Refractory Pulmonary Hemorrhage in Primary Antiphospholipid Syndrome: Unmet Need for More Effective Therapies

Pulmonary hemorrhage is a rare but life-threatening microvascular complication of antiphospholipid syndrome (APS). Despite aggressive immunosuppressive therapy, its prognosis remains guarded because of high rates of non-response to treatment and relapse, leading to increased mortality. We report the case of a 24-year-old Chinese woman with primary APS who developed recurrent diffuse pulmonary hemorrhage (DAH), in addition to superior vena cava thrombosis and myocardial infarction with normal coronary arteries. Her pulmonary hemorrhage was refractory to high-dose glucocorticoid therapy and anticoagulation but stabilized after escalation of treatment with a combination of rituximab and cyclophosphamide. In this report, the current evidence on the pathophysiology, treatment, and challenges of APS-associated DAH is reviewed and discussed.