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一种新型重叠综合征:系统性硬化症合并抗磷脂综合征——病例系列

A novel overlap syndrome: systemic sclerosis associated with antiphospholipid syndrome--a case series.

作者信息

Zandman-Goddard Gisele, Tweezer-Zaks Nurit, Shalev Tamar, Levy Yair, Ehrenfeld Michael, Langevitz Pnina

机构信息

Department of Medicine C, Wolfson Medical Center, Holon, Israel.

出版信息

Ann N Y Acad Sci. 2007 Jun;1108:497-504. doi: 10.1196/annals.1422.053.

Abstract

Antiphospholipid syndrome (APS) can be either primary or secondary to autoimmune diseases, malignancies, infectious diseases, or drug-induced conditions. The aim of this study was to describe a novel overlap syndrome of APS and systemic sclerosis (SSc) in a case series. A retrospective review of medical files of hospitalized patients who were followed in two rheumatology clinics in Israel for the diagnosis of SSc and APS was sought. A MEDLINE search was performed for reports of APS/SSc overlap syndrome. Five patients with the overlap syndrome of APS and SSc were retrieved. The diagnosis of both diseases was confirmed by the American College of Rheumatology classification criteria. Four patients were women and of an older age group (42-68 years old). Three patients had primary APS, and in two patients APS was secondary to SSc. Two of the five patients died. The interval between APS and SSc was < 1-18 years. APS/SSc overlap syndrome is described for the first time as a case series. The patients may be older, with an interval of up to 18 years between diseases. The APS patients did not suffer from SLE. The overlap syndrome was characterized in certain instances with severe disease and two patients died. With relevant clinical manifestations, APS should be sought in SSc patients and treated appropriately.

摘要

抗磷脂综合征(APS)可以是原发性的,也可以继发于自身免疫性疾病、恶性肿瘤、感染性疾病或药物诱发的疾病。本研究的目的是在一个病例系列中描述一种新型的APS与系统性硬化症(SSc)重叠综合征。我们对在以色列两家风湿病诊所接受随访以诊断SSc和APS的住院患者的病历进行了回顾性研究。我们在MEDLINE上搜索了关于APS/SSc重叠综合征的报告。检索到5例APS与SSc重叠综合征患者。两种疾病的诊断均通过美国风湿病学会分类标准得以证实。4例患者为女性,年龄较大(42 - 68岁)。3例患者患有原发性APS,2例患者的APS继发于SSc。5例患者中有2例死亡。APS与SSc之间的间隔时间为<1 - 18年。首次以病例系列形式描述了APS/SSc重叠综合征。患者可能年龄较大,疾病之间的间隔时间长达18年。APS患者未患系统性红斑狼疮(SLE)。在某些情况下,重叠综合征的特征为病情严重,有2例患者死亡。对于有相关临床表现的SSc患者,应排查APS并给予适当治疗。

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