Antiphospholipid syndrome in a patient with rapidly progressive fibrosing alveolitis

Abstract

The antiphospholipid syndrome is a thrombotic disorder which can occur in a primary form or more classically in systemic lupus erythematosus. An association between cryptogenic fibrosing alveolitis and the antiphospholipid syndrome has not previously been reported. We describe a patient with severe cryptogenic fibrosing alveolitis who developed pulmonary embolism and myocardial infarction in the presence of antiphospholipid antibody. The case also illustrates that worsening respiratory function may represent superimposition of one lung pathology on another, and may not simply be a deterioration of the pre-existing disease.