Objective: To describe clinical manifestations, laboratory findings, and treatment of patients with the antiphospholipid syndrome (APS) who develop diffuse alveolar hemorrhage.
Methods: Diffuse alveolar hemorrhage is an occasionally reported manifestation of the APS. The diagnosis, however, may be overlooked or manifestations attributed to another disease process. Seven episodes in 5 patients with primary APS were identified and retrospectively reviewed for presenting symptoms and signs, laboratory findings, and response to treatment.
Results: The severity of the condition varies, and diffuse alveolar hemorrhage may be the initial manifestation of APS. Patients may present with symptoms ranging from cough, dyspnea, and fever with or without hemoptysis, to symptoms of acute respiratory failure. Hypoxemia and anemia are usually present. Other causes need to be excluded. Bronchoscopy and bronchoalveolar lavage with or without biopsy often aid in confirming the diagnosis. The pathologic abnormality appears to be microvascular thrombosis with or without capillaritis. Treatment with corticosteroids usually leads to marked improvement.
Conclusion: Patients with APS may present with diffuse alveolar hemorrhage resulting in mild to life threatening symptoms. Prompt and thorough evaluation to confirm the diagnosis and treatment with corticosteroids usually leads to rapid improvement. The clinical setting will dictate whether other therapies such as immunosuppressive agents or intravenous immunoglobulin are required.