Image of the month: Ormond's disease: A diagnostic challenge

Almond Leung1, Haitham M Elasir2, Karim M Mahawish3

  • 1geriatric and stroke medicine, MidCentral DHB, Palmerston North, New Zealand.

Insights

Idiopathic retroperitoneal fibrosis, a rare condition, can mimic tuberculous aortitis. This case highlights successful treatment of idiopathic retroperitoneal fibrosis with latent tuberculosis using prednisone and methotrexate.

Area of Science:

  • Nephrology
  • Infectious Diseases
  • Cardiovascular Medicine

Background:

  • Idiopathic retroperitoneal fibrosis (IRPF) is a rare condition characterized by inflammation and fibrosis in the retroperitoneum.
  • It can present with constitutional symptoms and abdominal or flank pain, mimicking other serious conditions.
  • Infectious etiologies, particularly tuberculous aortitis, are critical differentials due to their high mortality.

Observation:

  • A 59-year-old man presented with a retroperitoneal periaortic mass.
  • Initial diagnosis suggested tuberculous aortitis.
  • Biopsy results revised the diagnosis to idiopathic retroperitoneal fibrosis with co-existing latent tuberculosis.

Findings:

  • Idiopathic retroperitoneal fibrosis can be mistaken for infectious causes like tuberculous aortitis.
  • Accurate diagnosis through biopsy is crucial for appropriate management.
  • Latent tuberculosis may coexist with idiopathic retroperitoneal fibrosis.

Implications:

  • This case underscores the importance of considering idiopathic retroperitoneal fibrosis in the differential diagnosis of retroperitoneal masses.
  • Timely and accurate diagnosis prevents unnecessary or incorrect treatments for conditions like tuberculous aortitis.
  • Successful management with immunosuppressants (prednisone) and disease-modifying agents (methotrexate) in IRPF with latent TB offers a therapeutic pathway.