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June 13, 1966

Idiopathic Retroperitoneal Fibrosis and Probable Systemic Lupus Erythematosus

Author Affiliations

From the departments of medicine (Mr. Lipman and Dr. Shapiro), pathology (Dr. Johnson), and urology (Dr. Berg), University of Pittsburgh School of Medicine.

JAMA. 1966;196(11):1022-1024. doi:10.1001/jama.1966.03100240156045

TWO CASES of retroperitoneal fibrosis were described by Ormond in 1948,1 and since then more than 125 cases have been reported. The classical description of the disease includes symptoms of anorexia, fatigue, backache, and flank pain with progressive oliguria and azotemia. Pathologically, the triad of hydronephrosis, medial ureteral deviation, and external ureteral compression by a mass of fibrous tissue has been prominent. Suggestions as to the etiology of the entity have ranged from a neoplastic process, an organized retroperitoneal hematoma, a primary urinary infection, and more recently to a primary vasculitis and hypersensitivity reaction.2-5 However, case reports demonstrating an association between idiopathic retroperitoneal fibrosis and other collagen diseases are sparse. Although it is probable that the disorder should not be considered of a unitarian origin, the present report describes a patient with idiopathic retroperitoneal fibrosis developing in the course of an illness suggestive of systemic lupus erythematosus. It

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