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      Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

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          Abstract

          Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.

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

          Tooru Shimosegawa, Suresh T. Chari, Luca Frulloni … (2011)
          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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            A new clinicopathological entity of IgG4-related autoimmune disease.

            Terumi Kamisawa, Nobuaki Funata, Yukiko Hayashi … (2003)
            Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases. To investigate the pathophysiology of AIP, we immunohistochemically examined the pancreas and other organs in eight patients with AIP, and in controls, using anti-CD4-T and CD8-T cell subsets, as well as IgG4 antibodies. In AIP patients, severe or moderate infiltration of IgG4-positive plasma cells associated with CD4- or CD8-positive T lymphocytes was detected in the peripancreatic tissue (6/6), bile duct (8/8), gallbladder (8/8), portal area of the liver (3/3), gastric mucosa (5/7), colonic mucosa (2/2), salivary glands (1/2), lymph nodes (6/6), and bone marrow (2/2), as well as in the pancreas (8/8). There were few IgG4-positive plasma cells at the same sites in controls. These results suggest that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in IgG4-related systemic disease with extensive organ involvement. We propose a new clinicopathological entity, of a systemic IgG4-related autoimmune disease in which AIP and its associated diseases might be involved. Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases.
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              Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011

              Hisanori Umehara, Kazuichi Okazaki, Yasufumi Masaki … (2014)
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                Author and article information

                Journal
                Journal ID (nlm-ta): Int J Rheumatol
                Journal ID (iso-abbrev): Int J Rheumatol
                Journal ID (publisher-id): IJR
                Title: International Journal of Rheumatology
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 1687-9260
                ISSN (Electronic): 1687-9279
                Publication date (Print): 2012
                Publication date (Electronic): 29 May 2012
                Volume: 2012
                Electronic Location Identifier: 357071
                Affiliations
                1Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, Hirakata, Osaka 573-1197, Japan
                2Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Kanazawa, Japan
                Author notes

                Academic Editor: Vikram Deshpande

                Article
                DOI: 10.1155/2012/357071
                PMC ID: 3368488
                PubMed ID: 22690221
                SO-VID: 58dfa3c5-707e-4a99-a8f9-a5124dfcf3cc
                Copyright © Copyright © 2012 K. Okazaki and H. Umehara.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 9 November 2011
                Date accepted : 25 March 2012
                Categories
                Subject: Review Article

                ScienceOpen disciplines: Rheumatology
                Data availability:
                ScienceOpen disciplines: Rheumatology

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