Comparison of the long‐term outcomes between proximal and distal IgG4‐related sclerosing cholangitis: a multicenter cohort study
- 29 January 2023
- journal article
- research article
- Published by Wiley in Journal of Gastroenterology and Hepatology
- Vol. 38 (4), 648-655
- https://doi.org/10.1111/jgh.16136
Abstract
Background and AimsImmunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is considered a biliary manifestation of IgG4-related diseases. However, there has been a controversy on the clinical outcomes according to the location of the involved bile duct. We therefore compared the clinical outcomes and long-term prognosis of IgG4-SC with proximal bile duct involvement (proximal IgG4-SC) and IgG4-SC with distal bile duct involvement (distal IgG4-SC). MethodsWe reviewed the data of patients with IgG4-SC that were prospectively collected at 10 tertiary centers between March 2002 and October 2020. Clinical manifestations, outcomes, association with autoimmune pancreatitis (AIP), steroid-responsiveness, and relapse of IgG4-SC were evaluated. ResultsA total of 148 patients (proximal IgG4-SC, n = 59; distal IgG4-SC, n = 89) were analyzed. The median age was 65 years (IQR, 56.25-71), and 86% were male. The two groups were similar in terms of jaundice at initial presentation (51% vs 65%; P = 0.082) and presence of elevated serum IgG4 (66% vs 70%; P = 0.649). The two groups showed significant differences in terms of steroid-responsiveness (91% vs 100%; P = 0.008), association with AIP (75% vs 99%; P = 0.001), and occurrence of liver cirrhosis (9% vs 1%; P = 0.034). During a median follow-up of 64 months (IQR, 21.9-84.7), the cumulative relapse-free survival was significantly different between the two groups (67% vs 79% at 5 years; P = 0.035). ConclusionsRelapse of IgG4-SC frequently occurred during follow-up. Proximal IgG4-SC and distal IgG4-SC had different long-term outcomes in terms of steroid-responsiveness, occurrence of liver cirrhosis, and recurrence. It may be advantageous to determine the therapeutic and follow-up strategies according to the location of bile duct involvement.Keywords
This publication has 27 references indexed in Scilit:
- Consensus statement on the pathology of IgG4-related diseaseLaboratory Investigation, 2012
- Recommendations for the nomenclature of IgG4‐related disease and its individual organ system manifestationsArthritis & Rheumatism, 2012
- Utility of serum immunoglobulin G4 in distinguishing immunoglobulin G4-associated cholangitis from cholangiocarcinomaJournal of Hepatology, 2011
- International Consensus Diagnostic Criteria for Autoimmune PancreatitisPancreas, 2011
- Clinical clues to suspicion of IgG4‐associated sclerosing cholangitis disguised as primary sclerosing cholangitis or hilar cholangiocarcinomaJournal of Gastroenterology and Hepatology, 2010
- Endoscopic evaluation of factors contributing to intrapancreatic biliary stricture in autoimmune pancreatitisGastrointestinal Endoscopy, 2010
- Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitisThe Esophagus, 2009
- Intrapancreatic Common Bile Duct Involvement of Autoimmune Pancreatitis: Is It Really IgG4-Associated Cholangitis?Gastroenterology, 2008
- Immunoglobulin G4–Associated Cholangitis: Clinical Profile and Response to TherapyGastroenterology, 2008
- Diagnosis of Autoimmune Pancreatitis: The Mayo Clinic ExperienceClinical Gastroenterology and Hepatology, 2006