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硬皮病和毛细血管扩张症日本患者的临床和实验室特征。

Clinical and laboratory features of Japanese patients with scleroderma and telangiectasia.

机构信息

Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.

出版信息

Clin Exp Dermatol. 2009 Oct;34(7):781-3. doi: 10.1111/j.1365-2230.2008.03148.x.

Abstract

BACKGROUND

Systemic sclerosis (SSc) is a connective tissue disease characterized by sclerotic changes of the skin and internal organs. Telangiectasia is a frequent complication of patients with SSc.

OBJECTIVE

To examine the prevalance of telangiectasia in patients with SSc and investigate the clinical and laboratory features of patients with SSc and telangiectasia.

METHODS

In total, 211 patients with SSc who fulfilled the diagnostic criteria for SSc of the American College of Rheumatology were examined by laboratory and clinical methods. The average of disease duration time was 7.4 years.

RESULTS

Telangiectasia was found in 119 of the 211 patients (56%) with SSc. The prevalence of oesophageal involvement, decreased diffusing capacity for carbon monoxide (DLCO), heart involvement, calcinosis, shortening of the sublingual frenulum, or pitting scars was significantly greater in patients with telangiectasia than in those without telangiectasia.

CONCLUSION

Our study suggests that the presence of telangiectasia may be a marker of oesophageal involvement, decreased DLCO, and heart involvement.

摘要

背景

系统性硬化症(SSc)是一种以皮肤和内脏器官硬化性改变为特征的结缔组织疾病。毛细血管扩张症是 SSc 患者的常见并发症。

目的

检查 SSc 患者中毛细血管扩张症的患病率,并研究 SSc 伴毛细血管扩张症患者的临床和实验室特征。

方法

共检查了 211 例符合美国风湿病学会 SSc 诊断标准的 SSc 患者,采用实验室和临床方法。疾病平均持续时间为 7.4 年。

结果

在 211 例 SSc 患者中,发现 119 例(56%)有毛细血管扩张症。有毛细血管扩张症的患者食管受累、一氧化碳弥散量(DLCO)降低、心脏受累、钙沉积、舌下系带缩短或凹陷性瘢痕的患病率明显高于无毛细血管扩张症的患者。

结论

本研究表明,存在毛细血管扩张症可能是食管受累、DLCO 降低和心脏受累的标志。

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