Harne Prateek, Sampat Parth J, Bisen Maneesh, BenGabr Jihad, Neupane Hom
Internal Medicine, State University of New York (SUNY) Upstate University Hospital, Syracuse, USA.
Rheumatology, State University of New York (SUNY) Upstate University Hospital, Syracuse, USA.
Cureus. 2020 Sep 16;12(9):e10482. doi: 10.7759/cureus.10482.
Antiphospholipid antibody syndrome (APS) is a multisystem disorder characterized by thromboembolic events in the presence of antiphospholipid antibodies (APLA). Catastrophic antiphospholipid antibody syndrome (CAPS) is an uncommon variant of APS which is associated with widespread coagulopathy that predominantly affects small vessels. Despite maximal treatment, CAPS has a very high mortality rate. We present a case of a 42-year-old woman with a history of APS who presented to our hospital with complaints of epistaxis, hemoptysis, menorrhagia, and shortness of breath. She was diagnosed with CAPS and developed multiorgan failure and sepsis. Despite maximal treatment with immune modulators, she unfortunately succumbed. With this case, we highlight the importance of early recognition of CAPS and review various treatment modalities that have been proven beneficial. Despite these modalities, CAPS remains a therapeutic challenge in many cases and has a high mortality rate.
抗磷脂抗体综合征（APS）是一种多系统疾病，其特征是在存在抗磷脂抗体（APLA）的情况下发生血栓栓塞事件。灾难性抗磷脂抗体综合征（CAPS）是APS的一种罕见变体，与主要影响小血管的广泛凝血病有关。尽管进行了最大程度的治疗，CAPS的死亡率仍然很高。我们报告一例42岁有APS病史的女性，她因鼻出血、咯血、月经过多和呼吸急促前来我院就诊。她被诊断为CAPS，并发展为多器官功能衰竭和脓毒症。尽管使用免疫调节剂进行了最大程度的治疗，但她不幸去世。通过这个病例，我们强调了早期识别CAPS的重要性，并回顾了各种已被证明有益的治疗方法。尽管有这些方法，但在许多情况下，CAPS仍然是一个治疗挑战，并且死亡率很高。
