Clinical features, responses to therapy and prognosis of 107 patients with IgG4-related cholangitis: a retrospective study

IgG4-related cholangitis (IRC) is an important hepatobiliary manifestation of IgG4-related diseases (IRD), frequently accompanied by other organ involvement of IRD especially autoimmune pancreatitis (AIP). Diagnosis of IRC requires comprehensive evaluation. The patients respond well to glucocorticosteroids and have good long-term prognosis regardless of relapse. This retrospective observational study described the clinical features, responses to therapy and prognosis of patients with IRC. The aim of our study was to describe and analyze the clinical features, responses to treatment and prognosis of 107 patients with IRC at our hospital, exploring the difficulties and clinical experience in the diagnosis and treatment of IRC. We conducted a retrospective study of 107 patients with IRC (88 males and 19 females; mean age, 60.8 years) treated at Peking Union Medical College Hospital between January 2013 and April 2023. Data on demographic, clinical, serologic, pathologic and imaging characteristics as well as treatment responses and prognosis were collected and analyzed. Statistical Package for Social Sciences software (SPSS, version 25.0) and Python (version 3.11) were used for the data analysis. Survival was assessed using Kaplan–Meier curves. Univariate and multivariate logistic regression analysis was used to analyze risk factors of relapse. A two-sided probability P < 0.05 was considered statistically significant. Receiver operating characteristic (ROC) curve was used to predict the value of steroid maintenance duration for relapse. The most common symptoms at presentation were jaundice (76.6%) and weight loss (72.9%). AIP was the most prevalent comorbidity (96.3%). Serum IgG4 levels were elevated in 99% of the patients, and isolated stenosis of the lower part of the common bile duct (type 1 IRC) was the most common imaging finding (49.5%). Nearly 90% of the patients were treated with steroids, and all presented with an excellent response. A relapse rate of 37.5% was observed during the follow-up period, most of which occurred after steroid withdrawal (47.2%). Steroid maintenance duration was the significant independent risk factor of relapse in patients with IRC (P = 0.001) (odds ratio, 0.875; 95% CI, 0.811–0.944). The patients were followed up for a median of 63.8 months. Four patients (4.0%) died, and none of them had cholangiocarcinoma. Overall 5-year and 10-year survival rates were 97.0% and 96.0%, respectively. The disease-specific survival rate was 100%. Among the 107 patients examined, IRC was found to be benign and exhibited an excellent response to steroid treatment. No patient progressed to cholangiocarcinoma, and relapse had no impact on the overall survival rate.