Survival After Catastrophic Antiphospholipid Syndrome With Diffuse Alveolar Hemorrhage.

IF 0.7 Q3 MEDICINE, GENERAL & INTERNAL Journal of investigative medicine high impact case reports Pub Date : 2026-01-01 Epub Date: 2026-06-24 DOI:10.1177/23247096261455115
Edy Quizhpe, Franklin Uguña, Javier Velastegui, José Luis Santana, Nelson Moran, Miguel Ochoa, Alex Robalino
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Abstract

Antiphospholipid syndrome is a systemic autoimmune disorder characterized by thrombotic events in the presence of antiphospholipid antibodies including lupus anticoagulant, anticardiolipin, and anti-β2-glycoprotein I antibodies. Catastrophic antiphospholipid syndrome is a rare but life-threatening variant, accounting for less than 1% of antiphospholipid syndrome cases, and is characterized by rapid progressive microvascular thrombosis and multiorgan failure. We report the case of a middle-aged man who developed catastrophic antiphospholipid syndrome associated with systemic lupus erythematosus, presenting with diffuse alveolar hemorrhage, acute kidney injury, vasculitis skin lesions, and severe thrombocytopenia. Management requires intensive care support, immunosuppressive therapy, intravenous immunoglobulin administration, cyclophosphamide administration, and delayed plasmapheresis. Despite multi-organ involvement and guarded prognosis, the patient survived following multidisciplinary management. This case highlights the importance of early recognition, rapid therapeutic intervention, and coordinated interdisciplinary care to improve survival in patients with catastrophic antiphospholipid syndrome.

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灾难性抗磷脂综合征伴弥漫性肺泡出血后的生存率。
抗磷脂综合征是一种系统性自身免疫性疾病,其特征是在抗磷脂抗体存在时发生血栓事件,包括狼疮抗凝血剂、抗心磷脂和抗β2-糖蛋白I抗体。灾难性抗磷脂综合征是一种罕见但危及生命的变体,占抗磷脂综合征病例的不到1%,其特征是快速进行性微血管血栓形成和多器官衰竭。我们报告一例中年男子谁发展灾难性抗磷脂综合征与系统性红斑狼疮,表现为弥漫性肺泡出血,急性肾损伤,血管炎皮肤病变,和严重的血小板减少症。治疗需要重症监护支持、免疫抑制治疗、静脉注射免疫球蛋白、环磷酰胺和延迟血浆置换。尽管多器官受累和预后谨慎,患者在多学科治疗后存活。该病例强调了早期识别、快速治疗干预和协调跨学科护理对提高灾难性抗磷脂综合征患者生存率的重要性。
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来源期刊
CiteScore
1.90
自引率
0.00%
发文量
165
审稿时长
12 weeks
期刊介绍: The AFMR is committed to enhancing the training and career development of our members and to furthering its mission to facilitate the conduct of research to improve medical care. Case reports represent an important avenue for trainees (interns, residents, and fellows) and early-stage faculty to demonstrate productive, scholarly activity.
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