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IgG4相关性疾病——一种罕见的奥蒙德病多囊型

IgG4-related Diseases - A Rare Polycystic Form of Ormond's Disease.

作者信息

Průcha Miroslav, Czinner Petr, Prokopová Petra

机构信息

Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, Prague, Czech Republic.

Department of Vascular Surgery, Na Homolce Hospital, Prague, Czech Republic.

出版信息

Prague Med Rep. 2016;117(2-3):124-128. doi: 10.14712/23362936.2016.13.

Abstract

Currently, Ormond's disease is classified among IgG4-associated diseases. Its clinical manifestation varies and is characterized by the presence of fibrous retroperitoneal tissue that often affects the ureters or abdominal aorta and iliac arteries. We present a unique case of the polycystic form of Ormond's disease, imitating tumour in the retroperitoneal space. At the time of diagnosis, the disease was not metabolically active and did not require immunosuppressive therapy. The polycystic mass was removed surgically. There has been no exacerbation of the disease during the last 12 months.

摘要

目前,奥蒙德病被归类为IgG4相关性疾病。其临床表现各异,特征为纤维性腹膜后组织的存在,常累及输尿管或腹主动脉及髂动脉。我们报告一例罕见的多囊型奥蒙德病病例,其在腹膜后间隙类似肿瘤。诊断时,该疾病无代谢活性,无需免疫抑制治疗。多囊肿块已通过手术切除。在过去12个月中,疾病未复发。

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