Comparing the clinical characteristics of IgG4-related sclerosing cholangitis with primary sclerosing cholangitis

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Objective To analyze and compare the clinical characteristics of primary sclerosing cholangitis (PSC) with IgG4-related sclerosing cholangitis (IgG4-SC). Methods The clinical data of 32 PSC patients and 72 IgG4-SC patients who were hospitalized in Peking Union Medical College Hospital (PUMCH) from January 2004 to December 2014 were retrospectively analyzed. Results Of the 32 PSC patients, there were 16 male and 16 female. Of the 72 IgG4-SC patients, there were 61 male and 11 female, (ratio=5.5∶1). The average ages were 44.9 (11~77) and 59.8 (28~83) years, respectively (P<0.05). The most common symptoms of PSC and IgG4-SC were abdominal pain and jaundice, and the incidences of abdominal pain and jaundice were 50.0% and 68.1%, 78.1% and 81.9%, respectively. The serum IgG4 level of the IgG4-SC patients was significantly higher than the PSC patients (P<0.05). The total protein in serum of the IgG4-SC patients was higher than the PSC patients (P<0.05). The rate of bile duct wall thickening as detected on endoscopic ultrasonography (EUS) was higher than by abdominal ultrasound and abdominal CT, which were 91.2%, 11.5% and 33.3%, respectively (P<0.05). 12 PSC patients were followed up for over 2 years, including 2 patients who underwent liver transplantation after failure of conservative treatment, 5 patients who died from hepatic failure and infection, and 3 with stable condition. 43 IgG4-SC patients were followed up for over 2 years, including 16 patients with relapse. The recurrence rate was 37.2% (16/43). The more the extrabiliary organs or bile duct segments were involved, the higher was the recurrence rate. Conclusions Both PSC and IgG4-SC are cholestatic diseases, and they have many similarities in clinical and imaging manifestations. However, they still have unique features. IgG4-SC is sensitive to glucocorticoids therapy and has good prognosis. Thus, it is important to differentiate PSC from IgG4-SC. Key words: Primary sclerosing cholangitis; IgG4-related sclerosing cholangitis; Autoimmune disease; Cholestasis; Diagnosis; Therapy