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      Clinical practice guidelines for IgG4‐related sclerosing cholangitis

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          Abstract

          IgG4‐related sclerosing cholangitis (IgG4‐ SC) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis and currently recognized as a biliary manifestation of IgG4‐related disease. Although clinical diagnostic criteria of IgG4‐ SC were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Furthermore, no practical guidelines for IgG4‐ SC are available. Because the evidence level of most articles retrieved through searching the PubMed, Cochrane Library, and Igaku Chuo Zasshi databases was below C based on the systematic review evaluation system of clinical practice guidelines MINDS 2014, we developed consensus guidelines using the modified Delphi approach. Three committees (a guideline creating committee, an expert panelist committee for rating statements according to the modified Delphi method, and an evaluating committee) were organized. Eighteen clinical questions ( CQs) with clinical statements were developed regarding diagnosis (14 CQs) and treatment (4 CQs). Recommendation levels for clinical statements were set using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4‐ SC.

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          Most cited references145

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          International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology.

          To achieve the goal of developing international consensus diagnostic criteria (ICDC) for autoimmune pancreatitis (AIP). An international panel of experts met during the 14th Congress of the International Association of Pancreatology held in Fukuoka, Japan, from July 11 through 13, 2010. The proposed criteria represent a consensus opinion of the working group. Autoimmune pancreatitis was classified into types 1 and 2. The ICDC used 5 cardinal features of AIP, namely, imaging of pancreatic parenchyma and duct, serology, other organ involvement, pancreatic histology, and an optional criterion of response to steroid therapy. Each feature was categorized as level 1 and 2 findings depending on the diagnostic reliability. The diagnosis of type 1 and type 2 AIP can be definitive or probable, and in some cases, the distinction between the subtypes may not be possible (AIP-not otherwise specified). The ICDC for AIP were developed based on the agreement of an international panel of experts in the hope that they will promote worldwide recognition of AIP. The categorization of AIP into types 1 and 2 should be helpful for further clarification of the clinical features, pathogenesis, and natural history of these diseases.
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            Rituximab for IgG4-related disease: a prospective, open-label trial.

            To evaluate the efficacy of rituximab (RTX) in IgG4-related disease (IgG4-RD) in an open-label pilot trial.
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              Standard steroid treatment for autoimmune pancreatitis.

              To establish an appropriate steroid treatment regimen for autoimmune pancreatitis (AIP). A retrospective survey of AIP treatment was conducted in 17 centres in Japan. The main outcome measures were rate of remission and relapse. Of 563 patients with AIP, 459 (82%) received steroid treatment. The remission rate of steroid-treated AIP was 98%, which was significantly higher than that of patients without steroid treatment (74%, 77/104; p<0.001). Steroid treatment was given for obstructive jaundice (60%), abdominal pain (11%), associated extrapancreatic lesions except the biliary duct (11%), and diffuse enlargement of the pancreas (10%). There was no relationship between the period necessary to achieve remission and the initial dose (30 mg/day vs 40 mg/day) of prednisolone. Maintenance steroid treatment was given in 377 (82%) of 459 steroid-treated patients, and steroid treatment was stopped in 104 patients. The relapse rate of patients with AIP on maintenance treatment was 23% (63/273), which was significantly lower than that of patients who stopped maintenance treatment (34%, 35/104; p = 0.048). From the start of steroid treatment, 56% (55/99) relapsed within 1 year and 92% (91/99) relapsed within 3 years. Of the 89 relapsed patients, 83 (93%) received steroid re-treatment, and steroid re-treatment was effective in 97% of them. The major indication for steroid treatment in AIP is the presence of symptoms. An initial prednisolone dose of 0.6 mg/kg/day, is recommend, which is then reduced to a maintenance dose over a period of 3-6 months. Maintenance treatment with low-dose steroid reduces but dose not eliminate relapses.
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                Author and article information

                Contributors
                kamisawa@cick.jp
                Journal
                J Hepatobiliary Pancreat Sci
                J Hepatobiliary Pancreat Sci
                10.1002/(ISSN)1868-6982
                JHBP
                Journal of Hepato-Biliary-Pancreatic Sciences
                John Wiley and Sons Inc. (Hoboken )
                1868-6974
                1868-6982
                18 January 2019
                January 2019
                : 26
                : 1 ( doiID: 10.1002/jhbp.2019.26.issue-1 )
                : 9-42
                Affiliations
                [ 1 ] Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital Tokyo Japan
                [ 2 ] Department of Gastroenterology Japanese Red Cross Nagoya Daini Hospital Nagoya Japan
                [ 3 ] Department of General Internal Medicine Hiroshima University Graduate School of Biomedical & Health Science Hiroshima Japan
                [ 4 ] Department of Diagnostic Pathology Kobe University Kobe Japan
                [ 5 ] Department of Medicine Teikyo University School of Medicine Tokyo Japan
                [ 6 ] Department of Community‐Based Medical Education Nagoya City University Graduate School of Medical Sciences Nagoya Japan
                [ 7 ] Department of Medicine, Gastroenterology Shinshu University Matsumoto, Nagano Japan
                [ 8 ] Department of Gastroenterology Second Teaching Hospital Fujita Health University Nagoya Japan
                [ 9 ] Department of Radiology Kanazawa University Graduate School of Medical Sciences Kanazawa Japan
                [ 10 ] Department of Gastroenterology Tokyo Womens’ Medical University Yachiyo Medical Center Yachiyo Japan
                [ 11 ] Department of Gastroenterology and Metabolism Nagoya City University Graduate School of Medical Sciences Nagoya Japan
                [ 12 ] Department of Gastroenterology and Hepatology Tokyo Medical University Tokyo Japan
                [ 13 ] Department of Anatomic Pathology Kurashiki Central Hospital Kurashiki Japan
                [ 14 ] Division of Gastroenterology Tohoku University Graduate School of Medicine Sendai Japan
                [ 15 ] Department of Endoscopy Yokohama City University Hospital Yokohama Japan
                [ 16 ] Department of Gastroenterology Tokyo Takanawa Hospital Tokyo Japan
                [ 17 ] Department of Gastroenterology Graduate School of Medicine Juntendo University Tokyo Japan
                [ 18 ] Department of Gastroenterology Tokyo Womens’ Medical University Tokyo Japan
                [ 19 ] Endoscopy Center Chiba University Hospital Chiba Japan
                [ 20 ] Division of Gastroenterology South‐Miyagi Medical Center Ohgawara Japan
                [ 21 ] Department of Internal Medicine Matsumoto Dental University Matsumoto Japan
                [ 22 ] Kansai Electric Power Hospital Osaka Japan
                [ 23 ] The Third Department of Internal Medicine Division of Gastroenterology and Hepatology Kansai Medical University Moriguchi Japan
                [ 24 ] Faculty of Medicine Departments of Gastroenterology and Gastroenterological, General, Breast, and Thyroid Surgery Yamagata University Yamagata Japan
                [ 25 ] Division of Hepato‐Biliary Pancreatic Surgery Tohoku University Graduate School, of Medicine Sendai Japan
                [ 26 ] Department of Hepato‐Biliary‐Pancreatic and Gastrointestinal Surgery School of Medicine International University of Health and Welfare Ichikawa Japan
                Author notes
                [*] [* ] Correspondence to: Terumi Kamisawa, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo 113‐8677, Japan.

                e‐mail: kamisawa@ 123456cick.jp

                Article
                JHBP596
                10.1002/jhbp.596
                6590186
                30575336
                c78ad024-de62-4295-b75e-ab42d77e5ee3
                © 2018 The Authors. Journal of Hepato‐Biliary‐Pancreatic Sciences published by John Wiley & Sons Australia, Ltd on behalf of Japanese Society of Hepato‐Biliary‐Pancreatic Surgery.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                Page count
                Figures: 28, Tables: 5, Pages: 34, Words: 21617
                Funding
                Funded by: Ministry of Health, Labor, and Welfare of Japan
                Categories
                Guideline
                Guideline
                Custom metadata
                2.0
                jhbp596
                January 2019
                Converter:WILEY_ML3GV2_TO_NLMPMC version:5.6.4 mode:remove_FC converted:24.06.2019

                Gastroenterology & Hepatology
                bile duct,guideline,igg4,sclerosing cholangitis,steroid
                Gastroenterology & Hepatology
                bile duct, guideline, igg4, sclerosing cholangitis, steroid

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