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A Le Joncour1, D Saadoun1, P Cacoub1

  • 1Department of Internal Medicine and Clinical Immunology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France; Sorbonne Université, UPMC Université Paris 06, UMR 7211, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005 Paris, France.

La Revue De Medecine Interne
|July 31, 2020
PubMed
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Retroperitoneal fibrosis (RPF) is a rare condition involving inflammation around the aorta. Treatment with corticosteroids and immunosuppressants is common, but relapses frequently occur.

Area of Science:

  • Medicine
  • Pathology
  • Immunology

Background:

  • Retroperitoneal fibrosis (RPF) is a rare condition characterized by fibro-inflammatory tissue encasing the aorta and surrounding structures.
  • RPF can be idiopathic or secondary to various underlying disorders.
  • Its exact pathophysiology remains unclear, though it may be linked to IgG4-related diseases.

Purpose of the Study:

  • To summarize the key aspects of retroperitoneal fibrosis (RPF), including its characteristics, diagnosis, and management.
  • To highlight the diagnostic tools and treatment strategies for RPF.
  • To emphasize the potential for relapse in RPF patients.

Main Methods:

  • Literature review of RPF focusing on diagnostic imaging and inflammatory markers.
  • Analysis of current treatment guidelines, including corticosteroids and immunosuppressive drugs.
Keywords:
AortiteAortitisFibrose rétro-péritonéaleIgG4 related diseaseMaladie associée aux IgG4PeriaortitisPéri-aortiteRetroperitoneal fibrosis

Related Experiment Videos

  • Review of management strategies for complications such as ureteral obstruction.

    • Main Results:

    • Imaging studies and inflammatory markers are crucial for RPF evaluation and monitoring.
    • Corticosteroids, often combined with immunosuppressants, form the primary treatment approach.
    • Ureteral stenting or nephrostomy is indicated for renal failure due to ureteral obstruction.

    Conclusions:

    • RPF requires a multi-faceted approach for diagnosis and management.
    • While initial treatment response is often positive, RPF has a high propensity for recurrence.
    • Further research into RPF pathophysiology may lead to improved therapeutic strategies.