Retroperitoneal fibrosis and ankylosing spondylitis: which links?

Objectives: To report a case of retroperitoneal fibrosis (RPF) in a patient with ankylosing spondylitis (AS) and to review the medical literature for similar cases to detect possible links between the 2 diseases.

Methods: The presentation, clinical course, diagnostic work-up, and treatment of our patient are described, and the English and French medical literature from 1960 to 2000 is reviewed using a MEDLINE search for cases with coexisting RPF and spondyloarthritis.

Results: Our patient with AS had RPF, which mimicked rectal cancer with retroperitoneal and vertebral metastases. Special attention is paid to the unusual clinical presentation, the multistep diagnostic process, and the therapeutic strategy which was both radiologically and histologically successful. Literature review revealed 18 cases of concomitant RPF and spondyloarthritis, mainly AS. Patients were more frequently male (M/F, 3/1) and developed spondyloarthritis several years before RPF.

Conclusions: RPF may result from a local immune response to products of aortic atheromatous plaques, with subsequent periaortic deposition of fibrous tissue. However, the clinical features and the frequent association with other fibrosing disorders suggest that RPF is a systemic inflammatory condition. The role of AS-associated aortitis in the development of RPF warrants consideration.