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Retroperitoneal fibrosis
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Retroperitoneal fibrosis

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Contributors: Abhijeet Waghray MD, Khaled Bittar MD, Nishant H. Patel MD, Desiree Rivera-Nieves MD, Paritosh Prasad MD
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Synopsis

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.

Codes

ICD10CM:
N13.5 – Crossing vessel and stricture of ureter without hydronephrosis

SNOMEDCT:
49120005 – Retroperitoneal fibrosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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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.

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References

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Last Reviewed: 02/08/2018
Last Updated: 03/30/2018
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Retroperitoneal fibrosis
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Retroperitoneal fibrosis : Abdominal pain, Fatigue, Flank pain, Low back pain, Weight loss, CRP elevated, ESR elevated
Copyright © 2018 VisualDx®. All rights reserved.