灾难性抗磷脂综合征的治疗
Treatment of catastrophic antiphospholipid syndrome.
作者信息
Kazzaz Nayef M, McCune W Joseph, Knight Jason S
机构信息
Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA.
出版信息
Curr Opin Rheumatol. 2016 May;28(3):218-27. doi: 10.1097/BOR.0000000000000269.
PURPOSE OF REVIEW
Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS.
RECENT FINDINGS
Data from the international 'CAPS registry', spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and precipitating diagnoses. Current guidelines also continue to support the role of anticoagulants and glucocorticoids as foundation therapy in all patients. Finally, new basic science and case series suggest that novel therapies, such as rituximab and eculizumab, warrant further study.
SUMMARY
Attention to associated diagnoses, such as infection and systemic lupus erythematosus (SLE), is critical at the time of diagnosis. All patients should be treated with anticoagulants, corticosteroids, and possibly plasma exchange. In patients with SLE, cyclophosphamide should be considered. In refractory or relapsing cases, new therapies, such as rituximab and possibly eculizumab, may be options, but need further study.
综述目的
灾难性抗磷脂综合征（CAPS）是抗磷脂综合征（APS）的一种严重表现形式。尽管仅影响1%的APS患者，但如果不及早识别和治疗，该病往往会致命。在此，我们将综述目前CAPS的诊断和治疗方法。
最新发现
由欧洲抗磷脂抗体论坛牵头的国际“CAPS登记处”的数据，增进了我们对高危患者、典型临床特征及诱发诊断的了解。当前指南也继续支持抗凝剂和糖皮质激素在所有患者中作为基础治疗的作用。最后，新的基础科学研究和病例系列表明，诸如利妥昔单抗和依库珠单抗等新型疗法值得进一步研究。
总结
在诊断时，关注相关诊断，如感染和系统性红斑狼疮（SLE）至关重要。所有患者均应接受抗凝剂、皮质类固醇治疗，可能还需进行血浆置换。对于SLE患者，应考虑使用环磷酰胺。在难治性或复发性病例中，诸如利妥昔单抗以及可能的依库珠单抗等新型疗法可能是选择，但需要进一步研究。
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