PULMONARY CAPILLARITIS, ALVEOLAR HEMORRHAGE, AND RECURRENT MICROVASCULAR THROMBOSIS IN PRIMARY ANTIPHOSPHOLIPID SYNDROME

Pulmonary manifestations of the antiphospholipid syndrome (APS) includ e pulmonary hypertension, pulmonary embolism, and in patients with the catastrophic APS, respiratory insufficiency associated with diffuse p ulmonary infiltrates. There are few descriptions of pathological findi ngs associated with these pulmonary complications. We document pulmona ry capillaritis, recurrent microvascular thrombosis and alveolar hemor rhage in patients with APS. These patients have a wide spectrum of cli nical presentations, ranging from recurrent episodes of fever, minimal hemoptysis, and mild dyspnea, to respiratory insufficiency requiring mechanical ventilation. Pulmonary involvement can occur alone or simul taneously with other manifestations of APS and may range from mild to severe. Pulmonary capillaritis and alveolar hemorrhage are complicatio ns of APS, and whether they are causally related to recurrent thromboe mbolism and pulmonary hypertension remains undetermined.