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[一例年轻患者出现晚期囊性和纤维化改变的结节病病例]

[A case of sarcoidosis with advanced cystic and fibrotic changes in a young patient].

作者信息

Tamura M, Hayashi T, Nagatomo H, Yoshii C, Tao Y, Nikaido Y, Nagata N, Kido M

机构信息

Division of Respiratory Disease, University of Occupational and Environmental Health, School of Medicine, Kitakyushu, Japan.

出版信息

Nihon Kyobu Shikkan Gakkai Zasshi. 1995 Mar;33(3):358-62.

PMID:7739183
Abstract

A 29-year-old man was referred to our hospital because of exertional dyspnea and progressive eruption on the buttocks and the lower extremities. Chest roentgenograms and computed tomograms taken at that time revealed diffuse fibrotic changes accompanied by multiple cavities and bullae in the lungs. There were no signs of mediastinal or hilar lymphadenopathy. A chest roentgenogram taken 7 years before admission showed no abnormalities. Serum ACE and lysozyme levels were high: 29.9 IU/l and 14.1 micrograms/ml, respectively. 67Ga scintigraphy showed diffuse uptake in both lung fields. The PPD skin test was negative, and repeated sputum smears and cultures were negative for pyogenic bacteria and acid-fast bacilli. Examination of transbronchial lung biopsy and skin biopsy specimens confirmed the diagnosis--they showed noncaseating epithelioid granulomas with giant cells and a negative reaction of the stain to acid-fast bacilli, which are compatible with sarcoidosis. The patient was given 30 mg/d of prednisone orally. The dyspnea and eruption were clearly alleviated, although there was little roentgenographic regression of cystic or fibrotic changes. There have been only a few reports of cystic and fibrotic changes early in the course of sarcoidosis. The cystic lesions in this case were probably secondary pulmonary cavities caused by the contracting and obstructive changes related to pulmonary fibrosis.

摘要

一名29岁男性因劳力性呼吸困难以及臀部和下肢出现进行性皮疹而转诊至我院。当时拍摄的胸部X线片和计算机断层扫描显示肺部有弥漫性纤维化改变,并伴有多个空洞和肺大疱。无纵隔或肺门淋巴结肿大的迹象。入院前7年拍摄的胸部X线片未见异常。血清血管紧张素转换酶(ACE)和溶菌酶水平升高,分别为29.9 IU/l和14.1微克/毫升。镓-67闪烁扫描显示双肺野弥漫性摄取。结核菌素皮肤试验阴性,多次痰涂片和培养未发现化脓性细菌和抗酸杆菌。经支气管肺活检和皮肤活检标本检查确诊——标本显示有非干酪样上皮样肉芽肿伴巨细胞,抗酸杆菌染色阴性,符合结节病。给予患者口服泼尼松30毫克/天。呼吸困难和皮疹明显缓解,尽管囊性或纤维化改变在影像学上几乎没有消退。关于结节病病程早期出现囊性和纤维化改变的报道很少。本例中的囊性病变可能是由与肺纤维化相关的收缩和阻塞性改变引起的继发性肺空洞。

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