局限性皮肤型系统性硬化症患者的复发性闭塞性细支气管炎伴机化性肺炎

Recurrent bronchiolitis obliterans organizing pneumonia in a patient with limited cutaneous systemic sclerosis.

作者信息

Shimizu Yuji, Tsukagoshi Hideo, Nemoto Toshikazu, Honma Manabu, Nojima Yoshihisa, Mori Masatomo

机构信息

Department of Internal Medicine, Nanokaichi Municipal Hospital, 643 Nanokaichi, Tomioka, Gunma 370-2343, Japan.

出版信息

Rheumatol Int. 2002 Sep;22(5):216-8. doi: 10.1007/s00296-002-0230-y. Epub 2002 Jul 27.

DOI:10.1007/s00296-002-0230-y

PMID:12215870

Abstract

Systemic sclerosis (SSc) is a generalized disorder characterized by fibrosis and vascular obliteration in the skin, lung, gastrointestinal tract, and kidney. One of its two subsets is a stable, limited cutaneous group (lSSc). Pulmonary involvement in scleroderma is common, and several types of pulmonary disorders are associated with SSc. Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare finding in lung disorders associated with SSc. We describe a case of lSSc with BOOP that was responsive to steroid therapy. Of interest is that the lung disorders appeared in different periods and areas. It might be important to diagnose abnormal shadows in lung fields before treatment of patients with SSc.

摘要

系统性硬化症（SSc）是一种全身性疾病，其特征为皮肤、肺、胃肠道和肾脏出现纤维化和血管闭塞。它的两个亚型之一是稳定的局限性皮肤型（lSSc）。硬皮病患者肺部受累很常见，多种类型的肺部疾病与SSc相关。闭塞性细支气管炎伴机化性肺炎（BOOP）在与SSc相关的肺部疾病中是一种罕见的表现。我们报告一例lSSc合并BOOP且对类固醇治疗有效的病例。有趣的是，肺部疾病出现在不同时期和不同部位。在对SSc患者进行治疗前诊断肺野异常阴影可能很重要。