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重叠结缔组织病综合征。

Overlap connective tissue disease syndromes.

机构信息

Division of Rheumatology, Department of Medicine, University of Padua, Italy.

出版信息

Autoimmun Rev. 2013 Jan;12(3):363-73. doi: 10.1016/j.autrev.2012.06.004. Epub 2012 Jun 26.

Abstract

Overlap Syndromes (OSs) have been defined as entities satisfying classification criteria of at least two connective tissue diseases (CTDs) occurring at the same or at different times in the same patient. CTDs include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), polymyositis/dermatomyositis (PDM), and Sjögren syndrome (SS). Every combination between these disorders has been reported. In some OS a specific autoantibody has been indentified, supporting the hypothesis that these syndromes are not a mere association of two or more CTD in the same patient, but a well defined clinical entity with specific clinical characteristics. As an example, anti-t-RNA synthetase syndrome is characterized by the presence of anti-t-RNA synthetase antibodies. Notably, clinical manifestations observed in OS may be different from those observed in the single CTD. The treatment of OS is mainly based on the use of corticosteroids and immunosuppressants. Biologic drugs, i.e. anti-TNFα or anti-CD20 monoclonal antibodies, have been recently introduced as alternative treatments in refractory cases. Moreover, there are some concerns with the use of anti-TNF agents in patients with systemic autoimmune diseases due to the risk of triggering disease exacerbations. In this paper the most frequent OS are described with a special focus on the specific immunologic and clinical aspects. Furthermore, some personal data on anti-t-RNA synthetase syndrome and rhupus syndrome are reported.

摘要

重叠综合征 (OSs) 被定义为同时或不同时在同一患者中满足至少两种结缔组织疾病 (CTDs) 分类标准的实体。CTDs 包括系统性红斑狼疮 (SLE)、类风湿关节炎 (RA)、系统性硬化症 (SSc)、多发性肌炎/皮肌炎 (PDM) 和干燥综合征 (SS)。这些疾病之间的每种组合都有报道。在一些 OS 中已经确定了特定的自身抗体,这支持了这些综合征不仅是同一患者中两种或多种 CTD 的简单关联,而是具有特定临床特征的明确临床实体的假说。例如,抗 tRNA 合成酶综合征的特征是存在抗 tRNA 合成酶抗体。值得注意的是,在 OS 中观察到的临床表现可能与在单一 CTD 中观察到的临床表现不同。OS 的治疗主要基于使用皮质类固醇和免疫抑制剂。生物药物,即抗 TNFα 或抗 CD20 单克隆抗体,最近已被引入作为难治性病例的替代治疗方法。此外,由于有引发疾病恶化的风险,在患有全身性自身免疫性疾病的患者中使用抗 TNF 药物存在一些担忧。本文描述了最常见的 OS,并特别关注其特定的免疫和临床方面。此外,还报告了一些关于抗 tRNA 合成酶综合征和 rhupus 综合征的个人数据。

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