To whom should a patient with retroperitoneal fibrosis be referred for management?

Referral for Retroperitoneal Fibrosis

Patients with retroperitoneal fibrosis should be referred to a urologist for initial management of ureteral obstruction and to a nephrologist for metabolic evaluation, immunosuppressive therapy guidance, and long-term kidney function monitoring.

Primary Specialist Referrals

Urology Referral

• Urologists manage the acute complications of retroperitoneal fibrosis, particularly ureteral obstruction requiring procedural intervention 1, 2
• Ureteral stenting or percutaneous nephrostomy placement is typically the first-line intervention to relieve obstruction and preserve renal function 1, 2, 3, 4
• Urologists perform definitive surgical procedures including ureterolysis with intraperitoneal displacement and omental wrapping when conservative management fails 3, 5
• Laparoscopic ureterolysis is an option for surgical management, though it requires specialized expertise and has an 85% success rate with 15% conversion to open surgery 5

Nephrology Referral

• Nephrology consultation is essential for patients with retroperitoneal fibrosis, as recommended by the American Society of Nephrology for recurrent or extensive nephrolithiasis and retroperitoneal conditions 6, 7
• Nephrologists manage the immunosuppressive therapy (corticosteroids with or without azathioprine) that forms the cornerstone of idiopathic retroperitoneal fibrosis treatment 2, 3, 4
• Patients with impaired renal function (eGFR <30 mL/min/1.73 m²) or persistent proteinuria require nephrology co-management 6
• Nephrology referral is indicated for metabolic evaluation and assessment of systemic involvement, particularly when IgG4-related disease is suspected 2

Multidisciplinary Management Approach

Initial Assessment Team

• A multidisciplinary sarcoma team should evaluate retroperitoneal masses before intervention, as patients with suspected retroperitoneal pathology need referral to high-volume specialized centers 1
• Radiologists with expertise in cross-sectional imaging (CT and MRI) are essential for diagnosis and differentiation of benign from malignant retroperitoneal fibrosis 8
• Pathologists are needed for tissue diagnosis, as histological examination is mandatory to exclude secondary causes and confirm IgG4-related disease 2

Coordinated Care Structure

• The multidisciplinary team should include urologists for procedural management, nephrologists for medical therapy and kidney function monitoring, and radiologists for imaging surveillance 6, 2, 8
• Rheumatology consultation may be beneficial when IgG4-related disease or other systemic inflammatory conditions are suspected 2
• Interventional radiology can provide image-guided biopsy (14-16 gauge core needle) for tissue diagnosis, though the biopsy pathway must be carefully planned to avoid transperitoneal contamination 1

Treatment Goals and Specialist Roles

Urologist's Role

• Relief of acute ureteral obstruction through stenting or nephrostomy placement 1, 2, 3
• Surgical ureterolysis when medical management fails or in cases of secondary retroperitoneal fibrosis after aortic surgery 3, 5
• Monitoring for recurrence, which occurs in only 5-8% of cases with combined medical-surgical management 3

Nephrologist's Role

• Initiation and monitoring of glucocorticoid therapy (prednisolone 1 mg/kg/day) with or without azathioprine (1 mg/kg/day) for 3-6 months 3, 4
• Assessment of renal function improvement, which occurs dramatically in most patients with conservative management 4
• Long-term follow-up to achieve freedom from stents/nephrostomy with withdrawal of glucocorticoids 2
• Management of complications including hypertension refractory to 4 or more antihypertensive agents 6

Common Pitfalls to Avoid

• Do not delay referral to specialized centers, as early combined medical and surgical management results in excellent long-term outcomes with recurrence rates of only 8% for idiopathic disease 3
• Avoid performing open or laparoscopic biopsies without multidisciplinary discussion, as the biopsy pathway must be carefully planned 1
• Do not assume all retroperitoneal masses are malignant; up to two-thirds of retroperitoneal fibrosis cases are idiopathic and respond well to medical management 2, 4
• Conservative management with ureteral stenting and steroids achieves symptom relief and improved renal function in 54% of patients without need for surgery 4
• Recognize that secondary retroperitoneal fibrosis (post-aortic surgery, radiation, drugs) may require different management strategies, with primary reconstructive surgery being most promising 3