IgG4-related systemic diseases: a report of eight cases

Abstract

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. 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. 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.