病例報告：以腎盂輸尿管接合處腫瘤為臨床表現之第四型免疫球蛋白G相關疾病

許家榮、張建祥

彰化基督教醫院 外科部 泌尿科

IgG4-related disease with ureteropelvic junction tumor – a case report

HSU CHIA JUNG, CHANG CHIEN HSIANG

Divisions of Urology, Department of Surgery, Changhua Christian Hospital, Changhua, Taiwan

IgG4-Related Disease is a rare condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, and elevation of serum immunoglobulin G4 can been noted during acute phase. The response of the disease to oral steroids are usually prompt. Patients with IgG4-Related Disease may present with lymphadenopathy, type I autoimmune pancreatitis, fibrosing mediastinitis, pulmonary inflammatory pseudotumor and retroperitoneal fibrosis and so on.

We herein report a rare presentation of IgG4-Related Disease who suffered from acute pancreatitis, and was found to have ureteropelvic junction tumor. CT-guide biopsy revealed fibroadipose tissue with dense lymphoid aggregate, and elevation of serum IgG concentration was also noted. The diagnosis of this disease was based on clinical symptoms, pathology and laboratory data.