An atypical cause of retroperitoneal fibrosis: Case report and literature review

Abstract

Introduction: Retroperitoneal fibrosis (RPF) is a rare inflammatory disease resulting in the development of a retroperitoneal mass, which may encase the aorta and its branches, as well as the ureters.

Observation: The systemic inflammatory response causes constitutional symptoms. The mass itself leads to symptoms of non-specific back pain and abdominal pain, as well as ureteric obstruction and subsequent renal insufficiency. Immunoglobulin 4 (IgG4) has recently been found to be involved in the pathogenesis of multiple autoimmune and inflammatory disorders, including RPF. Blood work-up, imaging and biopsy of the mass remain the mainstay of diagnosis.

Conclusion: RPF remains a diagnosis of exclusion. Treatment hinges on corticosteroids, but other immunosuppressants and disease-modifying agents are also being used more commonly. Surgical intervention is only carried out when conservative measures have failed or are contraindicated, and in order to preserve renal function.

Keywords: Histopathology; Idiopathic retroperitoneal fibrosis; IgG4-related;
Immunosuppressants; Management