1Amna Ahmed Almuhairi,1Aneja Ritu, 1Diab, Sehriban
1Sheikh Shakhbout Medical City
Diffuse alveolar hemorrhage (DAH) is a rare, potentially fatal presentation of antiphospholipid syndrome (APS) that can be triggered by infections, toxins or other factors. Due to rarity of DAH in APS, no randomized trials exist, and treatment is guided by case reports, case series, and inter-disciplinary experience. This abstract will describe a case of DAH in a patient with primary APS.
Material(s) and Method(s):
Mr. X; a 40-year-old male, with known primary APS. His APS presented with two years history of chronic mild hemoptysis, episodes of amaurosis fugax twice, and triple positive anti-phospholipid antibodies (APLa). A bronchoalveolar lavage had been done one year prior to the presentation of Mr. X with the DAH, and it was inconclusive. He was on treatment with Mycophenolate Mofetil (MMF), Methotrexate (MTX) plus Folic acid, high dose Prednisolone, without anticoagulation. Mr. X ran out of MMF tablets 2 weeks prior to the DAH attack. The DAH episode started with acute hemoptysis and dyspnea which started 10 days after his recovery from asymptomatic COVID-19 infection. On physical examination, Mr. X was alert and afebrile, desaturating down to 85% on room air. There were no positive musculoskeletal findings. Investigations revealed leukocytosis, anemia, thrombocytopenia, and mildly elevated inflammatory markers. Chest radiography showed a new extensive bilateral patchy consolidation and ground-glass changes. There were no large pulmonary embolism or smaller embolism. Therefore, DAH could not exclude. Mr. X was put on supportive care, , Oxygen supplement, empirical antimicrobial therapy, pulse corticosteroid, and plasma exchange. After confirming negative septic workup, cyclophosphamide and IVIG had been added. Mr. X condition improved with the given treatment.
DAH is a rare microvascular manifestation of APS in which blood oozes from the alveolar capillaries into the intra-alveolar space. The frequency of APS-associated DAH was 2%, and the mortality rate among primary APS ranged between 23 to 33%. Viral URTI was reported as a trigger inciting episodes of alveolar hemorrhage. In literature, in addition to supportive care, high-dose corticosteroids are the initial treatment for DAH in APS. The majority of patients need an immunosuppressive steroid-sparing agent.