IgG4-related retroperitoneal fibrosis: A case report and literature review

Abstract

Clinical data of one patient diagnosed as IgG4-related retroperitoneal fibrosis was analyzed.IgG4andretroperitoneal fibrosiswere used as the key words to search literatures from PubMed and Wanfang Database. Clinical data of 27 cases with IgG4 retroperitoneal fibrosis, including clinical manifestation, diagnostic basis, therapeutic method and prognosis, which were retrieved through literatures, were analyzed. A 82-year-old male patient presented with urinary tract obstruction and renal insufficiency. Imaging examination revealed a retroperitoneal irregular lump shadow, and showed chronic inflammation of soft tissues in posterior peritoneum with fibrosis by biopsy pathology. The IgG4-positive plasmocyte infiltration was found by immunohistochemical method. The serum level of IgG4 was significantly increased, which was in accord with IgG4-related retroperitoneal fibrosis. After treatment with methylprednisolone, retroperitoneal lump shadow was obviously dissipated. The serum level of IgG4 became normal. 27 cases with IgG4-related retroperitoneal fibrosis were searched through literature retrieval, with 19 confirmed cases, 3 suspected cases, and 5 suspicious cases. Male accounted for 92.6% (25/27), and the average age was 67.6 years old. The cases with the involvement of extraperitoneal organs accounted for 37.0% (10/27). The misdiagnosis rate was 55.6% (15/27). 23 cases took effective therapy with glucocorticoids.IgG4-related retroperitoneal fibrosis is an orphan disease, which can be misdiagnosed easily. The diagnosis is made with histopathological examination and determination on the serum level of IgG4. The therapy with glucocorticoids is effective. Key words: Retroperitoneal fibrosis; Immunoglobulin G