluation for osteoporosis in both men and women [Green AD, Colón-Emeric CS, Bastian L, Drake MT, Lyles KW. Does this woman have osteoporosis? JAMA. 2004;292:2890–2900]. CLINICAL OCCURRENCE: Congenital: Vitamin D-resistant rickets, Marfan syndrome, hemochromatosis, Ehlers–Danlos syndrome, hemophilia, thalassemia, positive family history; Endocrine: Postmenopausal estrogen deficiency, premature menopause, hypogonadism, hyperthyroidism, hyperparathyroidism, Cushing syndrome, glucocorticoid use, diabetes mellitus, pregnancy, adrenal insufficiency, acromegaly, hyperprolactinemia; Idiopathic: Advanced age; Inflammatory/Immune: Sarcoidosis, amyloidosis, RA, ankylosing spondylitis; Metabolic/Toxic: Vitamin D deficiency, malnutrition, chronic renal insufficiency, heparin, cirrhosis, postgastrectomy, parenteral nutrition, cigarette smoking, low body weight; Mechanical/Traumatic: Immobilization, disuse, and nonweightbearing; Neoplastic: Multiple myeloma, paraneoplastic (parathyroid hormone (PTH)-related protein secretion), lymphoma, prolactinoma; Neurologic: Paralysis, stroke, multiple sclerosis; Psychosocial: Anorexia nervosa; Vascular: Hyperemia of bone.
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Vitamin D deficiency, hypocalcemia, or hypophosphatemia after the epiphyses are closed prevents calcification of newly formed bony matrix. Early there are no symptoms or signs, whereas later, bone pain and tenderness occur. Low back pain and striking muscle weakness are common. Low serum calcium levels may produce spontaneous carpopedal spasm with the Chvostek and Trousseau signs. Insufficiency (stress) fractures are common and pseudofractures may be seen by X-ray. CLINICAL OCCURRENCE: Congenital: Vitamin D-resistant rickets; Endocrine: Hyperparathyroidism, rapid deposition of calcium and phosphorus in the tissues after ablation of the parathyroid glands in osteitis fibrosa cystica, hypoparathyroidism; Inflammatory/Immune: Celiac disease; Metabolic/Toxic: Vitamin D deficiency, hypocalcemia, hypophosphatemia, malabsorption, pancreatic insufficiency, malnutrition, chronic renal insufficiency, renal tubular acidosis, Fanconi syndrome, ureterosigmoidostomy, essential hypercalciuria, drugs (anticonvulsants, e.g., phenytoin, glucocorticoids, etidronate), fluoride and aluminum intoxication.
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Vitamin D deficiency in childhood before epiphyseal closure results in inadequate calcification of cartilage and new bone. See Chapter 8, Figure 8-30. Softening of bone produces widening of the cranial sutures and fontanelles (craniotabes), Parrot bosses, rachitic rosary, Harrison grooves, thoracic kyphosis or lordosis, genu valgum or varum, and a contracted pelvis. With the sole exception of the rosary, all deformities are permanent stigmas of childhood disease.
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Increased resorption of bone combines with rapid growth of new bone with disordered architecture and decreased mechanical strength. The cause is unknown. Bone pain may occur but is seldom severe. With the exception of the hands and feet, any bones may be involved. The skin over affected bones may be warm. The classic osseous deformities are increased girth of the calvarium, thoracic kyphosis, genu varum, and shortening of the spine by flattening of the vertebrae, giving the appearance of disproportionately long arms (Fig. 13-37). Spontaneous or stress fractures can occur and rarely osteogenic sarcoma develops. Increased blood flow through the spongy bone may produce an arteriovenous fistula leading to high-output cardiac failure.
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Primary hyperparathyroidism—osteitis fibrosa cystica
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Increased production of PTH causes bone resorption to exceed new bone formation, leading to hypercalcemia, hypercalciuria, hyperphosphaturia, and hypophosphatemia. The disease may be asymptomatic, although diffuse musculoskeletal aching and fatigue is seen in many patients. Late findings are bone tenderness, muscle weakness, and waddling gait. Subperiosteal cysts in the skull and long bones can cause visible and palpable swellings. Peptic ulcer and urolithiasis should prompt a search for hyperparathyroidism.
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Hyperparathyroidism, secondary, and tertiary
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Hyperphosphatemia and hypocalcemia combined with disordered vitamin D metabolism in chronic renal failure (renal osteodystrophy), or osteomalacia from other causes, leads to increased production of PTH, hyperplasia of the parathyroid glands, and progressive bone disease. Combinations of dietary, hormonal, and renal replacement therapies can help control the disorder. When PTH production is not suppressible with appropriate treatment, it is termed tertiary hyperparathyroidism.
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This malignant clonal proliferation of immunoglobulin-producing plasma cells (terminally differentiated B cells) in the bone marrow causes destruction of bone by activation of osteoclasts leading to hypercalcemia. Normal immunoglobulin production is suppressed, there is production of monoclonal plasma proteins (heavy chains, light chains, and intact immunoglobulin), and anemia and thrombocytopenia develop. Renal insufficiency results from tubular toxicity of light chains (Bence Jones proteins), hyperuricemia, and hypercalcemia. This is the most common malignant tumor primarily affecting bone. Fatigue or generalized aching discomfort may be the only early symptoms. The most common specific symptom is bone pain. With spinal compression fractures, localized pain in the back with radicular distribution occurs. The presenting complaints may be referable to anemia, renal failure, or a pathologic fracture. Multiple myeloma should be considered in patients more than 45 years of age with back pain and anemia.
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Metastatic disease presents as a localized swelling or pain in a bone, a pathologic fracture, or is found incidentally on radiographs taken for another reason. The most frequent primary carcinomas are breast, lung, and prostate.
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Mechanical disruptions of the mineralized bone and its collagenous matrix are caused by application of forces exceeding the tensile and/or compressive strength of the bone. This can occur from a single forceful event or from repetitive less forceful loading. Fracture of the mineralized bone leaving the collagenous matrix intact results from trauma in children (greenstick fracture) and repetitive activities in adults (stress fracture). If the bone is inherently weak because of local destructive disease, it may fracture at usual loads (pathologic fracture). When fracture fragments penetrate the skin, it is an open fracture. When fracture is suspected, immobilize the part and assess neurovascular function distal to the injury. Inspection often reveals deformity. Unavoidable movement reveals abnormal mobility, and bone crepitus, distinctive signs of fracture one should not deliberately try to elicit. Muscle contraction attempting to splint the fracture often aggravates pain. Localized bone tenderness indicates the site of the fracture. Shortening of a long bone may be the crucial sign of an impacted fracture.
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Spontaneous or pathologic fracture
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This is a fracture that occurs with trauma insufficient to break a normal bone. Judging the amount of trauma is difficult, hence spontaneous fractures are easily mistaken for traumatic fractures; a high index of suspicion is warranted. X-ray signs of generalized or local bone disease should be sought if pathologic fracture is suspected. Sometimes spontaneous fractures are less painful than those of healthy bone. Osteomalacia, osteoporosis, Paget disease, hyperparathyroidism, multiple myeloma, osteogenesis imperfecta, and primary and metastatic neoplasms in bone are common underlying conditions.
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Blood borne bacteria from superficial infections are carried to the terminal capillary loops of the metaphyseal cortex, causing a necrosing infection that erodes to the periosteum. The initial infection is in the metaphysis, near but not involving the epiphysis. S. aureus is the most common organism. Osteomyelitis is most common in children. The onset is usually sudden, with fever and pain. Older children may be able to point to the painful site, although the pain may be referred to the nearest joint where sympathetic effusion may be noted. Localized swelling and redness of the overlying skin with increased warmth may be seen. Light bone percussion frequently discloses tenderness; localize the site with finger pressure on the bone moving toward the suspected site until the point of maximum tenderness is located. The proximal femoral metaphysis is within the hip joint in children, so they present with a septic hip. Sometimes deep cellulitis cannot be distinguished clinically from osteomyelitis.
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Osteomyelitis, chronic
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After the acute phase, the purulent discharge from the necrosing bone breaks through the periosteum and drains through sinuses in the skin. The circulation of the cortex becomes impaired, producing islands of dead bone, sequestra. A sequestrum may be absorbed, discharged through the sinus, or surrounded by new bone, the involucrum. Continuing necrosis of bone and retention of sequestra is the cause of persistent infection.
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Osteogenesis imperfecta
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An inherited disorder of type I collagen results in decreased mechanical strength of all bones and pathologic fractures during the first decade of life. Autosomal dominant inheritance occurs in 60% of cases, but several different types have been recognized. The bones are harder and more brittle than normal, so spontaneous or pathologic fractures are common. The fractures are sometimes painless. Blue sclerae may be observed. Short stature is usual and skull deformity is often present. Hypermobility of joints is common.
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Fibrous dysplasia of bone
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The cause of this disease is unknown. The architecture of one or more bones is distorted by fibrosis; the cranium and long bones are especially involved. There is asymptomatic bowing of the affected long bones. The skin often contains melanotic spots with jagged borders. In young girls, precocious puberty may occur.
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Hereditary multiple exostoses—osteochondromatosis
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The autosomal dominant disease is characterized by exostoses arising from the bony cortex, deforming the metaphyseal region of some long bones. Involvement is usually bilateral but not symmetrical. The ulna may be shortened, producing ulnar deviation of the hand. Valgus deformities of the ankle are common. The only symptom may be mechanical interference with normal joint function.
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Neck, Spine, and Pelvis Syndromes
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Neck, shoulder, and arm pain—cervical spondylosis and radiculopathy
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Spondylosis results from degeneration of the intervertebral disks. Contusion of a cervical root in the neural foramen produces severe pain in the nerve distribution. Commonly, the cause is narrowing of intervertebral foramina or compression of nerve roots by osteophytes. Uncommonly, the lesion is caused by protrusion of an intervertebral disk or by C1–C2 instability from RA. Usually, minor trauma precedes the onset of pain. Painful spasm of the neck muscles causes temporary torticollis, with the head tilted away from the painful side. Sharp, shooting pains spread slowly down the shoulder, the lateral aspect of the upper arm, and the radial aspect of the forearm, to the wrist. The neck muscles are rigid on the affected side. The biceps tendon reflex is frequently diminished or absent with a radiculopathy of C5 or C6. The triceps reflex is decreased with a radiculopathy of C7. There may be tingling and numbness in the thumb, index, and middle fingers, but muscle wasting is rare. Extend and laterally rotate the neck; when pain is elicited, Spurling sign is present. Active and passive motions of the neck are restricted and may be painless, but often produce subjective and objective crepitus. Coughing with the head held in extension may reproduce the pain [Carette S, Fehlings MG. Cervical radiculopathy. N Engl J Med. 2005;353:392–399]. DDX: Other causes, such as incomplete rupture of the supraspinatus, Pancoast tumor, and peripheral neuropathy must be excluded. Symptoms remit gradually over days to weeks.
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Neck and shoulder pain—Pancoast tumor, superior sulcus syndrome
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Lung cancer at the thoracic apex invades locally to involve the pleura, thoracic muscles, and neurovascular bundles, including the brachial plexus and cervical sympathetic chain. Pain is felt in local structures and is referred in the distribution of the involved nerves. Severe pain is present in the posterior part of the shoulder and axilla, often shooting down the arm, with paresthesia of the arm and hand. Paresis or wasting of arm muscles may occur. In addition to neck and shoulder pain, the complete syndrome includes Horner syndrome (unilateral miosis, ptosis, and absence of sweating on the ipsilateral face and neck). It may be confused with rupture of the supraspinatus tendon, cervical spondylosis, and peripheral neuritis.
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Back pain and stiffness—infectious spondylitis
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Spinal infection most commonly starts in the disks (diskitis) and anterior endplates of the vertebrae leading to vertebral erosion and collapse. Infection may extend anteriorly into the psoas or posteriorly into the epidural space leading to spinal cord compression. Pain and tenderness of the spinous vertebral processes are usually present over the site of infection, often with spasm of the sacrospinalis. The pain may be referred along a spinal nerve to be mistaken for appendicitis, pleurisy, or sciatica, if the back is not examined. Collapse of the vertebral body causes a gibbus and paraplegia may result. Psoas abscess, classic in tuberculosis, may form along the sheath of the psoas and point beneath the inguinal ligament. Pain in the spine may be localized by the heel-drop test: have the patient rise onto tiptoes, and then drop onto the heels; pain will be elicited at the site of infection. Infections with pyogenic organisms (S. aureus) are most common. Tuberculosis, Salmonella, brucellosis, fungi, and actinomycosis are less common.
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Tuberculous spondylitis (Pott disease)
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Tuberculosis of the spine is an indolent osteomyelitis of the anterior vertebral endplates with extension to the paravertebral soft tissues. Patients present with fever, night sweats, and pain. With cervical infection, the neck is held stiffly and spontaneous rotation of the head is absent. When seated, the patient may support the head with the hands (Rust sign). An abscess of the cervical vertebra may track to the retropharyngeal space. Patients with epidural extension can develop spinal cord compression. Vertebral collapse in the thoracic or lumbar spine can produce gibbus deformity and risk of instability. Rarely, cold abscess present as an inguinal mass from prevertebral extension.
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Back pain—epidural spinal cord compression. Masses in the closed epidural space may erode bone, compress spinal nerves, and compress the spinal cord. Most epidural masses extend from the adjacent vertebrae or from retroperitoneal malignancy to press on the anterior or anterolateral cord. Progressive axial, radicular, or referred back pain, unrelieved by recumbency, should prompt an immediate search for spinal cord compression. Pain is the most common presenting symptom followed by leg weakness, constipation, incontinence, and sensory disturbances. The latter indicate advancing cord compression, from which there is less chance for complete recovery. Paraplegia may occur in a matter of hours after the onset of neurologic signs. The pain may occur at any level and is increased by straight-leg raising, Valsalva maneuver, neck flexion, and movement. MRI of the entire vertebral column and cord is the most sensitive and specific test. CLINICAL OCCURRENCE: Breast, prostate, and lung cancer are the most frequent causes. Other cancers in adults include multiple myeloma, malignant lymphoma, renal carcinoma, sarcoma, and melanoma. In children, consider lymphoma, sarcoma, and neuroblastoma. Epidural abscess complicates infectious spondylitis.
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Back pain and stiffness—diffuse idiopathic skeletal hyperostosis (DISH)
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Asymmetric osteophytes develop at multiple levels of the spine leading to bridging of multiple intervertebral spaces and irregular ankylosis with decreased spinal motion, especially in the cervical and lumbar regions. The disk spaces are preserved. Men are affected more often than men.
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Back pain and stiffness—rigid spine syndrome
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The clinical syndrome of rigid spine, proximal muscle weakness, scoliosis, and joint contractures can have several different etiologies. The combination of restrictive chest disease and muscle weakness can lead to respiratory failure [Janssen WJ, Collard HR, Saint S, Weinberger SE. A perfect storm. N Engl J Med. 2005;353:1956–1961].
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Low back pain—lumbosacral strain
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Mechanical injury commonly occurs to the soft tissues (muscles, tendons, and ligaments) of the low back where the mobile lumbar spine meets the fixed sacrum, concentrating mechanical forces at the transition zone. Injury can be the result of a single, large loading force or of a repetitive loading with lesser forces. Usually the patient complains of aching pain near L5 and S1. The pain may radiate laterally or to the lateral aspect of the thigh. There is an increased lumbar lordosis and attempted spinal motion is accompanied by muscle spasm. Spinal flexion is limited and painful. The patient cannot lie flat without flexing the knees and hips to relieve pain. The straight-leg-raising test is negative except that lumbosacral pain occurs at extreme flexion. Have the patient lie prone with the pelvis resting on four pillows to separate the spinous processes. In this position, palpation of the spines and supraspinous ligament may reveal tenderness above or below the spine of L5; sometimes a depression is found indicating spondylolisthesis.
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Low back pain—sciatica
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Compression or direct injury to the sciatic nerve produces pain, altered sensation (dermatomes L4-S2), loss of muscle reflexes (ankle jerk), and, if severe, muscle power in the distribution of the nerve (e.g., ankle flexion, extension, inversion and eversion, great toe extension). Sciatica is pain in the distribution of the sciatic nerve. Pain is initially felt in the buttock and posterior thigh, and may extend to involve the posterolateral aspect of the leg to the lateral malleolus, the lateral dorsum of the foot and the entire sole. When nerve function is compromised, paresthesias are felt in the same distribution. Pain and paresthesias are intensified by coughing or straining. The nerve trunk is tender when palpated at the sciatic notch or stretched when the leg is extended while the thigh is flexed (Lasègue sign, straight-leg raising). Rectal examination should always be done to look for pelvic masses. A pulsating rectal mass associated with sciatica suggests aneurysm of the internal iliac or common iliac artery. Most cases are caused by herniated intervertebral disk.
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Low back pain—herniated intervertebral disk
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Herniation of a desiccated nucleus pulposus through tears in the annulus fibrosa produces pressure on nerve roots in the neural foramina laterally or directly on the cauda equina, conus or spinal cord if the extrusion is directly posterior (Fig. 13-26B). The onset of pain may be gradual or sudden and is partially relieved in recumbency. Sciatica may be the presenting symptom with pain in the buttock radiating to the thigh. In severe cases, the pain involves the leg, usually the lateral aspect, and some or all of the toes (dermatomes L4 to S2). Coughing, sneezing, or the Valsalva maneuver accentuates the pain. Chronic herniated disk is attended by symptom-free periods; continuous back pain is usually caused by some other lesion. On examination, the spine may be flexed, frequently with a lateral deviation toward the affected side. Active flexion and extension of the spine are limited, more so than lateral bending and rotation. Muscle spasm is most severe over the ipsilateral sacrospinalis with rigidity and tenderness, most pronounced on the affected side 5 cm lateral to the midline. Palpate for muscle rigidity, and other areas of tenderness including trigger points and fibrositis tender points. Have the patient heel-and-toe walk and extend the great toe against resistance. Pain with the straight leg test often occurs at less than 40 degrees with a prolapsed disk; when pain occurs only at much greater angles, there may be another cause. If the straight-leg-raising test is positive, repeat it and as the painful angle is approached, dorsiflex the foot sharply stretching the sciatic nerve to test its irritability. Check sensation noting the dermatome involved. [Vroomen PC, de Krom MC, Knottnerus JA. Diagnostic value of history and physical examination in patients suspected of sciatica due to disc herniation. J Neurol. 1999;246:899–906; Vroomen PC, de Krom MC, Knottnerus JA. Consistency of history taking and physical examination in patients with suspected lumbar nerve root involvement. Spine. 2000;25:91–96].
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Low back pain—spinal stenosis
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Compression of the lower lumbar and sacral roots occurs from overgrowth of bony tissue, from congenital narrowing, or degenerative disk disease that narrows the spinal canal. Pseudoclaudication is pain in the buttocks, bilateral posterior thighs, and calves while standing or walking upright, that is relieved by sitting or bending forward (reversing the lumbar lordosis and decreasing the degree of stenosis). DDX: Unlike intermittent claudication from vascular insufficiency, pain occurs while standing without walking. Sitting gives relief, in contrast to lumbar disk disease. Osteoporosis, spondylolisthesis, trauma, laminectomy, spinal fusion, spondylosis, scoliosis, acromegaly, and Paget disease are all potential contributors. Congenital narrowing of the canal is not uncommon.
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Sacral pain—lesions of the cauda equina
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Compression of the cauda equina (“horse’s tail”: the spinal roots below the level of the spinal cord termination, or conus) results in compromise of nerve function in one or more lumbar or spinal nerves, often accompanied by pain. Pain is localized to the sacrum and inner thigh, and the skin may be anesthetic. Bladder symptoms (including decreased urinary stream, hesitancy, and urinary retention), anal sphincter laxity, absent anal wink, and impotence are common. Compression occurs from a herniated intervertebral disk with posterior protrusion, epidural hematoma or abscess, and neoplasms.
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Sacroiliac pain—sacroiliac arthritis
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On arising, there is painful stiffness in the upper medial buttocks, which improves with exercise. Pain may be referred to the upper outer quadrant of the buttock, or the posterolateral aspect of the thigh. Sometimes there is a limp. Spinal rotation accentuates pain. The joint is tender to palpation or percussion. SI arthritis is characteristic of spondyloarthritis.
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Sacroiliac pain—sacroiliac strain
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Mechanical strain or postpartum relaxation of the ligaments about the joint is followed by inflammation. The patient complains of pain in the joint or in the upper inner quadrant of the buttock or the posterolateral aspect of the thigh (dermatomes L4, L5, S1). The joint is tender. The pain is accentuated by compressing the anterior iliac crests while the patient lies supine. The tendon reflexes in the lower limbs are normal.
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Ischial tuberosity pain—ischiogluteal bursitis
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The gluteal muscles cover the ischial tuberosities when standing. With thigh flexion, such as sitting, the muscles slide upward exposing the tuberosities and their tendon insertions to pressure, protected only by intervening skin, subcutaneous tissue, and the ischiogluteal bursa. Irritation of the bursa and/or tendon insertions can lead to secondary sciatic nerve irritation. This is more common with occupations characterized by sitting on a poorly cushioned seat. The pain is aggravated by sitting, coughing, walking, and standing on tiptoe. The patient lists toward the affected side with standing and walking, and the stride is shortened with circumduction of the affected foot. The patient sits with the affected buttock elevated and on lying supports the pelvis on that side. There is point tenderness on the ischial tuberosity. The straight-leg-raising test causes pain. The FABER sign is present. On rectal examination a region of tender, bulging, doughy tissue may be palpated in the lateral rectal wall. DDX: Patients with herniated disk and lumbosacral disease lie quietly and lack the FABER sign. This bursa may also be affected by gout. Pain is referred to the tuberosity from intramedullary lesions of the femoral head.
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Upper Extremity Syndromes
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Finger snapping or locking—trigger finger
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A nodular thickening forms in the long flexor tendon just proximal to the metacarpal head as a result of inflammation or repetitive trauma. In extension, the nodule lies within the flexor tendon sheath; during flexion, it is easily pulled from the proximal end of the sheath. The constricted mouth of the sheath resists reentry of the nodule during extension until it is suddenly achieved with a noticeable click. The middle or ring finger is usually involved (Fig. 13-28D). Flexion of the finger feels normal, but extension is accompanied by a snap that the patient sometimes refers to the region of the PIP joint. The nodule can be palpated moving with the tendon on active or passive flexion–extension. Triggering may or may not be painful.
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It is essential to distinguish superficial from deep tissue infections in the fingers. The latter are initially limited to specific tissue compartments giving characteristic signs.
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The skin over the matrix of the nail and the lateral nail folds is swollen, reddened, painful, and tender (Fig. 13-38A). When infection is over the nail root, light palpation of the inflamed area provokes exquisite pain. Pain from pressure on the nail plate indicates subungual abscess, which occurs between the nail plate and periosteum.
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Candida paronychia is minimally tender and does not drain. Chronic ulceration of the nail mantle and lateral folds occurs in occupations requiring frequent immersion of the hands in water or contaminated oil. The ulcers are indolent; abscess formation is rare.
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Abscess of the apical space
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Symptoms follow a puncture wound beneath the nail. The distal nail bed becomes red and extremely painful without much swelling (Fig. 13-38B). Maximum tenderness is proximal to the free edge of the plate, unlike a felon, which produces tenderness at the fingertip. When the abscess ruptures, drainage is at the free edge of the nail plate.
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Abscess of the terminal pulp space (Felon)
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Infection of the finger pad is confined within small fascial compartments attached to the periosteum (Fig. 13-38C). There is swelling of the fingertip and dull pain that becomes intense and throbbing with exquisite tenderness. Induration of the pulp indicates the presence of pus. The abscess may drain through the volar surface of the finger pad; osteomyelitis may occur.
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Abscess of the middle volar pulp space
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The finger is held in partial flexion to reduce the pain. A tender swelling occurs on the volar aspect of the finger between the PIP and DIP joints (Fig. 13-38D). The symptoms resemble those of a felon. Osteomyelitis may occur or the abscess may drain after burrowing to the distal flexor crease.
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Abscess of the proximal volar pulp space
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An abscess forms on the volar aspect between the MCP and PIP joints (Fig. 13-38D). The symptoms and signs are similar to those in the middle space, except the infection burrows proximally to involve the web space.
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Barber’s pilonidal sinus
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Short hair shafts may penetrate the soft skin in the finger webs producing inflammation. One or more nodules are felt which are drained by sinuses; seen as black dots between the fingers.
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Localized thickening of palmar fascia—Dupuytren contracture
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There is a painless nodular thickening of the palmar aponeurosis from hyalinization of collagen fibers beginning near the base of the digit. It extends to form a plaque or band adhering to the palmar fascia and producing retraction and dimpling of the palmar skin and flexion contracture of the fingers. The condition occurs exclusively in Caucasians, predominately men and 40% have a positive family history. It is more common in patients with alcoholism, epilepsy, or diabetes mellitus; the cause for these associations is unknown. It usually begins after age 40 years as an inconspicuous hard nodule fixed to the skin in the distal palm. Both hands may be involved. The ring finger is more often affected than the little, long, index or thumb. Fascial retraction pulls the finger(s) into partial flexion (Fig. 13-30I). Palpation reveals a hard cord over the tendon that is raised in extension making it readily seen. With progression, a painless contracture of the digit(s) results requiring surgical correction. Other fibrosing conditions that may be part of the same diathesis, occurring singly or in combination, are plantar fibrosis (Lederhosen syndrome) and fibrosis of the corpus cavernosum (Peyronie disease).
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Infections in the hand can be trivial or tragic depending upon the site of infection and the organism. Care must be taken in evaluation to identify deep space infections resulting from puncture or bite wounds that can rapidly destroy hand function.
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Painful palmar swelling—web space infection
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Fever, malaise, and diffuse pain in the hand and dorsal edema occur early. The two involved fingers are separated at their bases by a tender erythematous swelling; tenderness is maximal on the palmar surface (Fig. 13-39A).
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Painful palmar swelling—infection of the thenar space
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The thenar eminence is swollen, tender, and may have erythema and warmth. DDX: Deep hematoma gives similar symptoms and signs but with a history of blunt trauma. Extension at the IP joint is not painful as it is in tenosynovitis of the flexor pollicis longus.
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Painfully swollen palm—deep abscess of the palm
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There is usually a history of penetrating trauma, often minor. There is pain, swelling and limited motion. In addition to severe dorsal edema, the concavity of the palm is obliterated, or even elevated; the raised area is tender and erythematous; the erythema may extend to the volar wrist extending under the flexor retinaculum. This is a surgical emergency.
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Painful palm and finger swelling—acute suppurative flexor tenosynovitis
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Throbbing pain begins in a finger, progressing toward the palm. The finger and dorsum of the hand are swollen. The finger is held slightly flexed; the patient resists moving it. Passive extension of adjacent fingers is painful, of the affected finger, exquisitely so. Test for the point of maximum tenderness by gently palpating the palm and flexor surface of the finger with the blunt end of an applicator or tongue depressor. If it is located at the proximal end of the tendon sheath of the index, middle, or ring finger, involvement of the sheath is certain. If there is no localization, the sheath may have ruptured.
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Painfully swollen palm—infection of the ulnar bursa
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There is dorsal edema and fullness on the ulnar side of the palm (Fig. 13-39A). The point of maximum tenderness is halfway between the lunate and the fifth MCP joint.
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Painfully swollen palm—infection of the radial bursa
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The interphalangeal joint of the thumb is flexed and passive extension produces pain. There is tenderness and swelling over the sheath of the flexor pollicis longus.
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Painfully swollen palm—metacarpal fracture
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In a transverse fracture, the fragments of the metacarpal bone are bowed into the palm to produce a painful prominence (Fig. 13-39B). The prominence may be obscured by soft-tissue swelling, but dorsal palpation will localize tenderness at the fracture site. In a spiral fracture, proximal slippage of the distal fragment produces shortening revealed by loss of prominence in the corresponding knuckle when the fist is closed. Rotation of the distal fragment is present if the affected finger is not aligned with the unaffected fingers when the tips are flexed onto the base of the thenar eminence. Bennett fracture is an oblique break through the base of the first metacarpal, frequently with subluxation of the carpal-metacarpal joint. The thumb is semiflexed, and it cannot be opposed to the ring or little finger and the fist cannot be clenched.
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Painful swelling in the anatomic snuffbox—tenosynovitis
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Inflammation in the tendon sheaths of the extensor pollicis longus and brevis as they pass under a fibrous band near the radial styloid causes pain and tenderness. Pain is felt in the region of the snuffbox. The patient has pain with pinching, thumb extension and ulnar deviation of the wrist. When the fist is clenched over the flexed thumb, gentle but firm ulnar deviation of the hand by the examiner elicits pain at the radial styloid process, Finkelstein test (Fig. 13-40B). This pain may be transmitted down the thumb or toward the elbow. Passive extension of the thumb is painless. Crepitus may be felt or auscultated over the tendon sheath during thumb flexion–extension.
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A sausage-like swelling approximately 4 cm long (Fig. 13-40C), involves the tendon sheaths at the radial border of the snuffbox. The cause is usually trauma, although inflammation can be produced by gout, pseudogout, or gonococcal infection.
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Chronic stenosing tenosynovitis—de Quervain tenosynovitis
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The symptoms are similar to the acute process and are frequently chronic and recurrent, exacerbated by repetitive movements of the wrist and thumb. Chronic inflammation involves all layers of the tendon sheath.
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Forearm pain and weakness—flexor compartment syndrome and Volkmann ischemic contracture
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See compartment syndrome). Volkmann contracture, the fibrosis and shortening of the muscles, is a late finding. Passive extension of the fingers produces pain in the forearm. The fingers may be cyanotic and are often edematous. The radial pulse is absent, the skin over the hands is cool, and median nerve sensation is diminished. Emergent surgical consultation is required. In Volkmann contracture, the fingers are fixed in flexion by shortening of the fibrotic bellies of the digital flexors in the forearm. Because the flexor tendons are free to move in their sheaths, slight extension of the fingers is permitted when the wrist is held in flexion. This distinguishes it from adhesions of flexor tendons to their sheaths. Precipitating events are forearm and supracondylar fractures and circumferential bandages or casts applied shortly after trauma.
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Elbow pain—lateral and medial epicondylitis, tennis elbow
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Repetitive forceful wrist and/or finger flexion and extension focus tension on the common proximal tendon insertions at the lateral (extensors) and medial (flexors) humeral epicondyles. Lateral Epicondylitis causes pain in the lateral aspect of the elbow, accentuated by use of the hand during a power grip (when the wrist extensors are also contracting), as in lifting objects. Palpation discloses tenderness over the lateral epicondyle and 1 cm distally. Having the patient hold the long finger in full extension against resistance reliably reproduces the pain. In the Cozen test (resisted wrist extension), the patient is asked to keep the fist clenched while extending the wrist against resistance reproducing the patient’s lateral epicondylar pain. In the Mill maneuver, have the elbow held in extension with the wrist flexed; when you pronate the forearm against the patient’s resistance, epicondylar pain is elicited. Medial Epicondylitis is less common. The pain is located just distal to the medial epicondyle in the flexor tendons and is reproduced by maneuvers requiring wrist and finger flexion or arm pronation against resistance.
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Pain in the upper arm—bicipital tenosynovitis
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Inflammation in the tendon sheath of the biceps where it emerges anteriorly from the capsule of the shoulder joint is usually the result of overuse. Pain is located near the insertion of the pectoralis major on the humerus and may shoot down the arm. Shoulder motions are somewhat limited, especially flexion of the arm. Palpation of the bicipital groove reproduces the pain. Yergason Sign: With the elbow flexed to 90 degrees and the forearm pronated, ask the patient to supinate against resistance; pain in the anteromedial aspect of the shoulder is a positive test.
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Pain in the shoulder—coracoiditis
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Repeated acute or chronic arm work produces inflammation at the origin of the short head of the biceps and the coracobrachialis muscles on the tip of the coracoid process. There is a history of trauma with pain and tenderness at the tip of the coracoid process. The pain is reproduced by adduction and external rotation of the humerus, or having the patient perform against resistance either supination of the forearm with the elbow flexed, forward flexion of the shoulder, or adduction of the flexed shoulder.
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Painful arc and impingement sign—rotator cuff injury
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During arm abduction the supraspinatus tendon and its insertion on the proximal greater humeral tuberosity must pass beneath the acromion. The subacromial bursa is positioned to decrease the friction, or impingement, which occurs with this motion. Repetitive forceful motion causes mechanical irritation to the tissues eliciting inflammation of the tendon and bursa. Sudden or severe loading of the shoulder may result in incomplete or complete tears of the tendon. Three conditions present with similar signs and symptoms. A history of sudden onset favors partial tear of the tendon, whereas repetitive shoulder motion and subacute or chronic symptoms favor tendonitis or bursitis. All have painful abduction between 60 and 120 degrees, often prohibiting full active range of motion. Less painful passive range of motion is preserved (Fig. 13-41). Precise distinction between the three is not possible with physical examination. Partial Rupture of the Supraspinatus Tendon. When the tendon is only incompletely torn, the rotator cuff is intact. Pain is referred to the humeral insertion of the deltoid muscle, but there is no tenderness at that point. Sometimes the pain extends down the arm to the elbow or beyond. Tenderness is elicited just beneath the acromial tip or in the notch between greater and lesser humeral tubercles. DDX: If weakness of the supraspinatus, weak external rotation and impingement signs are all present, a rotator cuff tear is likely [Murrell GA, Walton JR. Diagnosis of rotator cuff tears. Lancet. 2001;357:769–770]. Acute Supraspinatus Tendinitis: This usually occurs in a person 25 to 45 years of age, with a dull ache developing in the shoulder without antecedent trauma. The pain steadily worsens and may be excruciating. The diagnostic test is abduction to 90 degrees and then full internal rotation, which reproduces pain. Tenderness is pronounced beneath the acromial tip. Chronic Supraspinatus Tendinitis: Dull shoulder pain develops in a patient who is usually between 45 and 60 years old and without preceding trauma. Abduction is painless to 60 degrees, where the patient feels a jerk with pain in the region of the deltoid muscle. Pronounced tenderness can be elicited in the notch between the greater and lesser tubercles of the humeral head or beneath the acromial tip. Crepitus may also be present. Lying on the shoulder produces pain that prevents sleeping on the affected side for months. Subacromial Bursitis: This can be acute or chronic and often accompanies supraspinatus tendonitis. The pain is constant and aggravated by elevation in abduction. There is often a history of heavy arm use.
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Minimal arm elevation—complete rupture of the supraspinatus tendon, torn rotator cuff
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The supraspinatus muscle initiates arm abduction from 0 to 45 degrees, where the deltoid engages. Inability to initiate elevation in 90° abduction indicates rupture of the supraspinatus tendon. This usually occurs after age 40. The patient cannot elevate the arm against minimal resistance at 30 degrees elevation and 90 degrees abduction, but passive motion is free and painless. Initial shoulder motion is mostly with the scapula. There is resistance to external rotation when the arm is held at the side with elbow flexed. As the arm is moved forward, one may palpate a jerk, fine crepitus, or an indentation in the subacromial region between the greater and lesser humeral tubercles (Fig. 13-18D). Wasting of the supraspinatus and infraspinatus occurs after 3 weeks.
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Adhesive capsulitis—frozen shoulder
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Chronic inflammation of the rotator cuff and joint capsule leads to shortening of the usually loose tendinous cuff and to progressive loss of motion. All types of arthritis may involve the shoulder. In the early stages of inflammatory processes, motions are inhibited by pain; later, adhesions restrict motion. This often follows unresolved subacromial bursitis or supraspinatus tendinitis. At onset, pain is that of the original injury. Progressive limitation of motion ensues until capsular contraction and fibrosis abate the pain with associated muscle wasting. Osteoarthritis may be associated with crepitus in the joint, but effusions are relatively rare. Over time, there is progressive loss of glenohumeral motion. With chronically limited motion, generalized muscle wasting occurs. Joint effusion suggests rheumatoid arthritis, crystal arthritis, or less-common disorders such as villonodular synovitis.
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Conditions of the entire upper limb
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Lower motor neuron or peripheral nerve injuries produce muscular denervation with flaccid paralysis and muscle wasting. After injuries to the brachial plexus or poliomyelitis, the arm may hang limply at the side with the flexed palm facing backward (Fig. 13-32B).
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Injury to upper motor neurons leads to uninhibited muscular contraction with the antigravity flexors overpowering the weaker extensors. With hemiplegia, the arm is carried with flexion of the elbow, wrist, and fingers (Fig. 13-32C).
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Lower extremity syndromes
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Painless limp—osteonecrosis, aseptic hip necrosis
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Ischemia of the femoral head leads to bone necrosis with collapse and mushrooming of the femoral head. It may be unilateral or bilateral. Although the cause may be unknown, use of corticosteroids, diabetes, obesity, and alcohol are each associated with an increased risk. Osteonecrosis of the epiphysis in childhood (Legg–Calvé–Perthes disease) is relatively painless, whereas osteonecrosis in adults may be painful. Usually it begins with a painless limp and all motions of the hip are slightly impaired. Later, there is severe limitation of abduction and rotation. Muscle wasting and shortening of the limb are common. The Trendelenburg gait suggests hip dislocation.
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Painful hip—septic arthritis
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Usually, there is groin pain, fever, leukocytosis, and prostration. The thigh may be held in slight abduction and lateral rotation to best accommodate the joint effusion. All motion of the hip is painful. Rarely, fluctuation can be felt in the joint. Because pain may be referred only to the knee, knee pain should always prompt examination of the hip. Early diagnosis is urgent because the joint cartilage may be irreparably damaged in a few hours without proper drainage and antibiotics. Tuberculous arthritis is more insidious, often presenting with a limp and pain only after exertion.
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Painful hip—slipped capital femoral epiphysis
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The femoral head dislocates on the femoral neck at the relatively weak growth plate during rapid growth in adolescence. Patients walk with a limp accompanied by pain. The distinguishing feature is painful limitation of internal rotation when thigh and knee are both flexed. This may be succeeded by shortening and outward rotation from anterosuperior displacement of the femoral neck.
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Painful hip—torn labrum
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A tear in the acetabular labrum occurs because of trauma. Patients are usually young men with persistent hip pain without limitation of motion. There may be no history of specific injury, or only of injury thought to be minor at the time. Instability of the hip is increased. DDX: Hip impingement syndrome is being recognized more frequently and should be considered in the differential diagnosis.
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Painful hip—osteoarthritis
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Boring pain in the groin and buttocks, sometimes with referral to the knee is frequent. Initially, stiffness on arising disappears with exercise, but later walking is limited by pain. The thigh is held in adduction. Passive motion is restricted in internal rotation and abduction and may be painful. Palpable and audible crepitus may be present.
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Painful hip and limping—chronic hip dislocation
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Dislocation of the hip may be congenital or acquired. Weakness of the supporting muscles (from nerve or muscle injury), joint infections, and trauma lead to dislocation in adults. Prosthetic hips are especially susceptible to dislocation with flexion and external rotation. For congenital hip dislocation, consult pediatric texts. In adults, Trendelenburg gait (Thigh and leg girth) is present. With the patient supine and the hips and knees flexed, the knee of the affected leg will be lower when observed from the foot of the bed. Inspect the buttocks with the patient standing on one leg at a time; normally, the pelvis tilts upwards on the nonweightbearing side (Fig. 13-33A). In the Trendelenburg sign, when standing on the affected leg, the pelvis tilts downwards on the nonweightbearing side because the muscles are not strong enough to support the pelvis when the femur is not engaged in the acetabulum. The sign is also positive with disease of the glutei, fracture of the femoral head, and in severe degrees of coxa vara. In adults, there is hip pain and premature OA of the affected joint.
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Medial knee pain—anserine bursitis
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The anserine bursa lies superficial to the tibial collateral ligament and deep to the pes anserina (goose foot) formed by the tendons of the sartorius, gracilis, and semitendinosus (Fig. 13-34). The patient complains of pain in the anterior medial aspect of the knee 2 to 3 cm distal to the joint line. Pain is aggravated by walking; especially while climbing stairs. Tenderness, usually without swelling, is palpated on the medial aspect of the knee.
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Anterior knee pain—chondromalacia patellae
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Degeneration of the articular surface of the patella occurs from unknown cause. It is more common in women than men. Pain is felt anteriorly and is aggravated by prolonged sitting and walking down stairs. A small effusion may be present. Passive joint motion is painless. Rocking the patella in the femoral groove frequently elicits pain, often with palpable crepitation.
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Anterior knee pain—quadriceps and patellar tendonitis
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Pain is felt in the anterior knee either above (quadriceps) or below (patellar) the patella. It occurs following an unusual level of activity such as running, lifting, climbing, up and down squatting); it may occur as a result of chronic overuse. Pain is well localized to the involved tendon and reproduced by direct pressure and resisted knee extension.
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Anterior knee pain after exercise—osteochondritis of tibial tubercle (Osgood–Schlatter disease)
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Before closure of the epiphysis of the tibial tubercle during adolescence, vigorous exercise produces chronic traction injury with partial avulsion of the tubercle. Pain occurs after exercise and a tender, hard swelling is seen and felt at the attachment of the patellar tendon.
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Tibial pain after exercise—stress fracture of the tibia
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An incomplete cortical fracture of the tibia results from repeated strenuous use of the leg. Dull aching pain begins gradually with progressively shortening intervals after exercise and persists for hours. There is localized tenderness over the tibia, frequently along the medial border. Pain at the fracture site may be elicited by springing the tibia: with the patient supine, place one hand on the knee and the other on the heel; pull the tibia laterally against your knee as a fulcrum.
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Exertional calf pain—intermittent claudication
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Anterior leg pain after exercise—tibial compartment syndrome
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See Compartment syndrome. Several hours after unusually strenuous leg exercise, stiffness followed by severe pain occurs in the anterior tibial muscular compartment. The region becomes swollen, tense, tender, and warm. Ischemic necrosis of the muscles may occur if the pressure is not relieved by fasciotomy.
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Foot pain—tarsal tunnel syndrome
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The tarsal tunnel is behind and inferior to the medial malleolus of the tibia. Its bony floor is roofed by the flexor retinaculum extending from the medial malleolus to the calcaneus. Through the tunnel pass several tendons and the posterior tibial nerve, which divides into the calcaneal nerve to the skin of the heel, the medial plantar nerve to the skin and muscles of the medial aspect of the sole, and the lateral plantar nerve to the lateral portion of the sole. Compression of the nerves in the tunnel causes numbness, burning pain, or paresthesias in portions of the sole. Paresis or paralysis of some small muscles of the foot may occur. Occasionally, a tender area is palpated at the posterior margin of the medial malleolus. The diagnosis is confirmed by nerve conduction studies. Precipitating conditions include fracture or dislocation of the bones near the tunnel, traumatic edema, tenosynovitis, chronic stasis of the posterior tibial vein and foot strain.
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Forefoot pain—metatarsalgia
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Loss of the normal transverse metatarsal arch from the first to the fifth metatarsal heads leads to abnormal weight bearing on the second to fourth metatarsal heads, which are insufficiently cushioned. Ask the patient to point to the site of the pain. Pain occurs in the region of the distal metatarsal heads. Examination may show callus under the second to fourth metatarsals and decreased callus under the first and fifth. Pressure on the callus reproduces the pain. Symmetrical bilateral metatarsalgia may be an early symptom of RA. Fibroneuroma gives similar pain, so squeeze the foot transversely to see if the pain is reproduced.
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Forefoot pain—metatarsal stress fracture
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Excessive walking or standing may cause a stress fracture of the metatarsal shaft (Fig. 13-36C). Pain develops gradually. Muscle cramps and slight swelling may occur. Motion of the corresponding toe is painful. Pain is usually localized several centimeters proximal to the metatarsal head.
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Forefoot pain—infection in the interdigital space
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Puncture of the sole may cause infection in one of the four interdigital subcutaneous spaces. The abscess may point between two metatarsals causing swelling on the dorsum of the foot. Walking produces pain between the metatarsals. Tenderness is localized to the interdigital space.
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Chronic painless enlargement of the legs
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Several causes must be distinguished. Although some evidence of obesity is present elsewhere in the body, adipose tissue about the ankles may be disproportionately great, while the feet are spared. The tissue has the consistency of fat, and pitting edema is absent. Chronic deep venous obstruction usually is preceded by a history suggesting thrombophlebitis. The venous deficit may produce pain in the legs. Some pitting edema is usually present, although it may be obscured by thickening of the skin from chronic inflammation (dermatoliposclerosis). The skin is stained with hemosiderin and may be cyanotic. Superficial veins may be dilated. Postphlebitic syndrome results from previous occlusion of the deep veins either without recanalization or resulting in destruction of the valves with persistent venous incompetence. One-third of patients with acute deep vein thrombosis will develop postphlebitic syndrome (Post-phlebitic syndrome). The leg is persistently enlarged, usually with pitting edema. Sensations of fullness and pain occur with prolonged sitting or standing and chronic stasis changes are common. Early treatment decreases long-term disability. Varicose veins are readily recognized when they are associated with edema. Chronic edema from any cause leads to subcutaneous and cutaneous fibrosis with woody induration that does not pit (dermatoliposclerosis). Lymphedema causes firm, uniform, nonpitting swelling which extends to the feet, without venous engorgement or cyanosis.
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See Tender Points,. Fibromyalgia is at the extreme end of an ill-defined group of disorders variously described as polysomatic distress or functional somatic disorders [Barsky AJ, Borus JF. Functional somatic syndromes. Ann Intern Med. 1999;130:910–921]. It is characterized by chronic widespread pain (CWP) involving both sides of the body above and below the waist, and the axial skeleton. The pain is described as diffuse aching and is accompanied by stiffness, nonrestorative sleep, and fatigue, and must have been present for at least 3 months and not explained by another disorder. Fibromyalgia is more common in women than men. The 1990 American College of Rheumatology (ACR) diagnostic criteria included at least 11 of 18 paired, bilateral tender points elicited on physical examination (Fig. 13-42). In 2010 new ACR criteria were proposed that eliminated the tender points. The new criteria are based on a Symptom Severity (SS) scale and the Widespread Pain Index (WPI) [Wolfe F, Hauser W. Fibromyalgia diagnosis and diagnostic criteria. Ann Med. 2011;43:495–502]. Each specific symptom (fatigue, waking unrefreshed, and cognitive symptoms) and the widespread somatic symptoms are each scored 0 to 3. The WPI and SS are combined to form a polysymptomatic distress scale that is continuous rather than dichotomous. Worksheets and supportive materials can be obtained at www.arthritis-research.org/research/fibromyalgia-criteria.
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Myofascial pain syndrome
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Afferent signals from the trigger areas are believed to remodel spinal and possibly thalamic pain pathways to cause reflex muscle spasm and diminished blood flow, which, in turn, increase the sensitivity of the trigger area. See Trigger Points. Chronic recurrent pain occurs roughly in the distribution of one or more muscles and their distribution of referred pain. The pain may be lancinating, aching, boring, or a feeling of muscular stiffness. Often, the patient relates the onset to some specific trauma or activity. The distinguishing feature is the presence of one or more trigger points, with or without palpable muscle spasm. The examiner should palpate the entire region carefully with firm pressure of the fingertips. Often, the trigger point is discovered some distance from the referred pain area. The diagnosis is confirmed when the pain is relieved by injection of the primary trigger point with a local anesthetic.
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Polymyalgia rheumatica
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This inflammatory disease of unknown origin is characterized by pain in proximal muscle groups, with inflammation demonstrable by MRI in the shoulder girdle tendon sheaths and bursae, and less commonly the shoulder joint itself. It usually occurs after the sixth decade and is most common in women. Pronounced morning stiffness of the neck, shoulder, and upper back muscles usually begins gradually. Some patients have sudden onset and can note the day and time of their first symptoms. Muscles of the low back, pelvic girdle, and thighs are less prominently involved and/or may develop later. It is common in patients with temporal arteritis, but only 15% of patients presenting with PMR develop temporal arteritis. Although spontaneously painful, the muscles are seldom very tender, trigger points are not characteristic, and the stiffness is improved by activity. The erythrocyte sedimentation rate is usually markedly elevated; symptoms are promptly relieved by low-dose corticosteroids.
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Edema, hemorrhage, and inflammation from injury to muscle enclosed within a constricting fascial compartment lead to increasing pressure within the compartment sufficient to occlude venous drainage further increasing swelling. Eventually the pressure exceeds arteriolar pressure and ischemic necrosis supervenes. A high index of suspicion is required to recognize this before irreversible muscle injury with contractures occurs. The patient complains of severe pain often disproportionate to apparent injury. The onset of ischemia is indicated by the five P’s: Pain, Puffiness, Pallor, Pulselessness, and Paralysis. Palpation of the affected compartment reveals tense distention of the fascia with severe pain. If major arteries or nerves pass through the compartment, distal pulses may be diminished and sensation impaired. Without relief of pressure, muscle necrosis leads to fibrosis, shortening, and loss of function. Diagnosis is made by measuring compartment pressure; treatment is surgical fasciotomy.
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Heavy infection with Trichinella spiralis after ingestion of incompletely cooked infected meat (pork, bear meat) may cause severe and, infrequently, death. The organisms localize in muscle. One to four days after ingestion, the patient complains of nausea and vomiting, diarrhea, and abdominal pain. Within 10 days, there are fever, dyspnea, anorexia, myalgia, and asthenia. Physical findings include periorbital edema, scarlatiniform rash, splinter hemorrhages under nails, tremors, and involuntary movements.