Department of Medicine, Weill Cornell Medicine, Hospital for Special Surgery, New York, NY, USA.
Department of Medicine, Division of Pulmonary and Critical Care, Weill Cornell Medicine, New York, NY, USA.
Curr Rheumatol Rep. 2019 Sep 6;21(10):56. doi: 10.1007/s11926-019-0852-7.
Diffuse alveolar hemorrhage (DAH) is a rare but devastating manifestation of antiphospholipid syndrome (APS) patients with or without other systemic autoimmune diseases. Data regarding diagnosis and treatment are limited to case series. We review diagnostic and therapeutic strategies employed in APS patients with DAH and discuss our experience in managing these complex patients.
Pulmonary capillaritis likely contributes to the pathogenesis, however is only observed in half of the biopsies. Corticosteroids induce remission in the majority of patients, however almost half recur and require a steroid-sparing immunosuppressive to maintain remission. Cyclophosphamide- or rituximab-based regimens achieve the highest remission rates (50%); other strategies include intravenous immunoglobulin, plasmapheresis, mycophenolate mofetil, and/or azathioprine. Given the rarity of DAH in APS, treatment is guided by interdisciplinary experience. Why certain patients achieve full remission with corticosteroids while others require immunosuppressive agents is unknown; future research should focus on the pathophysiology and optimal management.
弥漫性肺泡出血（DAH）是抗磷脂综合征（APS）患者（伴或不伴其他系统性自身免疫性疾病）的一种罕见但严重的表现。关于诊断和治疗的数据仅限于病例系列研究。我们回顾了伴有 DAH 的 APS 患者的诊断和治疗策略，并讨论了我们在管理这些复杂患者方面的经验。
肺毛细血管炎可能促成了发病机制，但仅在一半的活检中观察到。皮质类固醇在大多数患者中诱导缓解，但近一半的患者复发，需要类固醇维持缓解。基于环磷酰胺或利妥昔单抗的方案实现了最高的缓解率（50%）；其他策略包括静脉注射免疫球蛋白、血浆置换、霉酚酸酯和/或硫唑嘌呤。鉴于 DAH 在 APS 中的罕见性，治疗是由多学科经验指导的。为什么某些患者用皮质类固醇就能完全缓解，而其他患者则需要免疫抑制剂，原因尚不清楚；未来的研究应集中在病理生理学和最佳治疗上。
