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Background . Immunoglobulin IgG4-associated cholangitis (IAC) disease is a systemic disease histologically characterized by extensive T lymphocytes and IgG4 positive plasma cell infiltration in various organs. Prevalence of IAC in PSC patients was reported to be between 7% and 11.6% in a few previous studies. This study was carried out to evaluate frequency of serum IgG4 level in PSC patient referred to the gastroenterology ward of Taleghani educational hospital in Tehran, Iran.  Material and Methods . This study was a prospective analytical cross-sectional study. Clinical presentation, laboratory values, imaging changes, inflammatory bowel disease (IBD), esophageal varices, ascites, and child score in newly PSC patients with elevated IgG4 were determined and compared with PSC patients with normal levels of IgG4. Data was analyzed by using SPSS software. The frequency and standard deviations were calculated. Differences among groups were evaluated by using the chi-square, fisher exact, and Mann-Whitney  U  tests.  Results . 34 patients with PSC were examined in the study period, of which 9 cases (26.5%) had high IgG4 levels. Most of the patients were male, 23 cases (67.6%) and nonsmoker, 26 cases (76.5%). Patient average age was 47 years old (range 21–67 years). There was not any significant relationship among patients with IAC and PSC patients in terms of variables such as age, smoking, presence of IBD, ascites, esophageal varices, child score, and imaging findings ( P  > 0.05).  Conclusion . IAC should be suspected in cases of unexplained biliary strictures with increased serum IgG4. Testing PSC patients for IgG4 and treating those who have high levels with corticosteroids in clinical trials should be considered in future studies.

Primary Sclerosing Cholangitis Associated with Elevated Immunoglobulin-G4: A Preliminary Study