L'eziopatogenesi della fibrosi retroperitoneale: Etiopathogenesis of retroperitoneal fibrosis

Abstract

The etiopathogenesis of retroperitoneal fibrosis is still obscure and probably multifactorial. Among the secondary forms due to demonstrable causes, the one caused by aorto-iliac atherosclerosis has recently been recognised. Its pathogenesis is linked to the low density oxidised lipoproteins of the atheromatous plaque, which are responsible for a local immunologic reaction. The most common form is still idiopathic or primitive, hypothetically related to genetic, environmental, vascular and/or immunologic factors. Idiopathic retroperitoneal fibrosis is sometimes associated with other sclerosing syndromes and/or systemic diseases. In such cases a common pathogenesis, probably immunologic may be postulated. After having illustrated the different categories of retroperitoneal fibrosis, the authors report their experience with 25 patients of whom 14 had idiopathic fibrosis and 11 secondary fibrosis. In the former group 11 patients (78.5%) smoked more than 10 cigarettes a day, while there was a history of prolonged professional exposure to asbestos in one case. The following associated pathologies were observed: hypertension in 7 cases (50%), ischemic cardiopathy in 2 (14.3%), diabetes mellitus in 2 (14.3%), multiple myeloma in 1 (7.1%) and juvenile rheumatoid arthritis in 1 (7.1%). The disease was also associated with other sclerosing pathologies in 3 cases: sclerosing cholangitis in 2 and Dupuytren's contracture in 1. The location of the fibrosis was typically periaortic in 13 cases (92.8%), as shown by CT. Lastly, 10 patients underwent immunosuppressive therapy with a favourable response, suggesting the probable immunologic pathogenesis of the disease.