a Division of RA and Autoimmune Diseases , Internal Medicine, Nagahama City Hospital , Shiga , Japan.
b The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology , Kansai Medical University , Osaka , Japan.
Mod Rheumatol. 2017 May;27(3):381-391. doi: 10.1080/14397595.2017.1290911.
IgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criterion has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan, published comprehensive diagnostic criteria for IgG4-RD. Several problems with these criteria have arisen in clinical practice, however, including the difficulty obtaining biopsy samples from some patients, and the sensitivity and the specificity of techniques used to measure serum IgG4 concentrations. Although serum IgG4 concentration is an important clinical marker for IgG4-RD, its diagnostic utility in differentiating IgG4-RD from other diseases, called IgG4-RD mimickers, remains unclear. This review describes the current optimal approach for the diagnosis of IgG4-RD, based on both comprehensive and organ-specific diagnostic criteria, in patients with diseases such as IgG4-related pancreatitis (AIP), sclerosing cholangitis, and renal, lung and orbital diseases.
IgG4 相关疾病（IgG4-RD）是日本研究者提出的一种引人关注的临床实体，包括多种疾病，以前被诊断为米库利茨病（MD）、自身免疫性胰腺炎（AIP）、间质性肾炎、前列腺炎、腹膜后纤维化等。尽管每个医学领域的所有临床医生都可能遇到这种新疾病，但尚未建立统一的诊断标准。2011 年，日本厚生劳动省（MHLW）组织的日本 IgG4 研究组发布了 IgG4-RD 的综合诊断标准。然而，这些标准在临床实践中出现了一些问题，包括一些患者获得活检样本的困难，以及用于测量血清 IgG4 浓度的技术的敏感性和特异性。尽管血清 IgG4 浓度是 IgG4-RD 的一个重要临床标志物，但它在区分 IgG4-RD 与其他疾病（称为 IgG4-RD 模拟物）方面的诊断效用仍不清楚。这篇综述描述了基于综合和器官特异性诊断标准，在 IgG4 相关胰腺炎（AIP）、硬化性胆管炎以及肾脏、肺部和眼部疾病等疾病患者中诊断 IgG4-RD 的最佳方法。
