Clinical analysis of 49 cases with IgG4-related disease / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical manifestations,radiographic presentations,histopathological features,treatment and clinical follow-up of IgG4 related disease (IgG4-RD).Methods Forty-nine cases in our hospital were enrolled from 2009 to 2012 and were followed up.Results Of the 49 patients with IgG4-RD,the male to female ratio was 2.1∶1,the mean age was (53±15) years,and the serum IgG4 ranged between 0.39 to 20.8 g/L.The most commonly affected organ was pancreas.Two or more organs were involved in 15 patients.Histopathological findings included tissue infiltration by lymphocytes,IgG+ plasma cells and IgG4+ plasma cells and diffuse fibrosis among 45 patients.The IgG4+ plasma cells were over 10/high power fields (HPF).Responses to glucocorticoid in combination with immunosuppressants were good except 3 patients suffered relapses.Conclusion IgG4-RD is an autoimmune disease affecting multiple organs,and the increased IgG4 positive plasma cells is characteristic.This disease can be effectively alleviated with prompt use of glucocorticoid.