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Copyright of Fudan University Journal of Medical Sciences is the property of Fudan University Journal of Medical Sciences and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Fudan University Journal of Medical Sciences is the property of Fudan University Journal of Medical Sciences and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective To verify the diagnostic efficiency of the IgG4-related disease (IgG4-RD) responder index (RI) in the assessment of IgG4-RD disease activity，and to further explore new comprehensive clinical diagnostic criteria. Methods A total of 117 IgG4-RD patients who met the 2012 diagnosis criteria in Japan were divided into the active group (n=78) and stable group(n=39) by three experts using physician global assessment (PGA). We collected the general situation，clinical data， laboratory tests and IgG4-RD RI of the patients. The differences between the general conditions and laboratory tests of the two groups were analyzed. The serum markers and IgG4-RD RI were further analyzed to evaluate the sensitivity，specificity，positive predictive value，negative predictive value，and area under the curve of IgG4-RD disease activity. Results Peripheral blood eosnophils percentage(E%) platelet(PLT), erythrocyte sedimentation rate(ESR)，C-reactive protein(CRP), IgG, IgG4, globulin， interleukin-6（IL-6）and soluble IL-2 receptor(sIL2R)in active group were significantly higher than those in the stable group；while hemoglobin，total cholesterol，and triglycerides were significantly reduced. IgG, IgG4, globulin, ESR and sIL-2R levels were positively correlated with IgG4-RD RI (P <0.05). In the assessement of disease activity，the area under the curve of IgG4-RD RI and serum IgG levels were 0.940 and 0.916, respectively.The sensitivity and specificity were up to 92.3% and 86.5%，respectively for IgG4- RD RI≥6.The sensitivity and specificity were 89.1% and 82.6% for IgG≥11.6 g/L. Conclusion In the assessment of IgG4-RD disease activity，the IgG4-RD RI exhibited the highest diagnostic efficiency.Serum IgG level can also be used as a marker of disease activity，with high sensitivity and specificity.Both IgG4- RD RI and IgG were reliable and practical methods in clinical disease assessment. [ABSTRACT FROM AUTHOR]

IgG4-related disease (IgG4-RD) is a kind of autoimmune disease which is characterized by multi-organomegaly and elevated serum IgG4. Till now, the pathogenesis of IgG4-RD is unknown. The pattern recognition receptor of antigen-presenting cell is activated by pathogen infection, then CD4+ T cell is activated by antigen presentation and transform into Th1, Th2, Treg and Tfh cell. The cytokines secreted by Th1, Th2, Treg and Tfh cell will promote the activation of B cells and transformation to plasma cells and production of IgG4. Futhermore, transforming growth factor β (TGF-β) and platelet-derived growth factor secreted by macrophage and Treg will promote the fibrosis. The above cytokines and immune cells play important roles in the pathogenesis of IgG4-RD, which provide new strategy for treatment in the future. [ABSTRACT FROM AUTHOR]

Copyright of Fudan University Journal of Medical Sciences is the property of Fudan University Journal of Medical Sciences and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)