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Mechanistic Insight into the Development of TNBS-Mediated Intestinal Fibrosis and Evaluating the Inhibitory Effects of Rapamycin
Published on: September 12, 2019
Idiopathic retroperitoneal fibrosis: a role for mycophenolate mofetil
R D Swartz1, A M Lake, W W Roberts
1Division of Nephrology, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI 48109-0364, USA. rswartz@umich.edu
Purpose:
Idiopathic retroperitoneal fibrosis (IRPF) is an unusual progressive illness for which consistent therapeutic recommendations have not been devised. The present report describes a collaborative nephrology and urology approach to distinguish IRPF from secondary disease and then combine necessary acute surgical or radiological intervention with short-term corticosteroid and with mycophenolate mofetil (MM) to facilitate steroid tapering and long-term management.
Materials And Methods:
21 patients have been evaluated and followed over a 7-year period, 16 with characteristic IRPF and 5 with secondary retroperitoneal disease. IRPF patients initially received high-dose corticosteroid and MM. We report clinical follow-up along with imaging studies of the retroperitoneum and related organs, serologic markers for systemic disease, and nonspecific acute-phase reactants as indicators of ongoing disease activity.
Results:
Among IRPF patients, uniform success in stabilizing clinical signs and symptoms, radiological disease in the retroperitoneum and associated organs, and inflammatory indicators have been observed. Corticosteroid therapy can be limited to 6 months or less and MM to approximately 2 years, all with substantial impact on the natural history of IRPF.
Conclusions:
This is not a randomized, controlled trial, and patients were often referred with prior complications and/or treatments, however, the systematic approach and consistent results support the utility of MM as a safe and effective choice for long-term stabilization in IRPF.
Insights
Idiopathic retroperitoneal fibrosis (IRPF) management improved with a combined approach. Corticosteroids and mycophenolate mofetil (MM) offer a safe and effective strategy for long-term stabilization of IRPF.
Area of Science:
- Nephrology
- Urology
- Immunology
Background:
- Idiopathic retroperitoneal fibrosis (IRPF) is a rare, progressive condition lacking standardized treatment guidelines.
- Differentiating IRPF from secondary retroperitoneal disease is crucial for appropriate management.
- A multidisciplinary approach involving nephrology and urology is essential for complex cases.
Observation:
- A 7-year study evaluated 21 patients, 16 with IRPF and 5 with secondary disease.
- IRPF patients received high-dose corticosteroids and mycophenolate mofetil (MM).
- Clinical, radiological, and serological markers were used to assess disease activity and treatment response.
Findings:
- The combined therapeutic strategy demonstrated uniform success in stabilizing IRPF.
- Corticosteroid treatment was limited to 6 months, and MM to approximately 2 years.
- This approach significantly impacted the natural progression of IRPF.
Implications:
- Mycophenolate mofetil (MM) is a safe and effective option for long-term IRPF stabilization.
- The study supports a systematic approach to managing IRPF, even in patients with prior complications.
- This strategy allows for reduced corticosteroid duration, potentially minimizing side effects.
