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特发性肉芽肿性血管炎:对免疫抑制治疗的反应

Idiopathic granulomatous vasculitis: response to immunosuppressive therapy.

作者信息

Alguacil-Garcia G F, Moreno-Requena J, Martinez-Albadalejo M, Hallal-Hachem H, Gonzalez-Pina B, de Paco-Moya M

机构信息

Hospital Comarcal del Noroeste, Caravaca de la Cruz, Murcia, Spain.

出版信息

J Clin Pathol. 1995 Jun;48(6):579-82. doi: 10.1136/jcp.48.6.579.

Abstract

A case of idiopathic granulomatous vasculitis (disseminated visceral giant cell arteritis) is described in an old woman, the seventh case of this rare disorder reported to date. The main organ affected was the liver and, to our knowledge, this is the first patient to be diagnosed while still alive and the only case to have received medical treatment. It is also the first time that muscular involvement has been documented in this condition. Cyclophosphamide treatment resulted in disappearance of symptoms and increase in weight. The patient died of an unrelated condition.

摘要

本文描述了一位老年女性特发性肉芽肿性血管炎(播散性内脏巨细胞动脉炎)的病例,这是迄今为止报道的该罕见疾病的第七例。主要受累器官为肝脏,据我们所知,这是首例在生前被诊断出且唯一接受过药物治疗的患者。这也是首次有文献记载该疾病出现肌肉受累的情况。环磷酰胺治疗使症状消失且体重增加。患者死于其他 unrelated condition(此处原文有误,应是“无关疾病”之类的表述,假设为“无关疾病”)。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e891/502695/2558f67fe90f/jclinpath00231-0090-a.jpg

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