Department of Medicine A, University Medicine Greifswald, Ernst-Moritz-Arndt-University Greifswald, Germany.
United European Gastroenterol J. 2014 Jun;2(3):165-72. doi: 10.1177/2050640614532457.
IgG4-related systemic disease is a recently recognized systemic condition characterized by unique pathological features that can affect a variety of organs. It includes a growing number of medical conditions which have the following features in common: diffuse organ swelling or focal mass formation, sclerosing storiforme (whirl-shaped) fibrosis with a lymphoplasmacytic infiltrate rich in IgG4-bearing plasma cells, as well as elevated levels of serum IgG4. It invariably responds to steroid treatment and is mostly diagnosed in elderly men. Well-known syndromes like Mikulicz's disease of the salivary or lacrimal gland, Küttner's tumour of the submandibular gland, Riedel's thyroiditis, or retroperitoneal fibrosis, as well as novel entities such as autoimmune pancreatitis type 1, are now regarded to be manifestations of this systemic disease. This article provides an overview of the epidemiology, concepts of pathogenesis, clinical presentation, proposed diagnostic approaches, treatment options, and differential diagnosis of IgG4-related disease.
IgG4 相关系统性疾病是一种新认识的系统性疾病，其特征为独特的病理学特征，可影响多种器官。它包含了越来越多的具有以下共同特征的医学病症：弥漫性器官肿胀或局灶性肿块形成，富于 IgG4 阳性浆细胞的淋巴浆细胞浸润性硬化性席纹状（漩涡状）纤维化，以及血清 IgG4 水平升高。它始终对类固醇治疗有反应，且主要发生于老年男性。现在，众所周知的综合征，如唾液腺或泪腺的 Mikulicz 病、颌下腺的 Küttner 瘤、甲状腺炎的 Riedel 或腹膜后纤维化，以及新出现的实体，如 1 型自身免疫性胰腺炎，都被认为是这种系统性疾病的表现。本文概述了 IgG4 相关疾病的流行病学、发病机制概念、临床表现、提出的诊断方法、治疗选择和鉴别诊断。
