Ochoa Minerva Lazos, López Belem Gabiño, Cabello Raúl Romero, Feregrino Raúl Romero
Department of Pathology, Hospital General de México, Mexico City, Mexico DF, Mexico.
BMJ Case Rep. 2013 May 2;2013:bcr2013009636. doi: 10.1136/bcr-2013-009636.
IgG4-related disease (IgG4RD) is a chronic recurring fibro-inflammatory pathology that is considered to be of autoimmune origin. Histopathology is considered to be the gold standard method for diagnosis. IgG4RD affects multiple organs. IgG4RD was first identified in the pancreas and was called autoimmune pancreatitis (AIP). During the following years, the disease spectrum was expanded and it was realised that the extrapancreatic lesions can precede, coexist or appear after the diagnosis of AIP. At present, several illnesses such as Mikulicz disease, Küttner tumour, multifocal fibrosclerosis, etc, are considered to be part of the IgG4RD spectrum. The symptoms of the disease tend to appear over months and years and diagnosis is achieved on average 13.5 months (4-60 months) after the onset. The purpose of this report was to provide information about a case that was sadly fatal but that permitted a complete histopathological study of the damaged tissues.
IgG4相关性疾病（IgG4RD）是一种慢性复发性纤维炎症性病变，被认为起源于自身免疫。组织病理学被认为是诊断的金标准方法。IgG4RD可累及多个器官。IgG4RD最初在胰腺中被发现，当时被称为自身免疫性胰腺炎（AIP）。在随后的几年里，疾病谱不断扩大，人们意识到胰腺外病变可能在AIP诊断之前、同时或之后出现。目前，诸如米库利奇病、库特纳瘤、多灶性纤维硬化症等多种疾病被认为是IgG4RD谱的一部分。该病症状往往在数月甚至数年中逐渐显现，平均在发病后13.5个月（4 - 60个月）得以确诊。本报告的目的是提供一个令人遗憾的致命病例的信息，但该病例使得对受损组织进行了完整的组织病理学研究。
