Retroperitoneal fibrosis accompanying immune thrombocytopenia

Retroperitoneal fibrosis represents an idiopathic systemic fibrosing syndrome which leads to the development of progressive hydronephrosis and azotemia (Stewart and Friberg: Southern Medical Journal 77:1185–1187, 1984; Morad et al.: American Journal of Medicine 82:363–366, 1987). Evidence in the literature supports a hypothesis that retroperitoneal fibrosis represents a systemic autoimmune process (Lichon et al.: Journal of Rheumatology 11:373,374, 1984; Lipman et al.: JAMA 196:204–206, 1966; Littlejohn and Keystone: Journal Rheumatology 8:665–669, 1981; Katz et al.: American Journal of Clinical Pathology 67:436–439, 1977). We report a case of a patient with retroperitoneal fibrosis, immune thrombocytopenia, presence of antinuclear antibody, and direct Coombs positivity which provides further evidence that retroperitoneal fibrosis is an autoimmune process. Progressive obstructive uropathy related to his fibrosis led to bilateral hydronephrosis and significant renal function impairment. Also, he developed marked immune thrombocytopenia. Treatment with prednisone produced a dramatic response in both platelet count and renal function.