From the Guidelines
The management of retroperitoneal fibrosis should prioritize a comprehensive approach including diagnostic workup, medical therapy, and sometimes surgical intervention, with a focus on relieving urinary obstruction and preserving renal function, as supported by recent guidelines such as the ACR Appropriateness Criteria 1. The initial workup for retroperitoneal fibrosis involves diagnostic imaging, including contrast-enhanced CT or MRI of the abdomen and pelvis to assess the extent of fibrosis and any ureteral obstruction 1. Key laboratory tests include ESR, CRP, creatinine, and IgG4 levels to determine if it's IgG4-related disease. First-line treatment typically involves glucocorticoid therapy, such as prednisone 40-60 mg daily for 4-8 weeks, followed by a slow taper over 6-12 months. For cases with urinary obstruction, percutaneous antegrade ureteral stenting (with or without safety nephrostomy) is an effective management option, as it has been shown to have similar incidence of postprocedural complications compared to other methods 1. In cases of steroid-resistant or relapsing disease, immunosuppressive agents like mycophenolate mofetil, azathioprine, or methotrexate may be added, and tamoxifen may be used as steroid-sparing therapy. Urgent urological intervention with ureteral stenting or nephrostomy is necessary for obstructive uropathy causing renal impairment, and surgical ureterolysis may be required in cases with persistent obstruction despite medical therapy. Treatment response should be monitored with serial imaging every 3-6 months initially, along with renal function tests and inflammatory markers, to prevent progression of fibrosis, preserve renal function, and relieve symptoms. Some key points to consider in the management of retroperitoneal fibrosis include:
- The importance of early diagnosis and intervention to prevent long-term renal damage
- The need for a multidisciplinary approach, involving radiology, urology, and nephrology
- The role of medical therapy in managing the inflammatory component of the disease
- The importance of monitoring treatment response and adjusting therapy as needed to achieve optimal outcomes.
From the Research
Diagnosis and Management of Retroperitoneal Fibrosis
- Retroperitoneal fibrosis (RPF) is a condition characterized by inflammation and fibrosis in the retroperitoneal space, with no standard diagnostic criteria existing 2.
- The management of RPF has shifted from a primarily surgical approach to an immunosuppressive-based therapy aimed at modulating the immune system 2.
Treatment Options
- A combination of mycophenolate mofetil and prednisone has been shown to be an efficacious and safe treatment for patients with RPF 3.
- Tamoxifen, an antiestrogen drug, has been reported to be effective in treating RPF, particularly in cases resistant to corticosteroids 4.
- Steroid treatment response seems to depend on the association of RPF with IgG4-related disease, with cases associated with IgG4-RD showing a dramatic response to steroid therapy 5.
Current Approach to Management
- The current approach to diagnosis and management of RPF involves assessing systemic lesions associated with IgG4-related disease and excluding secondary RPF 6.
- Histological examination of retroperitoneal lesions is desired for accurate diagnosis and management, with laparoscopic or open biopsy often being beneficial 6.
- Treatment for idiopathic RPF consists of meticulous glucocorticoid therapy, with ureteral obstruction usually managed with conservative procedures such as ureteral stenting or percutaneous nephrostomy 6.