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Primary antiphospholipid syndrome-associated diffuse alveolar hemorrhage
Rodrigo Cartin-Ceba1, Tobias Peikert, Aneel Ashrani
1Mayo Clinic, Rochester, Minnesota.
Arthritis Care & Research
|August 29, 2013
Summary
Diffuse alveolar hemorrhage (DAH) in primary antiphospholipid syndrome (APS) is rare and serious. Immunosuppression with cyclophosphamide or rituximab offers the best chance for remission in APS-DAH patients.
Area of Science:
- Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Diffuse alveolar hemorrhage (DAH) is an uncommon but severe complication of primary antiphospholipid syndrome (APS).
- Understanding the clinical course and treatment efficacy for APS-associated DAH is crucial due to its rarity and poor prognosis.
Purpose of the Study:
- To describe the clinical characteristics, treatment strategies, and patient outcomes of primary APS-associated DAH.
- To identify effective therapeutic options for this rare condition.
Main Methods:
- Retrospective review of adult patients with primary APS-associated DAH at a single center over 15 years.
- DAH diagnosis confirmed by bilateral pulmonary infiltrates and bronchoalveolar lavage (BAL) findings.
- Exclusion of other known causes of DAH.
Main Results:
- Eighteen patients with primary APS-associated DAH were identified.
- Treatment with cyclophosphamide (CYC) or rituximab (RTX) showed the highest remission rates, alone or in combination.
- High mortality (6/18) due to uncontrolled DAH or treatment complications, with limited efficacy of other therapies like MMF, azathioprine, plasma exchange, or IVIg.
Conclusions:
- Primary APS-associated DAH has a poor prognosis with limited therapeutic options.
- Early consideration of immunosuppression with CYC or RTX is associated with the highest likelihood of remission.
- This case series represents the largest reported cohort, highlighting the need for effective management strategies.
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