Tanaka Yuto, Kamimura Kenya, Nakamura Ryota, Ohkoshi-Yamada Marina, Koseki Yohei, Mizusawa Takeshi, Ikarashi Satoshi, Hayashi Kazunao, Sato Hiroki, Sakamaki Akira, Yokoyama Junji, Terai Shuji
Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata 9518510, Japan.
World J Clin Cases. 2020 Nov 26;8(22):5821-5830. doi: 10.12998/wjcc.v8.i22.5821.
A type 2b immunoglobulin G4 (IgG4)-related sclerosing cholangitis (SC) without autoimmune pancreatitis is a rare condition with IgG4-SC. While the variety of the imaging modalities have tested its usefulness in diagnosing the IgG4-SC, however, the usage of ultrasonography for the assessment of the response to steroidal therapy on the changes of bile duct wall thickness have not been reported in the condition. Therefore, the information of our recent case and reported cases have been summarized.
We report the case of an 82-year-old Japanese man diagnosed with isolated IgG4-related SC based on the increase of serum IgG4, narrowing of the bile duct, its wall thickness, no complication of autoimmune pancreatitis, and IgG4 positive inflammatory cell infiltration to the wall with the fibrotic changes. The cholangiogram revealed type 2b according to the classification. Corticosteroid treatment showed a favorable effect, with the smooth decrease in serum IgG4 and the improvement of the bile duct wall thickness.
As isolated type 2b, IgG4-SC is rare, the images, histological findings, and clinical course of our case will be helpful for physicians to diagnose and treat the new cases appropriately.
2b型免疫球蛋白G4（IgG4）相关硬化性胆管炎（SC）且无自身免疫性胰腺炎是IgG4-SC中的一种罕见情况。尽管多种成像方式已被用于检测其在诊断IgG4-SC中的效用，然而，关于超声检查在评估该疾病中胆管壁厚度变化对类固醇治疗反应方面的应用尚未见报道。因此，我们总结了近期病例及已报道病例的相关信息。
我们报告了一例82岁日本男性病例，该患者基于血清IgG4升高、胆管狭窄、胆管壁增厚、无自身免疫性胰腺炎并发症以及IgG4阳性炎性细胞浸润伴纤维化改变，被诊断为孤立性IgG4相关SC。根据分类，胆管造影显示为2b型。皮质类固醇治疗显示出良好效果，血清IgG4平稳下降，胆管壁厚度得到改善。
作为孤立的2b型，IgG4-SC较为罕见，我们病例的影像学表现、组织学发现及临床过程将有助于医生对新病例进行准确诊断和恰当治疗。
