Retroperitoneal fibrosis. A true connective tissue disease.
Retroperitoneal fibrosis, a disease that can occur at any age, is characterized by a periaortic fibrous mass that often surrounds the ureters, leading to ureteral obstruction. Patients who present with this disease may complain of flank pain and acute renal failure. There is a high correlation with atherosclerotic disease of the aorta, although the pathogenesis of this disease remains unknown. Although recommendations for therapy remain empiric, prednisone seems to be efficacious in treatment; tamoxifen also may be effective. Retroperitoneal fibrosis usually does not lead to long-term morbidity or affect survival.
Duke University Medical Center, Durham, North Carolina, USA.
G S
GS
Gilkeson
N B
NB
Allen
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012185:Retroperitoneal Fibrosis / Q000150:complications / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy