Atsushi Tanaka1, Susumu Tazuma2, Kazuichi Okazaki3, Takahiro Nakazawa4, Kazuo Inui5, Tsutomu Chiba6, Hajime Takikawa7. 1. Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan. Electronic address: a-tanaka@med.teikyo-u.ac.jp. 2. Department of General Medicine, Hiroshima University, Graduate School of Medical Science, Hiroshima, Japan. 3. The Third Department of Internal Medicine, Kansai Medical University, Hirakata, Japan. 4. Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan. 5. Department of Internal Medicine, Second Teaching Hospital, Fujita Health University, Nagoya, Japan. 6. Department of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 7. Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
Abstract
BACKGROUND & AIMS: 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.
BACKGROUND & AIMS: 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: Sumera Rizvi; Shahid A Khan; Christopher L Hallemeier; Robin K Kelley; Gregory J Gores Journal: Nat Rev Clin Oncol Date: 2017-10-10 Impact factor: 66.675
Authors: J-Matthias Löhr; Ulrich Beuers; Miroslav Vujasinovic; Domenico Alvaro; Jens Brøndum Frøkjær; Frank Buttgereit; Gabriele Capurso; Emma L Culver; Enrique de-Madaria; Emanuel Della-Torre; Sönke Detlefsen; Enrique Dominguez-Muñoz; Piotr Czubkowski; Nils Ewald; Luca Frulloni; Natalya Gubergrits; Deniz Guney Duman; Thilo Hackert; Julio Iglesias-Garcia; Nikolaos Kartalis; Andrea Laghi; Frank Lammert; Fredrik Lindgren; Alexey Okhlobystin; Grzegorz Oracz; Andrea Parniczky; Raffaella Maria Pozzi Mucelli; Vinciane Rebours; Jonas Rosendahl; Nicolas Schleinitz; Alexander Schneider; Eric Fh van Bommel; Caroline Sophie Verbeke; Marie Pierre Vullierme; Heiko Witt Journal: United European Gastroenterol J Date: 2020-06-18 Impact factor: 4.623