ORIGINAL ARTICLE ¯ ALIMENTARY TRACT The long-term outcomes of patients with immunoglobulin G4- related sclerosing cholangitis: the Mayo Clinic experience Ahmad Hassan Ali 1 • Yan Bi 2 • Jorge D. Machicado 3 • Sushil Garg 1 • Ryan J. Lennon 4 • Lizhi Zhang 5 • Naoki Takahashi 6 • Elizabeth J. Carey 7 • Keith D. Lindor 7,8 • J. Gage Buness 9 • James H. Tabibian 10,11 • Suresh T. Chari 1 Received: 24 March 2020 / Accepted: 27 July 2020 Ó Japanese Society of Gastroenterology 2020 Abstract Background The long-term outcomes of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are not well known. Methods The outcomes of patients with IgG4-SC at Mayo Clinic (1999–2018) were compared to an age- and gender- matched (1:1 ratio) group of patients with primary scle- rosing cholangitis (PSC). Results We identified 89 patients with IgG4-SC; median age at diagnosis was 67 years, 81% were males, and the median follow-up was 5.7 years. Seventy-eight patients received prednisone for induction of remission, and 53 received at least one other immunosuppressive agent for maintenance of remission. Of the IgG4-SC group, 10 died (median time from diagnosis until death was 6.5 years): 2 due to cirrhosis, 3 due to cholangiocarcinoma (CCA), and 5 due to non-hepatobiliary causes. Eleven patients in the PSC group underwent liver transplantation, while none did in the IgG4-SC group. The incidence of a hepatobiliary adverse event (cirrhosis or CCA) was 3.4 times greater in the PSC compared to the IgG4-SC group (events per 1000 person-years: 52.6; 95% CI 38–73; vs. 15.6; 95% CI 7–32). The probability of development of a hepatobiliary adverse event within 10 years was 11% in the IgG4-SC compared to 45% in the PSC group (P = 0.0001). The overall sur- vival tended to be higher in the IgG4-SC compared to the PSC group (10-year: 79% vs. 68%, respectively; P = 0.11). Conclusions In a cohort of IgG4-SC patients, 88% of whom were treated with immunosuppressive drugs, the risk of cirrhosis and CCA was significantly lower compared to an age- and gender-matched group with PSC. Keywords Immunoglobulin G4-related disease Á Immunoglobulin G4-related sclerosing cholangitis Á Primary sclerosing cholangitis Á Cirrhosis Á Cholangiocarcinoma Ahmad Hassan Ali and Yan Bi Co-first authors. & Suresh T. Chari Chari.Suresh@mayo.edu 1 Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200 1st St SW, Rochester, MN 55905, USA 2 Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL, USA 3 Division of Gastroenterology and Hepatology, Mayo Clinic Health System, Eau Claire, WI, USA 4 Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN, USA 5 Department of Pathology, Mayo Clinic, Rochester, MN, USA 6 Department of Radiology, Mayo Clinic, Rochester, MN, USA 7 Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, AZ, USA 8 Arizona State University, Tempe, AZ, USA 9 Arizona College of Osteopathic Medicine, Midwestern University, Glendale, AZ, USA 10 Division of Gastroenterology, Olive View-UCLA Medical Center, Sylmar, CA, USA 11 David Geffen School of Medicine at UCLA, Los Angeles, CA, USA 123 J Gastroenterol https://doi.org/10.1007/s00535-020-01714-7