Retroperitoneal Fibrosis: Still a Diagnostic Challenge

Area of Science:

• Fibroinflammatory disorders
• Abdominal imaging
• Autoimmune diseases

Background:

• Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disorder impacting the abdominal aorta and surrounding structures.
• RPF is categorized into primary (idiopathic) and secondary forms, with primary RPF potentially linked to immunoglobulin (Ig) G4-related disease.
• Despite an increase in case reports, RPF awareness remains suboptimal.

Observation:

• A 49-year-old female presented with chronic abdominal pain, initially attributed to pancreatitis.
• Computed tomography (CT) and magnetic resonance imaging (MRI) revealed signs of RPF progression, ruling out malignancy.
• The patient had a history of psoriasis and cholecystectomy, with pancreatitis-associated inflammation noted.

Findings:

• The patient was diagnosed with idiopathic RPF, with potential predisposing factors including psoriasis, prior surgeries, and pancreatitis.
• Steroid therapy led to significant symptom improvement, indicating effectiveness in managing non-malignant RPF.
• Idiopathic RPF constitutes over two-thirds of all RPF cases, and autoimmune disease overlap is common.

Implications:

• This case underscores the importance of considering RPF in patients with unexplained abdominal symptoms and relevant risk factors.
• Steroids are effective for non-malignant RPF, but prospective trials and consensus guidelines for diagnosis and management are lacking.
• Streamlined diagnostic and management protocols are crucial for improving patient outcomes in RPF.