Clinical Scenario
This protocol targets idiopathic retroperitoneal fibrosis confirmed to be unrelated to IgG4-related disease, in patients with concurrent ureteral obstruction. Long-term immunosuppression alongside urinary drainage is frequently required to protect renal function in this population.
Prior Treatment — Escalation Trigger
First-line management combining urinary drainage (ureteral stenting or percutaneous nephrostomy) with a prednisolone-based induction and taper course has not achieved the required treatment goals: reduction of retroperitoneal plaque on CT and resolution of ureteral obstruction. This protocol addresses the next step after that failure.
Approach in This Protocol
For glucocorticoid-refractory disease, there is no single agreed salvage regimen. The protocol incorporates alternative immunosuppressive agents — agents whose benefit in this specific setting has been reported — with structured criteria for selection and follow-up. Full details, including the specific agents considered and sequencing, are available in the structured protocol.
References
DOI: 10.1111/iju.14218
The same protocol of glucocorticoid therapy for systemic IgG4RD can apply to idiopathic RPF that does not fulfill the criteria of IgG4RD.
Generally, in idiopathic RPF causing ureteral obstruction, a good outcome for renal function is expected, although long-term steroid therapy along with urinary drainage is often required.
For glucocorticoid-refractory disease, there is no consensus about salvage treatment.
The effectiveness of other immunosuppressive drugs, such as mycophenolate mofetil, cyclophosphamide, azathioprine, methotrexate and tacrolimus, has been reported.
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