血清 IgG 水平和 IgG4 相关性疾病反应指数在 IgG4-RD 活动性评估中的应用.

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]