Pleural involvement

Pleurisy is the most common and often the presenting manifestation of SLE, and is found in up to 83 per cent of patients at necropsy. Pleural effusions are seen more frequently in older patients and in drug-induced SLE. Non-specific pathological changes in the pleura include infiltration by lymphocytes, mononuclear cells, and plasma cells, together with various degrees of fibrosis. Painful pleurisy occurs in 41 to 51 per cent of patients, and pleuritic pain may be the first symptom in many. Young females who present with new-onset pleurisy or pleural effusion should be evaluated for SLE. An important differential diagnosis is pulmonary embolism, since SLE patients are at higher risk of thromboembolic phenomena. Pleuritic pain may be unilateral or bilateral and is usually located at the costophrenic margins. Pleural effusions are usually small to moderate, bilateral in nearly 50 per cent, occasionally associated with a small pericardial effusion, and frequently accompanied by dyspnea, cough, and fever. Hemorrhagic effusions have been infrequently reported. Pleural effusions may also occur as a complication of lupus-induced nephrotic syndrome or an infectious process. Pleural biopsy is rarely required as histological findings are non-specific.

Thoracentesis is usually unnecessary unless the cause of the pleural effusion is uncertain. The pleural fluid may be clear or serosanguinous; grossly bloody effusions are uncommon. The fluid is almost always an exudate with varying numbers of leukocytes, a normal (greater than 60 mg/dl) or high glucose, decreased levels of total hemolytic complement and of C3 and C4 components, and increased immune complexes. Although decreased, and occasionally undetectable, levels of complement components, observed in nearly 80 per cent of lupus effusions, have been used to corroborate the diagnosis of lupus pleuritis, they are not diagnostic because similar findings have been described in rheumatoid pleural effusion and other disorders. The presence of antinuclear antibody in the pleural fluid is non-diagnostic. Likewise, the nature of immune complexes in SLE-induced pleural effusion is unclear. Lupus erythematosus cells have been found in zero to over 85 per cent of lupus effusions. The presence of in vivo lupus erythematosus cells in pleural fluid is considered to be characteristic for SLE, and has not been described in other conditions except drug-induced SLE.

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