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, 48 (3), 303-14

Current Concept and Diagnosis of IgG4-related Disease in the Hepato-Bilio-Pancreatic System


Current Concept and Diagnosis of IgG4-related Disease in the Hepato-Bilio-Pancreatic System

Kazuichi Okazaki et al. J Gastroenterol.


Recently, IgG4-related disease (IgG4-RD) has been recognized as a novel clinical entity with multiorgan involvement and unknown origin, associated with abundant infiltration of IgG4-positive cells. The Japanese research committee, supported by the Ministry of Health, Labor and Welfare of Japan, unified many synonyms for these conditions to the term "IgG4-RD" in 2009. The international symposium on IgG4-RD endorsed the comprehensive nomenclature as IgG4-RD, and proposed the individual nomenclatures for each organ system manifestations in 2011. Although the criteria for diagnosing IgG4-RD have not yet been established, proposals include the international pathological consensus (IPC) and the comprehensive diagnostic criteria (CDC) for IgG4-RD for general use, and several organ-specific criteria for organ-specialized physicians, e.g., the International consensus diagnostic criteria (ICDC) and the revised clinical diagnostic criteria in 2011 by the Japan Pancreas Society (JPS-2011) for type1 AIP; the Clinical Diagnostic Criteria 2012 for IgG4-sclerosing cholangitis (IgG4-SC-2012); the diagnostic criteria for IgG4-positive Mikulicz's disease by the Japanese Society for Sjogren's syndrome; and diagnostic criteria for IgG4-related kidney disease by the Japanese Society of Nephrology. In cases of probable or possible IgG4-RD diagnosed by the CDC, organ-specific diagnostic criteria should be concurrently used according to a diagnosis algorithm for IgG4-RD, with referral to a specialist.


Fig. 1
Fig. 1
Histologic diagnostic schema of IgG4-related disease (reproduced from [6])
Fig. 2
Fig. 2
Algorism for Diagnosis of IgG4RD (reproduced from [5], with permission) Diagnostic algorithm performance for comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD) using comprehensive diagnostic criteria combined with organ-specific criteria. A diagnosis of IgG4-RD is definitive in patients with (1) organ enlargement, mass or nodular lesions, or organ dysfunction, (2) a serum IgG4 concentration >135 mg/dl, and (3) histopathological findings of >10 IgG4+ cells/HPF and an IgG4/IgG cell ratio >40 %

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