The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Moriguchi, Japan.
J Gastroenterol. 2010;45(1):122-9. doi: 10.1007/s00535-009-0130-y. Epub 2009 Sep 18.
Immunoglobin G4-related sclerosing cholangitis (IgG4-SC) is recognized as one of the systemic sclerosing diseases characterized by abundant IgG4-positive plasma cells with effective steroid therapy. On the other hand, primary sclerosing cholangitis (PSC), recognized as a sclerosing cholangitis of unknown origin without steroid efficacy, has been often clinically confused with IgG4-SC. To date, the prognosis of IgG4-SC is unclear, while the prognosis of PSC is well known to be poor. Therefore, it is clinically very important to be able to distinguish IgG4-SC from PSC. However, at the present time it still remains unclear whether PSC may sometimes be misdiagnosed as IgG4-SC or not. Herein, we report three rare cases of PSC with elevated serum IgG4 levels and/or an infiltration of abundant IgG4-positive plasma cells in the liver: a young male with ulcerative colitis (UC), and elderly female and a young female, each with elevated serum IgG4 levels. The first two patients showed infiltration of abundant IgG4-positive plasma cells in the portal area of the liver without response to steroid therapy. From our experiences, we emphasize that some patients with PSC, who do not respond to steroid therapy, show elevated serum IgG4 levels and/or infiltration of abundant IgG4-positive plasma cells, although the mechanism still remains unclear.
免疫球蛋白 G4 相关硬化性胆管炎（IgG4-SC）被认为是一种全身性硬化性疾病，其特征是大量 IgG4 阳性浆细胞，对类固醇治疗有效。另一方面，原发性硬化性胆管炎（PSC）被认为是一种原因不明的硬化性胆管炎，对类固醇无效，临床上常与 IgG4-SC 相混淆。迄今为止，IgG4-SC 的预后尚不清楚，而 PSC 的预后众所周知较差。因此，临床上能够区分 IgG4-SC 和 PSC 非常重要。然而，目前尚不清楚 PSC 是否有时可能被误诊为 IgG4-SC。在此，我们报告三例罕见的 PSC 伴有血清 IgG4 水平升高和/或肝脏内大量 IgG4 阳性浆细胞浸润的病例：一例年轻男性合并溃疡性结肠炎（UC），两例老年女性和一例年轻女性，血清 IgG4 水平均升高。前两例患者表现为肝脏门脉区大量 IgG4 阳性浆细胞浸润，对类固醇治疗无反应。根据我们的经验，我们强调一些对类固醇治疗无反应的 PSC 患者，尽管其机制尚不清楚，但表现为血清 IgG4 水平升高和/或大量 IgG4 阳性浆细胞浸润。
