title: Clinical Features, Response to Treatment, and Outcomes of IgG4-Related Sclerosing Cholangitis.
abstract: OBJECTIVE
  Immunoglobulin G4 sclerosing cholangitis (IgG4-SC) is a biliary tract manifestation of IgG4-related systemic disease. We investigated the demographics, clinical features at presentation, treatment response, and outcomes of IgG4-SC using data from a large-scale survey in Japan.
  
  
  METHODS
  We performed a retrospective cohort study of 527 patients with IgG4-SC (436 female; median age, 66.2 years; range, 23-89 years) in Japan from 2000 to 2015. Data on patient demographics, presentation, treatment response, and outcomes were collected from questionnaires given to patients at 211 referral centers in Japan in 2015. Patients were diagnosed with IgG4-SC based on the clinical diagnostic criteria established by the Japanese Biliary Association in 2012. Patients were followed for a median of 4.1 ± 3.1 years. Survival was assessed using the Kaplan-Meier curve and log-rank test.
  
  
  RESULTS
  Symptoms at presentation included jaundice (35% of patients) and pruritus (13% of patients); 28% of patients were asymptomatic. It was extremely rare for patients with IgG4-SC to present with symptoms of decompensated cirrhosis. At presentation, serum levels of IgG4 were increased (>135 mg/dL) in 84% of patients. Response to prednisolone was excellent in 90% of patients. No patients received liver transplants, and cholangiocarcinoma was found in only 4 patients (0.7%). Restenosis of bile ducts was observed in 19% of patients but did not affect overall survival.
  
  
  CONCLUSIONS
  In a retrospective cohort study of 527 patients with IgG4-SC in Japan, we found the disease to be benign. Most patients (90%) respond to treatment with prednisolone and few develop decompensated cirrhosis or cholangiocarcinoma.
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan. Electronic address: a-tanaka@med.teikyo-u.ac.jp.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tanaka
authors.affiliation: Department of General Medicine, Hiroshima University, Graduate School of Medical Science, Hiroshima, Japan.
authors.forename: Susumu
authors.initials: S
authors.lastname: Tazuma
authors.affiliation: The Third Department of Internal Medicine, Kansai Medical University, Hirakata, Japan.
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
authors.affiliation: Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Nakazawa
authors.affiliation: Department of Internal Medicine, Second Teaching Hospital, Fujita Health University, Nagoya, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Inui
authors.affiliation: Department of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Chiba
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Takikawa
mesh_terms: D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007564:Japan; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D044967:Serum / Q000737:chemistry; D011795:Surveys and Questionnaires; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D007074:Immunoglobulin G; D011239:Prednisolone
keywords: Cancer; Inflammation; Liver Transplantation; Natural History