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Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation

E F H van Bommel1, I Jansen, T R Hendriksz

  • 1From the Department of Internal Medicine (EFHVB, ALHJA) and Department of Radiology (IJ, TRH), Albert Schweitzer Hospital, Dordrecht, The Netherlands.

Medicine
|July 14, 2009
PubMed

Insights

Retroperitoneal fibrosis (RPF) occurs more frequently than previously thought, affecting patients with high cardiovascular risk. This study details its incidence, clinicoradiologic features, and risk factors.

Area of Science:

  • Nephrology
  • Radiology
  • Epidemiology

Background:

  • Retroperitoneal fibrosis (RPF) is a rare disorder with unknown etiology and potentially delayed diagnosis due to nonspecific symptoms.
  • Understanding the incidence and characteristics of idiopathic RPF is crucial for timely diagnosis and management.

Purpose of the Study:

  • To determine the annual incidence of idiopathic RPF.
  • To describe the clinicoradiologic features, patient demographics, and associated risk factors of idiopathic RPF.

Main Methods:

  • Prospective evaluation of 53 consecutive patients diagnosed with idiopathic RPF over a 10-year period (April 1998 - January 2008).
  • Data collection included patient demographics, symptom duration, clinical presentation, laboratory values (ESR, WBC, hemoglobin), and imaging findings (CT scan).
  • Statistical analysis included incidence calculation, comparison between genders, and multivariate analysis for risk factor association.

Main Results:

  • The calculated annual incidence of RPF was 1.3/100,000 inhabitants.
  • The mean age of patients was 64 years, with a male-to-female ratio of 3.3:1.0. Major symptoms included abdominal, flank, and/or back pain.
  • Patients were often at high cardiovascular risk, with increased aortic diameter. Asbestos exposure was frequent in males. ESR values correlated with pain severity, and smoking with aortic diameter.

Conclusions:

  • The incidence of RPF is higher than previously estimated, with potential shifts in age at diagnosis and gender ratio over time.
  • RPF commonly affects patients with cardiovascular risk factors, including enlarged aortic diameter. Clinical presentation varies based on sex, inflammation severity, and hydronephrosis.
  • Frequent lymphadenopathy adjacent to the RPF mass should not lead to misdiagnosis.

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