Abstract Objectives Diffuse alveolar hemorrhage (DAH) is a rare and poorly understood manifestation of antiphospholipid (AP) syndrome (APS). This study describes the clinical presentation, treatment, and prognosis of DAH in APS with or without CAPS. Methods Retrospective multicentre French study includes all APS patients diagnosed according to the Sydney criteria with a history of definite DAH. Results Of the 26 patients included, 15 (58%) were female (median age, 45.5 years; 95%CI, 35–58.1); 23 (88%) patients had a history of thrombotic events (6 arterial), and 13 (50%) of CAPS. Twenty (77%) were triple-positive for APL antibodies, 5 (19%) had systemic lupus erythematosus, and DAH was inaugural for 7 (27%) patients. Twenty-two patients (85%) were treated with anticoagulants, 22 (85%) with steroids, and 8 (31%) with immunosuppressive therapy. Complete remission was achieved in 18 (69%) cases. DAH relapses occurred in 14 patients (54%) after a median of 1.8 (95% CI 1.1–3.6) years. Risk factors for DAH relapse were histories of arterial thrombosis (HR 4.6, 95%CI 1.2–17), TIA or stroke (HR 7.8, 95%CI 2.2–28), mechanical ventilation during DAH episode (HR 6.1, 95%CI 0.99–37) and triple APL positivity. Multivariate analysis showed a higher risk of DAH relapse in patients without (vs with) CAPS (HR 6.8, 95%CI 1.0–45.3; p = 0.048). Overall mortality was higher in patients with CAPS (7.7% at 1 year and 37.1% at 5 years); no patients without CAPS died. Conclusion The immediate prognosis of DAH, mainly treated with anticoagulants and steroids, was good. Patients with CAPS had a higher mortality rate in the long-term follow-up. Triple-positive and/or arterial phenotype patients more frequently experienced DAH relapses even when treated with anticoagulants.
Grobost et al. (Fri,) studied this question.