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[嗜酸性筋膜炎]

[Eosinophilic fasciitis].

作者信息

Niklas Karolina, Niklas Arkadiusz, Puszczewicz Mariusz

机构信息

Katedra i Klinika Reumatologii i Chorób Wewnętrznych Uniwersytetu Medycznego w Poznaniu.

Klinika Hipertensjologii, Angiologii i Chorób Wewnętrznych Uniwersytetu Medycznego w Poznaniu.

出版信息

Postepy Hig Med Dosw (Online). 2015 Jan 2;69:488-95. doi: 10.5604/17322693.1149872.

Abstract

Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is characterized by fibrosis of the skin and subcutaneous tissues with significant thickening of fascia. Visceral involvement is rare. Characteristic feature in laboratory tests is peripheral blood eosinophilia. Differential diagnosis should be performed, including ruling out systemic sclerosis, nephrogenic systemic fibrosis, eosinophilia-myalgia syndrome, scleromyxedema, hypereosinophilic syndrome or Churg-Strauss syndrome. Final diagnosis is confirmed by histopathological examination. In treatment of the disease corticosteroids and/or immunosuppressive drugs are used. Some other drugs showed activity in this disease e.g. dapsone, infiximab or rituximab. Prognosis is rather good but sometimes a long-term treatment is necessary. In this paper we summarized the current knowledge on eosinophilic fasciitis.

摘要

嗜酸性筋膜炎是一种病因和发病机制不明的罕见结缔组织病。它被归类为硬皮病样综合征。该病的特征是皮肤和皮下组织纤维化,伴有筋膜明显增厚。内脏受累罕见。实验室检查的特征性表现是外周血嗜酸性粒细胞增多。应进行鉴别诊断,包括排除系统性硬化症、肾源性系统性纤维化、嗜酸性粒细胞增多性肌痛综合征、硬化性黏液水肿、高嗜酸性粒细胞综合征或Churg-Strauss综合征。最终诊断通过组织病理学检查得以证实。治疗该病使用皮质类固醇和/或免疫抑制药物。其他一些药物在该病中显示出活性,如氨苯砜、英夫利昔单抗或利妥昔单抗。预后相当好,但有时需要长期治疗。在本文中,我们总结了关于嗜酸性筋膜炎的当前知识。

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