Färkkilä Martti
Helsingin yliopisto ja HYKS:n gastroenterologian klinikka, PL 340, 00029 HUS.
Duodecim. 2011;127(1):49-55.
Immunoglobulin G4 associated autoimmune cholangitis (IAC) is a recently described cholangiopathy, which closely resembles primary sclerosing cholangitis (PSC) and belongs to IgG4 sclerosing disease family. Unlike PSC the disease is most commonly seen in elderly men presenting with abdominal pain and obstructing jaundice as the first symptoms. In cholangiograms, distal biliary tract changes are most frequent. IAC is seldom associated with inflammatory bowel disease, less than in 10 % of cases. Differential diagnosis between Iac and PSC is essential because both the therapy and prognosis are totally different. Definite diagnosis is based on immunohistology or concomitant presentation of other IgG4 associated sclerosing diseases in addition to elevated IgG4-serum concentration. Steroid therapy has been shown to ameliorate symptoms and leads to regression of bile duct abnormalities and normalizes the liver function tests.
免疫球蛋白G4相关性自身免疫性胆管炎（IAC）是一种最近才被描述的胆管病，它与原发性硬化性胆管炎（PSC）极为相似，属于IgG4硬化性疾病家族。与PSC不同的是，这种疾病最常见于老年男性，首发症状为腹痛和梗阻性黄疸。在胆管造影中，远端胆道改变最为常见。IAC很少与炎症性肠病相关，病例占比不到10%。Iac与PSC之间的鉴别诊断至关重要，因为两者的治疗方法和预后完全不同。明确诊断基于免疫组织学检查，或者除血清IgG4浓度升高外，还伴有其他IgG4相关性硬化性疾病。已证明类固醇疗法可改善症状，使胆管异常消退，并使肝功能检查结果恢复正常。
