Kleger Alexander, Seufferlein Thomas, Wagner Martin, Tannapfel Andrea, Hoffmann Thomas K, Mayerle Julia
Dtsch Arztebl Int. 2015 Feb 20;112(8):128-35. doi: 10.3238/arztebl.2015.0128.
IgG4-associated autoimmune diseases are systemic diseases affecting multiple organs of the body. Autoimmune pancreatitis, with a prevalence of 2.2 per 100,000 people, is one such disease. Because these multi-organ diseases present in highly variable ways, they were long thought just to affect individual organ systems. This only underscores the importance of familiarity with these diseases for routine clinical practice.
This review is based on pertinent articles retrieved by a selective search in PubMed, and on the published conclusions of international consensus conferences.
The current scientific understanding of this group of diseases is based largely on case reports and small case series; there have not been any randomized controlled trials (RCTs) to date. Any organ system can be affected, including (for example) the biliary pathways, salivary glands, kidneys, lymph nodes, thyroid gland, and blood vessels. Macroscopically, these diseases cause diffuse organ swelling and the formation of pseudotumorous masses. Histopathologically, they are characterized by a lymphoplasmacytic infiltrate with IgG4-positive plasma cells, which leads via an autoimmune mechanism to the typical histologic findings--storiform fibrosis ("storiform" = whorled, like a straw mat) and obliterative, i.e., vessel-occluding, phlebitis. A mixed Th1 and Th2 immune response seems to play an important role in pathogenesis, while the role of IgG4 antibodies, which are not pathogenic in themselves, is still unclear. Glucocorticoid treatment leads to remission in 98% of cases and is usually continued for 12 months as maintenance therapy. Most patients undergo remission even if untreated. Steroid-resistant disease can be treated with immune modulators.
IgG4-associated autoimmune diseases are becoming more common, but adequate, systematically obtained data are now available only from certain Asian countries. Interdisciplinary collaboration is a prerequisite to proper diagnosis and treatment. Treatment algorithms and RCTs are needed to point the way to organ-specific treatment in the future.
IgG4相关性自身免疫性疾病是影响身体多个器官的系统性疾病。自身免疫性胰腺炎就是其中一种，其患病率为每10万人中有2.2例。由于这些多器官疾病的表现高度多变，长期以来人们一直认为它们仅影响单个器官系统。这凸显了在常规临床实践中熟悉这些疾病的重要性。
本综述基于在PubMed中通过选择性检索获得的相关文章以及国际共识会议已发表的结论。
目前对这组疾病的科学认识很大程度上基于病例报告和小病例系列；迄今为止尚未有任何随机对照试验（RCT）。任何器官系统都可能受到影响，包括（例如）胆道、唾液腺、肾脏、淋巴结、甲状腺和血管。从宏观上看，这些疾病会导致器官弥漫性肿胀和假瘤性肿块形成。组织病理学上，其特征是淋巴细胞和浆细胞浸润，伴有IgG4阳性浆细胞，通过自身免疫机制导致典型的组织学表现——席纹状纤维化（“席纹状”=呈漩涡状，像草席）和闭塞性静脉炎，即血管阻塞性静脉炎。混合的Th1和Th2免疫反应似乎在发病机制中起重要作用，而本身无致病性的IgG4抗体的作用仍不清楚。糖皮质激素治疗在98%的病例中可导致缓解，通常作为维持治疗持续12个月。即使未经治疗，大多数患者也会缓解。对类固醇耐药的疾病可用免疫调节剂治疗。
IgG4相关性自身免疫性疾病正变得越来越常见，但目前仅从某些亚洲国家获得了充分的、系统获取的数据。跨学科合作是正确诊断和治疗的前提。需要治疗算法和随机对照试验为未来的器官特异性治疗指明方向。
