A 51year‐old male presented to medical ICU with hemoptysis, acute blood loss anemia and acute hypoxic respiratory distress. Bronchoscopy with transbronchial biopsy revealed diffuse alveolar hemorrhage (DAH) with bronchoalveolar lavage negative for infectious etiology. Serum ANCA was negative clinical and serological criteria for systemic lupus erythematous was not met. He was, however, positive for lupus anticoagulant. He was diagnosed with diffuse pulmonary hemorrhage secondary to pulmonary capillaritis from antiphospholipid syndrome (APS). The patient was treated with high dose intravenous steroids, cyclophosphamide, plasmapheresis, and intravenous immunoglobulins (IVIG) with excellent response. Hemostasis was achieved and he never required intubation. He was discharged on 5 Liters/minute supplemental oxygen via nasal cannula with oral prednisone, plans for continued monthly cyclophosphamide.
Review of literature revealed only twenty‐one cases of DAH associated with APS and only two cases where it was the initial presentation. Although mainstay treatment is high dose steroids, the roles of cyclophosphamide, plasmapheresis and IVIG have not been well researched and is still implemented on a case by case basis depending on severity of disease.
This case presents a rare bleeding manifestation of APS, which classically and conversely involves thrombosis. Although less likely a cause compared to other vasculitides (i.e. Wegener’s granulomatosis and SLE), APS should be considered in the differential diagnosis of DAH. Additionally, it adds to the current understanding of treatment of APS associated DAH. Treatment in this patient with high dose steroids, cyclophosphamide, IVIG and plasmapheresis was successful in controlling the pulmonary capillaritis and preventing worsening of diffuse alveolar hemorrhage.
To cite this abstract:Vo P, Saksena S, Bhowansingh R. Diffuse Alveolar Hemorrhage Due to Pulmonary Capillaritis As Initial Presentation of Antiphospholipid Syndrome. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 668. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/diffuse-alveolar-hemorrhage-due-to-pulmonary-capillaritis-as-initial-presentation-of-antiphospholipid-syndrome/. Accessed April 4, 2020.