A case of retroperitoneal fibrosis accompanying immune thrombocytopenic purpura / 대한내과학회지

ABSTRACT

Retroperitoneal fibrosis is a slowly progressing syndrome that is a part of a systemic fibrosing disease. Most causes are idiopathic, whereas the remainder are associated with methysergide ingestion, malignancy, or aneurysm of abdominal aorta. The pathogenesis is unclear, but the evidences supporting systemic autoimmune process are present, i.e. the apprearance of autoimmune antibodies, especially antinuclear antibody, positive direct or indirect Coombs' test, and the association with immune thrombocytopenia. Effective treatment with corticosteroid is another suggestion of autoimmune nature of this disease. We experienced a case of retroperitoneal fibrosis with immune thrombocytopenic purpura and positive antinuclear antibody. A 44-years old man who was in splenectomy state due to immune thrombocytopenic purpura for 15 years visited us for obstructive uropathy caused by retroperitoneal fibrosis. He was treated with double J catheter insertion in both ureters, and oral medication of corticosteroid and tamoxifen. Renal failure and thrombocytopenia was improved after treatment and the retroperitoneal fibrotic mass size decreased.