Clinical features of IgG4-related disease

doi:10.7504/nk2015020404

Abstract：Objective To explore the clinical features,differential diagnosis and management of immunoglobulin 4-related disease(IgG4-RD).Methods The records of patients diagnosed with IgG4-RD at Chinese PLA General Hospital during Jan 2012 and Sep 2014 were retrospectively analyzed.Results Fifty-four patients (mean age 58.6± 1.6 years,40 men and 14 women) were retrieved.Multi-organ involvement was discovered in 31 (57.4％) patients,which involved pancreas (51.9％),lachrymal glands and salivary glands (48.1％),lymph nodes (20.4％),bile duct (18.5％),et al.51(94.4％) patients were accompanied by the hyperglobulinemia and elevation of serum IgG4 [mean 6.9 g/L (1.4-59.5 g/L)].While good clinical response to glucocorticoid therapy is observed in the majority of the patients(97.4％),immunosuppressive or rituxirnab can be required in case of poor efficacy.Conclusions IgG4-related disease is a rare autoimmune disease which affects mostly middle-aged and elderly men.More than two organs can be involved simultaneously,the commonest sites of involvement were pancreas and salivary gland.Steroid therapy is effective.