Ahmad H. Ali, MBBS1, Yan Bi, MD, PhD2, Jorge Machicado, MD3, Sushil Garg, MBBS4, Elizabeth J. Carey, MD5, Keith D. Lindor, MD6, James H. Tabibian, MD, PhD7, Suresh T. Chari, MD, FACG4
1University of Missouri, Columbia, MO; 2Mayo Clinic, Jacksonville, FL; 3Mayo Clinic, Eau Claire, WI; 4Mayo Clinic, Rochester, MN; 5Mayo Clinic, Phoenix, AZ; 6Arizona State University, Phoenix, AZ; 7Olive View - UCLA Medical Center, Sylmar, CA
Introduction: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) has been recognized worldwide. However, its natural history and long-term outcomes are not well known.
Methods: In this retrospective, single center, cohort study, we identified patients who received a diagnosis of IgG4-SC at Mayo Clinic Rochester from 1999 to 2018.The diagnosis of IgG4-SC was rigorously ascertained by review of medical records and using the validated HISORt criteria. Comprehensive clinical data was extracted from patients’ charts. Long-term outcomes of interest included cirrhosis, cholangiocarcinoma (CCA), and death. To compare outcomes, a random sample of PSC patients was selected in a 1:1 ratio matched for age of diagnosis and gender. Survival analyses were performed for comparisons using the Kaplan-Meier method, and log-rank test, and the cumulative incidence of cirrhosis and CCA was assessed using the competing risks regression method.
Results: A total of 61 patients were diagnosed with IgG4-SC during the study period. The median age of diagnosis was 66 years (IQR: 56-71 years), 85% were male, and the median follow-up was 6.1 years (IQR: 3.2-8.3 years). The pancreas was the most commonly involved extra-biliary organ (93%). Fifty-five patients received prednisone of whom 96% experienced complete or partial remission. Sixteen patients received rituximab as maintenance therapy, of whom 88% maintained remission. Three patients (5%) developed cirrhosis, and 2 patients (3%) developed CCA. Nine patients (15%) died; 2 due to cirrhosis, 2 due to CCA, 1 due to pancreatic cancer, and 4 due to non-hepatobiliary causes. Patients with IgG4-SC had significantly lower 5- and 10-year cumulative incidence of developing cirrhosis (5y: 4% vs. 34%; 10y: 6% vs. 43%; P< 0.001) and CCA (5y: 3% vs. 17%; 10y: 4% vs. 22%; P=0.012) compared with PSC patients. Two patients in the PSC group underwent liver transplantation, while none in the IgG4-SC group. Overall 5- and 10-year survival was greater in the IgG4-SC cohort (5y: 92% vs. 70%; 10y: 77% vs. 57%; P=0.02).
Discussion: Patients with IgG4-SC, the majority of whom received treatment, have a small potential risk of long-term hepatobiliary complications. Their risk of cirrhosis, CCA and death, was lower than in patients with PSC. The better outcome in the IgG4-SC group is likely due to the benefit of treatment. Surveillance in this population needs further assessment, especially the subset that does receive or respond to treatment.
Citation: Ahmad H. Ali, MBBS; Yan Bi, MD, PhD; Jorge Machicado, MD; Sushil Garg, MBBS; Elizabeth J. Carey, MD; Keith D. Lindor, MD; James H. Tabibian, MD, PhD; Suresh T. Chari, MD, FACG. P0931 - A 20-YEAR OUTCOMES’ STUDY OF PATIENTS WITH IMMUNOGLOBULIN G4-RELATED SCLEROSING CHOLANGITIS: THE MAYO CLINIC EXPERIENCE. Program No. P0931. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.