Article

Idiopathic Retroperitoneal Fibrosis and Necrotizing Vasculitis

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Abstract

ORMOND,1–3 is generally considered to be the first person to recognize the entity of idiopathic retroperitoneal fibrosis. Since his report in 1948 numerous case reports have been published, with remarkably little variation in the clinical and pathologic findings. Kay4 recently reviewed the world literature and summarized the findings in 125 cases. The disease is believed to be increasing in incidence.5

The lesion is characterized by formation of a retroperitoneal fibrotic plaque that causes constriction and loss of function of involved structures. The extent of the fibrosis and the amount of inflammation vary considerably.1–5 The disease seems to be self-limited, and operative intervention that frees the involved structures results in apparent cure. Etiologic concepts have been widely varied, and little progress has been made in defining the basic process involved.

The rarity of published autopsy studies of idiopathic retroperitoneal fibrosis, and an unusual combination of pathologic findings in a recent example of this disorder prompted the reporting of the following case.

Report of a Case

A 51-year-old white man, an electrician, was first admitted to the Cleveland Clinic Hospital, on April 22, 1964, because of severe anorexia of one month's duration, recently progressing to nausea and vomiting. During that month, tenderness and pain developed over the lumbosacral region, both thighs and calves associated with fatiguability and weakness. There were daily febrile episodes of shaking chills, sweating, and elevation of temperature of from 99 to 102 F. orally. Intermittent edema of the ankles had occurred beginning eight months before admission, . . .


 

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