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Antiphospholipid antibodies-associated diffuse alveolar hemorrhage
Ralph Yachoui1, Rahul Sehgal1, Barkha Amlani2
1Department of Rheumatology, Marshfield Clinic, Marshfield, 1000 N Oak Ave, WI 54449.
Seminars in Arthritis and Rheumatism
|December 8, 2014
Summary
Diffuse alveolar hemorrhage (DAH) is a rare complication of primary antiphospholipid syndrome (PAPS) and antiphospholipid antibodies (aPL). Early diagnosis and treatment are crucial for managing DAH in these patients, potentially improving outcomes.
Area of Science:
- Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Primary antiphospholipid syndrome (PAPS) and antiphospholipid antibodies (aPL) are associated with various thrombotic and obstetric events.
- Diffuse alveolar hemorrhage (DAH) is a rare but serious pulmonary manifestation that can occur in patients with PAPS or aPL.
- Understanding the clinical presentation and outcomes of DAH in this population is crucial for timely diagnosis and management.
Purpose of the Study:
- To delineate the clinical features and outcomes of patients experiencing diffuse alveolar hemorrhage (DAH) in the context of primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL).
- To identify pathological findings and treatment strategies associated with DAH in this patient cohort.
- To assess the long-term prognosis and remission rates for DAH in patients with PAPS or aPL.
Main Methods:
- A retrospective review of medical records was conducted for patients diagnosed with PAPS-associated DAH or aPL-associated DAH between January 1, 1997, and December 31, 2013.
- Data collected included clinical features, laboratory results, radiographic findings, management approaches, and patient outcomes.
- Surgical lung biopsies were analyzed for pathological characteristics, including bland hemorrhage and pulmonary capillaritis.
Main Results:
- Seventeen patients met the criteria for DAH with aPL; 10 had PAPS-associated DAH. The average age at diagnosis was 57.6 years, with secondary causes of DAH excluded.
- Lung biopsies revealed bland hemorrhage in 5 of 6 cases; pulmonary capillaritis was noted in only one case.
- Fifty-nine percent (10 patients) achieved complete and sustained remission after a median follow-up of 48 months. Mortality was 23% (4 patients), primarily due to uncontrolled DAH. Relapse occurred in 18% of patients.
Conclusions:
- Diffuse alveolar hemorrhage (DAH) represents a rare but significant complication in individuals with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL).
- Pathological examination of lung tissue typically shows bland hemorrhage rather than capillaritis.
- Early recognition and prompt therapeutic intervention are essential for potentially improving the clinical outcomes of this uncommon complication.
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