A case of retroperitoneal fibrosis accompanying immune thrombocytopenic purpura.
- Author:
Yoon Ju OH
1
;
Won PARK
;
Sung Kwon BAE
;
Jung Su SONG
;
Seung Won CHOI
;
Yoon Suk HUR
;
Ze Hong WOO
Author Information
1. Department of Internal Medicine, College of Medicine, Inha University, Inchon, Korea.
- Publication Type:Case Report
- Keywords:
retroperitoneal fibrosis;
autoimmune disease;
idiopathic thrombocytopenic purpura
- MeSH:
Adult;
Aneurysm;
Antibodies;
Antibodies, Antinuclear;
Aorta, Abdominal;
Autoimmune Diseases;
Catheters;
Coombs Test;
Eating;
Humans;
Methysergide;
Purpura, Thrombocytopenic, Idiopathic*;
Renal Insufficiency;
Retroperitoneal Fibrosis*;
Splenectomy;
Tamoxifen;
Thrombocytopenia;
Ureter
- From:Korean Journal of Medicine
1999;56(6):757-760
- CountryRepublic of Korea
- Language:Korean
-
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.
