SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferences
Retroperitoneal fibrosis
Other Resources UpToDate PubMed

Retroperitoneal fibrosis

Contributors: Abhijeet Waghray MD, Khaled Bittar MD, Nishant H. Patel MD, Desiree Rivera-Nieves MD, Paritosh Prasad MD
Other Resources UpToDate PubMed


Retroperitoneal fibrosis (RPF, Ormond disease) is a rare disease distinguished by extensive fibrosis in the peritoneum. It most commonly presents in individuals aged 40-60. There is a higher prevalence of RPF in males.

The condition can be idiopathic or secondary to infection, drugs, or trauma. The idiopathic form is thought to be immune mediated.

The most common presenting symptom is lower abdominal pain, lower back pain, or flank pain. Other clinical signs and symptoms include fever, lower extremity edema, renal obstruction or insufficiency, scrotal pain, phlebitis, and deep venous thrombosis.

Some rare presentations include weight loss, malaise, anorexia, Raynaud phenomenon, ureteric colic, hematuria, claudication, and urinary frequency.

Possible complications include urinary tract obstruction and chronic kidney damage. Some uncommon clinical features that may result from complications include ascites, peripheral edema, hydrocele, jaundice, small or large bowel obstruction, and spinal cord compression.

Treatment is dependent on the severity, location, and cause of RPF. If the diagnosis of idiopathic RPF is made early, corticosteroids, anti-inflammatory medications, or immunosuppressants may be suggested. If diagnosis is made once fibrosis has developed into ureteral blockage, the obstruction will have to be addressed with drainage tubes or surgery. For some patients, treatment may involve a combination of medication and internal intervention.

Treatment of secondary RPF is aimed at treating the underlying etiology or stopping the causative agent.

Prognosis will depend on the extent and severity of RPF. Kidney damage, if present, may be temporary or permanent.


N13.5 – Crossing vessel and stricture of ureter without hydronephrosis

49120005 – Retroperitoneal fibrosis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Acute appendicitis
  • Vascular abnormalities causing ischemia (see Acute mesenteric ischemia)
  • Urinary tract infection
  • Pancreatitis (Acute pancreatitis, Chronic pancreatitis)
  • Bowel obstruction (Small bowel obstruction, Large bowel obstruction)
  • Bowel hematoma
  • Malignancy

Best Tests

Subscription Required

Management Pearls

Subscription Required


Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required


Subscription Required

Last Reviewed:02/07/2018
Last Updated:03/29/2018
Copyright © 2024 VisualDx®. All rights reserved.
Retroperitoneal fibrosis
A medical illustration showing key findings of Retroperitoneal fibrosis : Abdominal pain, Fatigue, Flank pain, Low back pain, CRP elevated, ESR elevated
Copyright © 2024 VisualDx®. All rights reserved.