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CONNECTIVE TISSUE DISEASE

DEFINITION

Heterogeneous disorders that share certain common features, including inflammation of skin, joints, and other structures rich in connective tissue; as well as altered patterns of immunoregulation, including production of autoantibodies and abnormalities of cell-mediated immunity. While distinct clinical entities can be defined, manifestations may vary considerably from one pt to the next, and overlap of clinical features between and among specific diseases can occur.

SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

DEFINITION AND PATHOGENESIS

Disease of unknown etiology in which tissues and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes. Genetic, environmental, and sex hormonal factors are likely of pathogenic importance. T and B cell hyperactivity, production of autoantibodies with specificity for nuclear antigenic determinants, and abnormalities of T cell function occur.

CLINICAL MANIFESTATIONS

90% of pts are women, usually of child-bearing age; more common in blacks than whites. Course of disease is often characterized by periods of exacerbation and relative quiescence. May involve virtually any organ system and have a wide range of disease severity. Common features include:

  • Constitutional—fatigue, fever, malaise, weight loss

  • Cutaneous—rashes (especially malar "butterfly" rash), photosensitivity, vasculitis, alopecia, oral ulcers

  • Arthritis—inflammatory, symmetric, nonerosive

  • Hematologic—anemia (may be hemolytic), neutropenia, thrombocytopenia, lymphadenopathy, splenomegaly, venous or arterial thrombosis

  • Cardiopulmonary—pleuritis, pericarditis, myocarditis, endocarditis. Pts are also at increased risk of myocardial infarction usually due to accelerated atherosclerosis.

  • Nephritis—classification is primarily histologic (Table 319-2, p. 2727, in HPIM-18)

  • GI—peritonitis, vasculitis

  • Neurologic—organic brain syndromes, seizures, psychosis, cerebritis

Drug-Induced Lupus

A clinical and immunologic picture similar to spontaneous SLE may be induced by drugs; in particular: procainamide, hydralazine, isoniazid, chlorpromazine, methyldopa, minocycline, anti-TNF agents. Features are predominantly constitutional, joint, and pleuropericardial; CNS and renal disease are rare. All pts have antinuclear antibodies (ANA); antihistone antibodies may be present, but antibodies to dsDNA and hypocomplementemia are uncommon. Most pts improve following withdrawal of offending drug.

EVALUATION

  • Hx and physical exam

  • Presence of ANA is a cardinal feature, but a (+) ANA is not specific for SLE. Laboratory assessment should include: CBC, ESR, ANA and ANA subtypes (antibodies to dsDNA, ssDNA, Sm, Ro, La, histone), complement levels (C3, C4, CH50), serum immunoglobulins, VDRL, PT, PTT, anticardiolipin antibody, lupus anticoagulant, urinalysis.

  • Appropriate radiographic studies

  • ECG

  • Consideration of renal biopsy if evidence of glomerulonephritis

DIAGNOSIS

Made in the presence of four or more published criteria (Table 319-3, p. 2728, in HPIM-18).

TREATMENT Systemic Lupus Erythematosus

Choice of therapy is based on type and severity of disease manifestations. Goals are to control acute, severe flares and to develop maintenance strategies whereby symptoms are suppressed to an acceptable level. Treatment choices depend on (1) whether disease is life-threatening or likely to cause ...

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