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IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity.

Umehara H, Nakajima A, Nakamura T, Kawanami T, Tanaka M, Dong L, Kawano M - Int. Immunol. (2014)

Bottom Line: IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world.The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4(+) plasma cells.Recently, many reports of aberrant acquired immunity such as Th2-diminated immune responses have been published.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan Present address: Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan umehara606@gmail.com.

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Algorithm for the diagnosis of IgG4-RD (27). A diagnosis of IgG4-RD is definitive in patients with all three items (category 1; C1). A diagnosis of IgG4-RD is possible in patients who fulfill criteria 1 and 2, but with negative results on histopathology or without histopathologic examination (categories 2 and 3; C2 and C3), whereas a diagnosis of IgG4-RD is probable in patients with organ involvement (1) and fulfilled histopathologic criteria, but without increased serum IgG4 concentration (2) (category 4; C4). Patients without satisfying the serologic or histopathologic criteria are considered unlikely to have IgG4-RD (categories 5 and 6; C5 and C6). For patients in category 2–6, organ-specific criteria for IgG4-RD could be applied, such as those for AIP, MD, kidney disease and sclerosing cholangitis associated with IgG4. Patients who fulfill the organ-specific criteria for IgG4-RD have a definite diagnosis of this disease (category 7; C7).
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Figure 2: Algorithm for the diagnosis of IgG4-RD (27). A diagnosis of IgG4-RD is definitive in patients with all three items (category 1; C1). A diagnosis of IgG4-RD is possible in patients who fulfill criteria 1 and 2, but with negative results on histopathology or without histopathologic examination (categories 2 and 3; C2 and C3), whereas a diagnosis of IgG4-RD is probable in patients with organ involvement (1) and fulfilled histopathologic criteria, but without increased serum IgG4 concentration (2) (category 4; C4). Patients without satisfying the serologic or histopathologic criteria are considered unlikely to have IgG4-RD (categories 5 and 6; C5 and C6). For patients in category 2–6, organ-specific criteria for IgG4-RD could be applied, such as those for AIP, MD, kidney disease and sclerosing cholangitis associated with IgG4. Patients who fulfill the organ-specific criteria for IgG4-RD have a definite diagnosis of this disease (category 7; C7).

Mentions: The CD criteria we have proposed for IgG4-RD consist of three parts: concept, diagnostic criteria and explanatory notes (27). The concept clarifies the features characteristic of IgG4-RD, such as location of lesions, symptoms and prognosis. Diagnostic criteria are based on the two major characteristics of IgG4-RD: increased serum IgG4 concentrations and infiltration of IgG4+ cells. Although tissue biopsies are difficult to obtain from some organs, including the pancreas, retroperitoneum and ocular cavity, histopathological examination is important. Because IgG4+ plasma cell infiltration has been reported in various diseases and clinical conditions, such as rheumatoid synovitis, inflammatory oral and skin lesions and carcinomas with a peritumoral inflammatory response, pathological criteria should be rigorous. Histopathological findings of marked IgG4+ cell infiltration [>10 cells per high-power field (HPF)] and an IgG4/IgG cell ratio >40% are diagnostic of IgG4-RD. A diagnostic algorithm for IgG4-RD, using comprehensive diagnostic criteria combined with organ-specific criteria, is shown in Fig. 2.


IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity.

Umehara H, Nakajima A, Nakamura T, Kawanami T, Tanaka M, Dong L, Kawano M - Int. Immunol. (2014)

Algorithm for the diagnosis of IgG4-RD (27). A diagnosis of IgG4-RD is definitive in patients with all three items (category 1; C1). A diagnosis of IgG4-RD is possible in patients who fulfill criteria 1 and 2, but with negative results on histopathology or without histopathologic examination (categories 2 and 3; C2 and C3), whereas a diagnosis of IgG4-RD is probable in patients with organ involvement (1) and fulfilled histopathologic criteria, but without increased serum IgG4 concentration (2) (category 4; C4). Patients without satisfying the serologic or histopathologic criteria are considered unlikely to have IgG4-RD (categories 5 and 6; C5 and C6). For patients in category 2–6, organ-specific criteria for IgG4-RD could be applied, such as those for AIP, MD, kidney disease and sclerosing cholangitis associated with IgG4. Patients who fulfill the organ-specific criteria for IgG4-RD have a definite diagnosis of this disease (category 7; C7).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4201844&req=5

Figure 2: Algorithm for the diagnosis of IgG4-RD (27). A diagnosis of IgG4-RD is definitive in patients with all three items (category 1; C1). A diagnosis of IgG4-RD is possible in patients who fulfill criteria 1 and 2, but with negative results on histopathology or without histopathologic examination (categories 2 and 3; C2 and C3), whereas a diagnosis of IgG4-RD is probable in patients with organ involvement (1) and fulfilled histopathologic criteria, but without increased serum IgG4 concentration (2) (category 4; C4). Patients without satisfying the serologic or histopathologic criteria are considered unlikely to have IgG4-RD (categories 5 and 6; C5 and C6). For patients in category 2–6, organ-specific criteria for IgG4-RD could be applied, such as those for AIP, MD, kidney disease and sclerosing cholangitis associated with IgG4. Patients who fulfill the organ-specific criteria for IgG4-RD have a definite diagnosis of this disease (category 7; C7).
Mentions: The CD criteria we have proposed for IgG4-RD consist of three parts: concept, diagnostic criteria and explanatory notes (27). The concept clarifies the features characteristic of IgG4-RD, such as location of lesions, symptoms and prognosis. Diagnostic criteria are based on the two major characteristics of IgG4-RD: increased serum IgG4 concentrations and infiltration of IgG4+ cells. Although tissue biopsies are difficult to obtain from some organs, including the pancreas, retroperitoneum and ocular cavity, histopathological examination is important. Because IgG4+ plasma cell infiltration has been reported in various diseases and clinical conditions, such as rheumatoid synovitis, inflammatory oral and skin lesions and carcinomas with a peritumoral inflammatory response, pathological criteria should be rigorous. Histopathological findings of marked IgG4+ cell infiltration [>10 cells per high-power field (HPF)] and an IgG4/IgG cell ratio >40% are diagnostic of IgG4-RD. A diagnostic algorithm for IgG4-RD, using comprehensive diagnostic criteria combined with organ-specific criteria, is shown in Fig. 2.

Bottom Line: IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world.The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4(+) plasma cells.Recently, many reports of aberrant acquired immunity such as Th2-diminated immune responses have been published.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan Present address: Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan umehara606@gmail.com.

Show MeSH
Related in: MedlinePlus