The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience
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O R I G I N A L A R T I C L E¯ A L I M E N T A R Y T R A C T
The long-term outcomes of patients with immunoglobulin G4related sclerosing cholangitis: the Mayo Clinic experience Ahmad Hassan Ali1 • Yan Bi2 • Jorge D. Machicado3 • Sushil Garg1 • Ryan J. Lennon4 • Lizhi Zhang5 • Naoki Takahashi6 • Elizabeth J. Carey7 • Keith D. Lindor7,8 • J. Gage Buness9 • James H. Tabibian10,11 • Suresh T. Chari1
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 gendermatched (1:1 ratio) group of patients with primary sclerosing 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 survival 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 Immunoglobulin G4-related sclerosing cholangitis Primary sclerosing cholangitis Cirrhosis Cholangiocarcinoma
Ahmad Hassan Ali and Yan Bi Co-first authors. & Suresh T. Chari [email protected] 1
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200 1st St SW, Rochester, MN 55905, 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 Arizona College of Osteopathic Medicine, Midwestern University, Glendale, AZ, USA
2
Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL, USA
9
3
Division of Gastroenterology and Hepatology, Mayo Clinic Health System, Eau Claire, WI, USA
10
Division of Gastroenterology, Olive View-UCLA Medical Center, Sylmar, CA, USA
4
Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN
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