[IgG4-related systemic diseases: a report of eight cases].

Objective To explore the clinical features of IgG4-related systemic diseases (IgG4-RSD)Methods A total of 8 inpatients with IgG4-RSD diagnosed at our hospital during August 2010 to December 2011 were recruited.We analyzed the clinical data,laboratory profiles,radiological and pathologic features and prognostic factors of these patients with IgG4-RSD.Results There were 5 males and 3 females with a mean onset age of 52.5 years.IgG4-related diseases were described in multiple organ systems:pancreas (n =5),biliary tree (n =3),kidneys (n =6),lungs (n =3),aorta and periaortic tissue,retroperitoneum (n =5) and lymph nodes (n =6).Hyperglobulinemia,elevated serum levels of IgG and IgG4,anemia(n =6),renal dysfunctions(n =5) and obstructive jaundice (n =2) were common laboratory findings.Lymphoplasmacytic infiltration and fibrosis were common pathologic findings.A diffuse infiltration of plasma cells with over 30 IgG4-positive cells per high-power field provided compelling evidences of IgG4-related disease.Patients with IgG4-RSD responded well to glucocorticoids.Conclusion With heterogeneous clinical characteristics,IgG4-RSD is found in various organ systems.The prominent histopathologic features of IgG4-RSD include a diffuse infiltration of plasma cells with over 30 IgG4-positive cells per high-power field.And the therapy of glucocorticoids is efficacious. Key words: Immunoglobulin G;  Pathology, clinical;  IgG4-related systemic diseases