IgG₄-related sclerosing disease:A case report and review of literature

Objective To report a rare case of IgG4-related sclerosing disease involving multiple organs in order to improve our understanding of it. Methods A rare case of IgG4-related sclerosing disease admitted to our hospital in November 2010 with its diagnosis established by pathology was analyzed and its clinical manifestations,diagnosis,treatment,and prognosis were discussed according to its domestic and foreign literature. Results The patient’s clinical manifestations included diabetes mellitus,impaired renal function,enlargement of lymph nodes in the neck and groin,high IgG and IgG4 level,and enlargement of pancreas.Abdominal CT showed enlargement of pancreas and interstitial pneumonia.Biopsy of lymph nodes revealed infiltration of IgG4-positive plasma cells.Biopsy of kidney tissue displayed focal sclerosing glomerulonephritis.The renal function became normal and the size of pancreas and lymph nodes was decreased 3 weeks after treatment with corticosteroids.The serum IgG and IgE levels returned to normal and diabetes mellitus was well controlled 20 weeks after treatment with corticosteroids. Conclusion IgG4-related sclerosing disease is a very rare systemic disease,which can be controlled with prompt use of corticosteroids.