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. 2012:2012:357071.
doi: 10.1155/2012/357071. Epub 2012 May 29.

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

Kazuichi Okazaki  1 , Hisanori Umehara
Affiliations

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

Kazuichi Okazaki et al. Int J Rheumatol. 2012.

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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Figures

Figure 1
Figure 1
Clinical findings of IgG4-related disease. Physical examinations and imaging on US/CT/MRI can show the characteristic diffuse/localized swelling, masses, or thickness in single or multiple organs.
Figure 2
Figure 2
Diagnostic algorithm for IgG4-RD in Japan.
See this image and copyright information in PMC

References

    1. Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. Journal of Gastroenterology. 2003;38(10):982–984. - PubMed
    1. van der Vliet HJ, Perenboom RM. Multiple pseudotumors in IgG4-associated multifocal systemic fibrosis. Annals of Internal Medicine. 2004;141(11):896–897. - PubMed
    1. Kamisawa T, Funata N, Hayashi Y. Lymphoplasmacytic sclerosing pancreatitis is a pancreatic lesion of IgG4-related systemic disease. The American Journal of Surgical Pathology. 2003;27(8):1119–1127. - PubMed
    1. Kamisawa T, Okamoto A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. Journal of Gastroenterology. 2006;41(7):613–625. - PMC - PubMed
    1. Kamisawa T, Nakajima H, Egawa N, Funata N, Tsuruta K, Okamoto A. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006;6(1-2):132–137. - PubMed

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