Retroperitoneal Fibrosis

• Definitions
· Retroperitoneal Fibrosis is an autoimmune inflammatory disease which causes scarring and fibrosis behind the abdominal cavity.
· Retroperitoneal Fibrosis can produce chronic flank pain, back pain and obstruction of urine flow from of the kidneys.

• Overview
· Retroperitoneal Fibrosis, also known as Ormond’s disease, is uncommon.
· Retroperitoneal Fibrosis incidence rate is about 1 case in 200,000.
· Retroperitoneal Fibrosis is twice as common in men and is usually diagnosed in people between 40 and 60 years of age.
· Certain drugs may sensitize the immune system in Retroperitoneal Fibrosis.
· The drugs include methysergide, beta-adrenergic blockers, lysergic acid diethylamide, methyldopa, amphetamines, phenacetin, and cocaine.
· Retroperitoneal Fibrosis is usually not associated with a known inciting event.

• Types
· Retroperitoneal Fibrosis or Idiopathic Retroperitoneal Fibrosis
· Malignant Retroperitoneal Fibrosis
· Malignant Retroperitoneal Fibrosis is a type of the disease associated with metastatic tumors (cancer) of the retroperitoneum.
· Malignant Retroperitoneal Fibrosis has a very poor prognosis and a life expectancy of three to six months after diagnosis.

• Symptoms
· Retroperitoneal Fibrosis most common symptoms:
· Flank pain
· Back pain
· Lower abdominal pain
· Retroperitoneal Fibrosis patients also complain of:
· fever
· weight loss
· nausea
· vomiting
· fatigue
· frequent urination and blood in the urine.

• Treatment
· Retroperitoneal Fibrosis treatment with medications that suppress the immune system have been shown to have some benefit in decreasing the progression of fibrosis.
· Surgical dissection and clearing the the fibrosis around the ureters is helpful in maintaining kidney function.

References
• Rheum Dis Clin North AM. 1996;22:23-38

Posted on November 26, 2008

4 Responses to “Retroperitoneal Fibrosis”

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