Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Catalonia, Spain
Autoimmun Rev. 2011 Sep;10(11):664-8. doi: 10.1016/j.autrev.2011.04.031. Epub 2011 May 1.
The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. Refractory catastrophic APS may be defined as patients who did not respond to first-line therapies (anticoagulation, glucocorticoids and plasma exchange and/or intravenous immunoglobulins) and died in the acute phase of the episode or patients with recurrent episodes of catastrophic APS. The purpose of this review is to focus on the current management of these refractory patients and some of the potential new therapeutic approaches.
灾难性抗磷脂综合征（APS）是 APS 中最严重的形式，具有急性多器官受累和小血管血栓形成。难治性灾难性 APS 可定义为对一线治疗（抗凝、糖皮质激素和血浆置换和/或静脉注射免疫球蛋白）无反应且在发作急性期死亡的患者，或反复发作灾难性 APS 的患者。本文旨在关注这些难治性患者的当前治疗方法和一些潜在的新治疗方法。
