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[系统性硬化症]

[Systemic scleroderma].

作者信息

Hunzelmann N

机构信息

Klinik und Poliklinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln.

出版信息

Hautarzt. 2013 Apr;64(4):299-310; quiz 311-2. doi: 10.1007/s00105-013-2537-x.

Abstract

Systemic scleroderma is a chronic autoimmune disease affecting the skin, internal organs and the musculoskeletal system. The presence of Raynaud phenomenon, anti-nuclear antibodies and pathologic capillaroscopy are early signs of the disease. Limited cutaneous SSc, diffuse cutaneous SSc and SSc-overlap syndromes are the main clinical subtypes. Multidisciplinary care is mandatory. Follow-up examinations should be performed at least annually in order to recognize in a timely fashion treatable organ involvement such as pulmonary arterial hypertension. Besides symptomatic treatment of organ involvement, immunosuppressive therapy is indicated for a progressive inflammatory course.

摘要

系统性硬化症是一种影响皮肤、内脏器官和肌肉骨骼系统的慢性自身免疫性疾病。雷诺现象、抗核抗体和病理毛细血管镜检查是该疾病的早期体征。局限性皮肤型系统性硬化症、弥漫性皮肤型系统性硬化症和系统性硬化症重叠综合征是主要的临床亚型。多学科护理是必不可少的。应至少每年进行一次随访检查,以便及时识别可治疗的器官受累情况,如肺动脉高压。除了对器官受累进行对症治疗外,对于进行性炎症病程,还需进行免疫抑制治疗。

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