DOI: 10.1097/MD.0b013e3181afc4d1

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PMID: 19593225

Issn Print: 0025-7974

Publication Date: 2009/07/01


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Retroperitoneal Fibrosis: New Insights Into Clinical Presentation and Diagnosis

Augusto Vaglio

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Excerpt

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.
The rarity of RPF, the heterogeneity of the conditions included in the previously reported series, and the lack of population-based studies have also limited a correct assessment of the epidemiologic characteristics of the disease, particularly its incidence and prevalence. Additional areas of uncertainty regarding the clinical management of RPF involve the definition of an appropriate diagnostic workup, with particular regard to the role of cross-sectional imaging and retroperitoneal biopsy, and the characterization of the disease phenotype, that is, the complete spectrum of clinical manifestations clinicians must assess when evaluating a patient with RPF. Clarifying these points is a conditio sine qua non for designing future controlled therapeutic trials.
In the current issue of Medicine, the studies by van Bommel et al13 and Scheel et al,7 performed prospectively on large cohorts of patients with the idiopathic form of RPF, address most of the issues outlined above, thus extending our knowledge regarding the clinical characteristics and imaging findings of the disease. Although the 2 studies have been carried out on separate patient cohorts, their results partially overlap, which proves their appropriate patient selection and collection of the clinical data; however, each study also raises some unique points.
Idiopathic RPF has traditionally been considered a very rare condition. Based on the results of a 2004 retrospective, case-control study performed in Finland,8 its incidence was estimated to be 1 × 10−6 person-years and its prevalence 1.4 × 10−5 inhabitants. In that study, however, the cases were retrieved from hospital discharge data using diagnostic codes, a method that can be fallacious particularly in the case of idiopathic RPF, where the diagnosis requires a complex workup and often needs a prolonged follow-up for its final confirmation and for the thorough exclusion of an underlying malignancy. The study by van Bommel et al13 is instead population-based, that is, it considers all RPF cases seen at the Albert Schweitzer Hospital in Dordrecht, The Netherlands, during a study period of 10 years, and precisely reports the patient referral pattern. Using this approach, the annual incidence of idiopathic RPF is shown to be 1.3 × 10−5 inhabitants,13 which is more than 10-fold higher than that reported in the Finnish study.8 Thus, idiopathic RPF is probably much more frequent than it appeared and, given the widespread use of cross-sectional imaging and the growing awareness of this disease among clinicians, it is likely that its incidence will rise in the near future.
Idiopathic RPF usually presents with 2 (often coexisting) types of manifestations: localized and systemic.

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