Abstract
Background & Aims: Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. The aim of the present study was to characterize the clinical features and therapeutic response of patients with IgG4-RD and identify risk factors for disease relapse.Methods: We collected baseline data of eligible patients with IgG4-RD and analyzed clinical feature by interview and review of medical records. The patients who received medical therapy with at least 3 months follow-up were used to characterize the therapeutic response and identify risk factors for relapse. Result: Totally 127 IgG4-RD patients, including 92 males and 35 females, were included in the present study. The median age at diagnosis was 63.0 years, ranging from 23 to 86. The pancreas, bile duct and lymph nodes were the most frequently involved organs. The serum IgG4 level was elevated in 94.5% of the patients and was correlated with the number of organs involved. Patients classified as head and neck limited group were more likely to be female. Patients classified as pancreato-hepatobiliary group had highest aminotransferase, alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (GGT), bilirubin level (all p<0.05). Mikulicz syndrome and systemic involvement group had the highest IgG-RI score, serum globulin, IgG and IgG4 level. Among 92 patients who received medical therapy with at least 3 months follow-up,76 received glucocorticoids (GC) alone or in combination with immunosuppressive agents and 16 patients did not take GC. 74 out of the 76 patients (97.3%) achieved remission, with 59 of them remained in remission and 15 of them relapsed. Wheras16 patients did not take GC, among them, 6 patients achieved remission with one patient relapsed. On multivariate analysis, higher initial score of ACR/EULAR IgG4-RD Classification Criteria, GC monotherapy and GC withdrawal were independent predictors for relapse.Conclusion: Different phenotypes of IgG4-RD showed different demographic and serological features. The risk factors of relapse were GC withdrawal, GC monotherapy and higher score of ACR/EULAR IgG4-RD Classification Criteria.