Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis.

摘要

Retroperitoneal fibrosis (RPF) is characterized by the presence of an aberrant fibro-inflammatory retroperitoneal tissue that usually develops around the infrarenal portion of the abdominal aorta and the iliac arteries.10 Because of its rarity and the lack of diagnostic and classification criteria, RPF has often been considered to embrace a spectrum of diseases of varying etiology; this has led to the inclusion of heterogeneous patient populations in most of the studies carried out so far (especially in urologic and surgical series), which is why most clinical data are unreliable or difficult to interpret. In the last 2 decades, several attempts have been made to define a correct nosology of RPF and, although large-scale systematic studies are still lacking, it has become clear that RPF is to be distinguished in idiopathic and secondary forms, where the latter have clearly identifiable causes such as infections, radiation therapy, drugs, surgery, and cancer.10 This distinction, however, is not always clear-cut, since in some patients these conditions (particularly surgery, radiation, and the use of drugs) may be considered only "predisposing factors," and a direct causal relationship cannot be demonstrated.