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Retroperitoneal fibrosis accompanying immune thrombocytopenia
Author(s) -
Wallach Paul M.,
Flannery Michael T.,
Adelman Harold M.,
Powsang Julio,
Saba Hussein,
Altus Philip,
Espinoza Luis R.
Publication year - 1991
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830370315
Subject(s) - retroperitoneal fibrosis , medicine , obstructive uropathy , prednisone , hydronephrosis , rheumatology , fibrosis , anti nuclear antibody , nephrology , pathology , gastroenterology , immunology , autoantibody , urinary system , antibody
Abstract 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.