Diffuse Alveolar Hemorrhage in Autoimmune Diseases

Abstract

Purpose of review: The present paper establishes a narrative and analytical review of diffuse alveolar hemorrhage (DAH) in ANCA-associated vasculitis, systemic lupus erythematosus, and antiphospholipid syndrome.

Recent findings: Recent studies found a frequent association between DAH and infections and systemic lupus erythematosus and its associated factors. Biological therapies like rituximab have demonstrated benefit mainly in patients with ANCA-associated vasculitis. Main clinical manifestations of diffuse alveolar hemorrhage in these three diseases include dyspnea, pulmonary infiltrates, cough, and hypoxemia. The presence of hemorrhagic bronchoalveolar lavage, hemosiderin containing macrophages, or an increase of carbon monoxide diffusing capacity have been described in some series as helpful findings for the diagnosis. Hemoptysis has been seen mainly in systemic lupus erythematosus. The cornerstone of therapy includes glucocorticoids and cyclophosphamide, and recent findings in ANCA-associated vasculitis suggest the similar benefit of rituximab. Future evaluations and systematic reviews will help to define the real benefit for therapies that appeared to be controversial at the moment.

Keywords: Antiphospholipid syndrome; Diagnosis; Diffuse alveolar hemorrhage; Systemic lupus erythematosus; Treatment; Vasculitis.