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Key Features

Essentials of Diagnosis

  • Occurs mainly in young women

  • Rash over areas exposed to sunlight

  • Joint symptoms in 90% of patients

  • Anemia, leukopenia, thrombocytopenia

  • Glomerulonephritis, central nervous system disease, and complications of antiphospholipid antibodies are major sources of disease morbidity

  • Serologic findings: antinuclear antibodies (100%), anti–double-stranded DNA antibodies (approximately two-thirds), and low serum complement levels (particularly during disease flares)

General Considerations

  • An inflammatory autoimmune disorder

  • Characterized by autoantibodies to nuclear antigens

  • Can affect multiple organ systems

  • Many of its clinical manifestations are secondary to the trapping of antigen-antibody complexes in capillaries of visceral structures or to autoantibody-mediated destruction of host cells (eg, thrombocytopenia)

  • The clinical course is marked by spontaneous remission and relapses

  • Severity may vary from a mild episodic disorder to a rapidly fulminant, life-threatening illness

Demographics

  • About 85% of patients are women

  • Sex hormones appear to play some role; most cases develop after menarche and before menopause

  • Among older individuals, the sex distribution is more equal

  • Race is also a factor, as SLE occurs in 1:1000 white women but in 1:250 black women

  • Familial occurrence of SLE has been repeatedly documented

Clinical Findings

Symptoms and Signs

  • The systemic features include fever, anorexia, malaise, and weight loss

  • Most patients have skin lesions at some time

    • The characteristic "butterfly" (malar) rash affects less than half of patients

    • Other cutaneous manifestations are

      • Panniculitis (lupus profundus)

      • Discoid lupus

      • Typical fingertip lesions

      • Periungual erythema

      • Nail fold infarcts

      • Splinter hemorrhages

  • Alopecia is common

  • Mucous membrane lesions tend to occur during periods of exacerbation

  • Raynaud phenomenon, present in about 20% of patients, often antedates other features of the disease

  • Joint symptoms, with or without active synovitis

    • Occur in over 90% of patients

    • Often the earliest manifestation

    • The arthritis can lead to reversible swan-neck deformities, but erosive changes are almost never noted on radiographs

    • Subcutaneous nodules are rare

  • Ocular manifestations include

    • Conjunctivitis

    • Photophobia

    • Transient or permanent monocular blindness

    • Blurring of vision

    • Cotton-wool spots on the retina

  • Pulmonary manifestations

    • Pleurisy

    • Pleural effusion

    • Bronchopneumonia

    • Pneumonitis

    • Restrictive lung disease

    • Alveolar hemorrhage (uncommon but life-threatening)

  • Cardiac manifestations

    • The pericardium is affected in the majority of patients.

    • Heart failure may result from myocarditis and hypertension.

    • Cardiac arrhythmias are common.

    • Atypical verrucous endocarditis of Libman-Sacks is usually clinically silent but occasionally can produce acute or chronic valvular regurgitation—most commonly mitral regurgitation.

  • Mesenteric vasculitis

    • May closely resemble polyarteritis nodosa, including the presence of aneurysms in medium-sized blood vessels

    • Abdominal pain (particularly postprandial), ileus, peritonitis, and perforation may result

  • Several forms of glomerulonephritis may occur, including mesangial, focal proliferative, diffuse proliferative, and membranous; some patients may also have interstitial nephritis

Differential Diagnosis

  • Drug-induced lupus

  • Rheumatoid arthritis

  • Systemic vasculitis

  • Scleroderma

  • Primary antiphospholipid syndrome

  • Inflammatory myopathies

  • Viral hepatitis

  • Sarcoidosis

  • Acute drug reactions

Diagnosis

  • The diagnosis ...

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