The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience

Ahmad Hassan Ali, Yan Bi, Jorge D. Machicado, Sushil Garg, Ryan J. Lennon, Lizhi Zhang, Naoki Takahashi, Elizabeth J. Carey, Keith D. Lindor, J. Gage Buness, James H. Tabibian, Suresh T. Chari

Research output: Contribution to journalArticlepeer-review

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 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.

Original languageEnglish (US)
Pages (from-to)1087-1097
Number of pages11
JournalJournal of gastroenterology
Volume55
Issue number11
DOIs
StatePublished - Nov 1 2020

Keywords

  • Cholangiocarcinoma
  • Cirrhosis
  • Immunoglobulin G4-related disease
  • Immunoglobulin G4-related sclerosing cholangitis
  • Primary sclerosing cholangitis

ASJC Scopus subject areas

  • Gastroenterology

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