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Retroperitoneal fibrosis (RPF) is an inflammatory disorder that affects the infrarenal great vessels and surrounding structures. Although first described in 1948 by Ormond, much of the information currently available on this disease is conflicting and results from multiple definitions used by different investigators. We conducted the current study to describe the clinical, laboratory, and radiographic presentation of RPF from a single center using data collected in a prospective fashion.Data on all patients who were referred to our RPF clinic were prospectively collected. We obtained information on presenting signs and symptoms, and on the presence or absence of "traditional risk factors," comorbidities, and family histories. All patients underwent the same battery of laboratory testing, including complete blood count, renal function profile, erythrocyte sedimentation rate (ESR), thyroid-stimulating hormone, and antinuclear antibodies (ANA). All patients had contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) of the abdomen and pelvis. The extent of disease was classified based on the anatomic location of the soft-tissue density.Forty-eight patients (26 male and 22 female) met the criteria for RPF and were included in the study. The mean age was 54.25 years. Few patients had traditional risk factors for RPF. Pain and weight loss were the most common presenting symptoms. The mean hemoglobin was 11.6 g/dL. The mean ESR was 40.5 mm/h. Nine patients had positive ANA, all with negative antibodies to dsDNA. CT or MRI revealed the presence of a periaortic soft-tissue density in all (100%) patients; 69% had pericaval involvement, 62% had obstruction of 1 or both ureters, and 35% of patients had involvement of the renal artery or renal vein.In the current report we present the demographics and laboratory and radiographic presentation of a homogenous group of patients with RPF. We attempt to solidify a proper scheme of classification for the disease, and suggest an anatomic classification system that may be used for future studies.

Retroperitoneal fibrosis (RPF) is a rare disorder of unknown etiology. Its incidence is unknown, and the insidious and nonspecific nature of symptoms may contribute to considerable diagnostic delay. We conducted the current study to assess the incidence and clinicoradiologic characteristics of idiopathic RPF. For this, we evaluated prospectively 53 consecutive patients with a diagnosis of idiopathic RPF at our tertiary care referral center from April 1998 through January 2008.Calculated annual incidence of RPF was 1.3/100,000 inhabitants. Mean age was 64 +/- 11.1 (SD) yr; male-female ratio was 3.3:1.0. Median duration of symptoms was 6.0 mo (IQR 3.0-12.0). Abdominal, flank, and/or back pain and discomfort were the major symptoms, with visual analogue scale scores of 49 +/- 27.2 mm and 43 +/- 29.4 mm for pain and discomfort, respectively. Female patients had higher erythrocyte sedimentation rate (ESR), higher white blood cell count, and lower hemoglobin content than male patients at presentation. Computed tomography-documented maximal mass thickness amounted to 35 +/- 16.6 mm; craniocaudal length amounted to 137 +/- 48.8 mm. RPF mass extension up to or above the level of the renal vessels was noted in 3 patients (6%). Six patients (11%) presented with atypical RPF localization and/or bulky mass. Localized lymphadenopathy adjacent to the RPF mass was observed frequently (25%). Patients with hydronephrosis (56%) presented earlier than patients without hydronephrosis, with higher creatinine and greater mass thickness but similar pain severity. Patients were typically at high cardiovascular risk with increased-often aneurysmal-infrarenal aortic diameter (25.0 mm, IQR 22.0-30.0). RPF mass distribution was similar in patients with or without aneurysmal dilation. Occupational asbestos exposure (20%) and asbestos-related pleural changes (17%) were frequent among males. Previous or concurrent chronic inflammatory disease and/or autoimmune disease was noted in 8 patients (15%). Multivariate analysis revealed an independent association of ESR values with severity of pain and discomfort. Smoking was independently associated with infrarenal aortic diameter.In summary, annual RPF incidence is higher than previously assumed. Age at diagnosis and male-female ratio seem to have changed over time. RPF typically affects patients at high cardiovascular risk, including increased aortic diameter. Clinical presentation is influenced by sex, severity of inflammation and presence of hydronephrosis. Prolonged asbestos exposure and asbestos-related pleural changes were frequent among males. Localized lymphadenopathy adjacent to the RPF mass occurs frequently and should not confuse RPF diagnosis.