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
Differential Diagnosis & Pitfalls
Drug Reaction Data