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  • Retroperitoneal fibrosis (RPF; also known as Ormonds\' disease) is a rare disorder that is idiopathic in most cases (68%). - Evidence suggests autoimmunity. o Response to ceroid leaking from arteries thinned by atheromatous plaque - Many diverse causes for the 32% non-idiopathic cases o Drugs: Methysergide, ergotamine, some beta-adrenergic blocking agents, hydralazine, and methyldopa o Other associations (immune-mediated): E.g., primary biliary cirrhosis, systemic lupus erythematosus o Malignant form (poor prognosis): Occurs in response to metastatic tumor cells in the retroperitoneum o Other implicated causes: Abdominal aortic aneurysm, ureteric injury, infection, retroperitoneal malignancy, radiation therapy, and chemotherapy - Characterized by extensive deposition of fibrous tissue in retroperitoneal space compressing ureters bile ducts, blood vessels, and other structures - Symptoms generally vague; clinical picture nonspecific o Radiologic and other imaging modalities needed to confirm diagnosis - Insidious symptomatology: o Flank pain, back pain, lower abdomen pain, malaise, anorexia, weight loss, pyrexia, nausea, vomiting, lower extremity edema and/or thrombophlebitis, and symptoms of arterial compromise of abdomen or lower extremities (e.g., intermittent claudication)
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