Nagai Kazuki, Hosaka Hiroo, Takahashi Yutaka, Kubo Shuichi, Nakamura Noriko, Andou Kazuo
Nagai Clinic, Internal Medicine, 1-7-25, Yokodi, Isogo-ku,, Yokohama City, Kanagawa, 2350045, Japan.
BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.02.2009.1590. Epub 2009 Jun 1.
We present a case of IgG4-related sclerosing disease complicated by sclerosing cholangitis (SC), idiopathic retroperitoneal fibrosis (IRF) and orbital pseudotumour (OPT). Clinical, radiographic and pathological findings later suggested that the patient had SC complicated by IRF. The patient's SC and IRF were well controlled for the first 10 years of the follow-up period; OPT developed in the tenth year. During investigation of the OPT, serum IgG4 level was found to be significantly elevated. The patient was then diagnosed with IgG4-related sclerosing disease complicated by SC, IRF and OPT. This is a rare manifestation of IgG4-related sclerosing disease, which was diagnosed incidentally during OPT work-up. We suggest that this is a variation of the so-called IgG4-related sclerosing disease or hyper-IgG4 disease.
我们报告一例IgG4相关硬化性疾病，并发硬化性胆管炎（SC）、特发性腹膜后纤维化（IRF）和眼眶假瘤（OPT）。临床、影像学和病理检查结果后来提示该患者患有合并IRF的SC。在随访的前10年，患者的SC和IRF得到了良好控制；第10年出现了OPT。在对OPT进行检查时，发现血清IgG4水平显著升高。该患者随后被诊断为并发SC、IRF和OPT的IgG4相关硬化性疾病。这是IgG4相关硬化性疾病的一种罕见表现，在OPT检查过程中偶然被诊断出来。我们认为这是所谓的IgG4相关硬化性疾病或高IgG4疾病的一种变体。
