LINICAL – LIVER , PANCREAS , AND BILIARY RACT mmunoglobulin G 4 – Associated Cholangitis : Clinical Profile and esponse to Therapy

ackground & Aims: Immunoglobulin (Ig)G4-asociated cholangitis (IAC) is the biliary manifestaion of a steroid-responsive multisystem fibroinammatory disorder in which affected organs have characteristic lymphoplasmacytic infiltrate rich in gG4-positive cells. We describe clinical features, reatment response, and predictors of relapse in AC and compare relapse rates in IAC with inrapancreatic vs proximal bile duct strictures. ethods: We reviewed clinical, serologic, and imging characteristics and treatment response in 53 AC patients. Results: IAC patients generally were lder (mean age, 62 y) men (85%), presenting with bstructive jaundice (77%) associated with autoimune pancreatitis (92%), increased serum IgG4 levls (74%), and abundant IgG4-positive cells in bile uct biopsy specimens (88%). At presentation, bilary strictures were confined to the intrapancreatic ile duct in 51%; the proximal extrahepatic/intraepatic ducts were involved in 49%. Initial presenation was treated with steroids (n 30; median ollow-up period, 29.5 months), surgical resection n 18; median follow-up period, 58 months), or as conservative (n 5; median follow-up period, 35 onths). Relapses occurred in 53% after steroid withrawal; 44% relapsed after surgery and were treated ith steroids. The presence of proximal extrahepatic/ ntrahepatic strictures was predictive of relapse. Steoid therapy normalized liver enzyme levels in 61%; iliary stents could be removed in 17 of 18 patients. ifteen patients treated with steroids for relapse fter steroid withdrawal responded; 7 patients on dditional immunomodulatory drugs remain in teroid-free remission (median follow-up period, 6 onths). Conclusions: IAC should be suspected in nexplained biliary strictures associated with inreased serum IgG4 and unexplained pancreatic disase. Relapses are common after steroid withdrawal, specially with proximal strictures. The role of immuomodulatory drugs for relapses needs further study.