IDIOPATHIC PERIAORTIC FIBROSIS - A DIFFIC ULT DIAGNOSIS IN INTERNAL-MEDICINE

Periaortitis retroperitoneal fibrosis is characterized by a reaction o f a variable inflammatory nature while constricting the organs crossin g the retroperitoneal space, notably the ureters and the blood vessels . It is difficult to diagnose such a rare disease. We bring about here six cases of periaortic retroperitoneal fibrosis diagnosed from syste mic, digestive, urinary or vascular signs. Early diagnosis is often di fficult and is shown to be established after an average of three month s investigation. The average age of these patients, all of the male se x, is 58 years old (54 to 90). In the six cases this disease appears t o be idiopathic even though in two cases it is associated to giant tem poral arteritis and polymyalgia rheumatica. TDM remains the best diagn ostic tool to point out the existence of this fibrosis, to observe its evolution and to investigate for any extension of the disease. The et iology of this fibrosis remains a mysterious one; however an immunolog ic origin has been suggested. Medical treatment by corticosteroids is often successful but the long term evolution of the disease is still u ncertain (J Mal Vasc 1994; 19: pages 294-297).