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坏死性结节病样肉芽肿病。一种罕见的肉芽肿性疾病。

Necrotising sarcoid granulomatosis. A rare granulomatous disease.

作者信息

Doubková Martina, Hausnerová Jitka, Výška Ondřej, Richter Svatopluk, Merta Zdeněk

机构信息

Department of Pulmonary Diseases and Tuberculosis, Masaryk University Faculty of Medicine and University Hospital, Brno, Czech Republic.

Institute of Pathological Anatomy, University Hospital, Brno, Czech Republic.

出版信息

Sarcoidosis Vasc Diffuse Lung Dis. 2018;35(4):395-398. doi: 10.36141/svdld.v35i4.7047. Epub 2020 Mar 9.

Abstract

Necrotizing sarcoid granulomatosis (NSG) is a very rare disease of unknown etiology characterized by sarcoid-like granulomas, vasculitis and necrosis in pulmonary and extrapulmonary localizations. We describe a case of a 34-year-old Caucasian male with fever, pleural pain, and nodular pulmonary opacities on chest radiograph. Histological examination of the lung tissue confirmed NSG. Diagnostically, infectious causes, vasculitis, and malignancy were excluded. A tendency to partial regression was observed, without the need for corticosteroid treatment. NSG is a rare disease which must be distinguished from other systemic diseases including vasculitides. The key to diagnosis, emphasized in our paper, is the histopathological finding. The course of NSG is similar to sarcoidosis. Corticosteroids are considered the treatment of choice, but the disease exhibits a tendency towards spontaneous regression. .

摘要

坏死性结节病样肉芽肿病(NSG)是一种病因不明的极为罕见的疾病,其特征为肺内及肺外部位出现结节病样肉芽肿、血管炎和坏死。我们描述了一例34岁的白种男性病例,该患者有发热、胸痛,胸部X线片显示肺部有结节状阴影。肺组织的组织学检查确诊为NSG。在诊断过程中,排除了感染性病因、血管炎和恶性肿瘤。观察到有部分消退的趋势,无需使用皮质类固醇治疗。NSG是一种罕见疾病,必须与包括血管炎在内的其他全身性疾病相鉴别。我们的论文强调,诊断的关键是组织病理学发现。NSG的病程与结节病相似。皮质类固醇被认为是首选治疗方法,但该疾病有自发消退的趋势。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0ed/7170123/0d86f9f6d868/SVDLD-35-395-g001.jpg

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