Medical Challenge Posed by Retroperitoneal Fibrosis: Case Reports and Literature Review

Area of Science:

• Nephrology
• Vascular Surgery
• Rheumatology

Background:

• Idiopathic retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory disease impacting the abdominal aorta and iliac arteries.
• RPF can lead to ureteral obstruction, renal failure, and compression of adjacent structures, with idiopathic cases linked to IgG4-related disease and environmental factors like asbestos and tobacco.
• Clinical presentation is nonspecific, ranging from pain to symptoms of ureteral compression, a primary complication.

Observation:

• Clinical presentation is nonspecific, ranging from pain to symptoms of ureteral compression.
• Computed tomography (CT) and magnetic resonance imaging (MRI) are vital diagnostic tools.
• Two clinical cases of idiopathic RPF, one associated with IgG4, are presented.

Findings:

• The primary goal of RPF treatment is to halt the fibro-inflammatory reaction's progression.
• Medical management is the first-line treatment, with biological agents like rituximab and infliximab used sparingly.
• Surgery is reserved for refractory cases, focusing on ureteral obstruction relief alongside systemic steroids.

Implications:

• Accurate diagnosis through advanced imaging (CT/MRI) is essential for effective RPF management.
• A conservative approach combining systemic therapy and interventions like ureteral stenting is recommended.
• Understanding RPF's association with IgG4 and autoimmune conditions aids in comprehensive patient care.