摘要
IgG4相关性疾病是一个新的独立疾病,临床表现复杂多样,可以浸润多种器官,出现器官弥漫性肿胀或局部肿块形成,病理可见IgG4+浆细胞为主的淋巴浆细胞浸润、闭塞性静脉炎、组织席纹状纤维化改变,血清IgG4浓度升高。此病多见于老年男性,对类固醇激素反应敏感。临床常见的受累器官有:浸润唾液腺、泪腺的米库利兹病,浸润下颌下腺的Küttner肿瘤,Riedel’s甲状腺炎,腹膜后纤维化,自身免疫性胰腺炎1型等全身多系统疾病。本文从概念,流行病学概况、发病机制,病理特征,临床表现,诊断和治疗作一综述。
IgG4-related systemic disease（ IgG4-RD） is a newly novel clinical disease entity, multiorgan involvement,which shows organ enlargement or nodular / hyperplastic lesions in various organs concurrently or metachronously. Pathological features diffuse organ swelling or focal mass formation,obliterative phlebitis,whirlshaped fibrosis with a lymphoplasmacytic infiltrate rich in IgG4-bearing plasma cells,as well as elevated levels of serum IgG4. It responds well 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 and autoimmune pancreatitis type 1 are now regarded to be manifestations of this systemic disease. This article summarized the concept,an overview of the epidemiology,pathogenesis characteristics,clinical presentation,proposed diagnostic approaches and treatment options.
出处
《标记免疫分析与临床》
CAS
2016年第3期334-338,共5页
Labeled Immunoassays and Clinical Medicine
