Treatment of Idiopathic Retroperitoneal Fibrosis Without IgG4-Related Disease Presenting With Ureteral Obstruction
This protocol covers the clinical management of idiopathic retroperitoneal fibrosis (RPF) in patients who do not fulfill criteria for IgG4-related disease and who present with ureteral obstruction requiring active intervention.
Idiopathic RPF without IgG4-related disease, complicated by ureteral obstruction. The therapeutic framework used in IgG4-related systemic disease can be extended to idiopathic RPF that does not meet IgG4-RD criteria. Long-term management — including urinary drainage — is frequently required to preserve renal function, although a good outcome is generally expected when the condition is identified and treated.
Initial management addresses urinary obstruction directly through a drainage procedure, followed by a structured course of glucocorticoid therapy. Maintenance treatment is continued beyond the induction phase, as premature discontinuation is associated with relapse.
References
- 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.
- At first, ureteral obstruction is usually conservatively managed with ureteral stenting or PNS.
- As aforementioned, the first-line treatment is glucocorticoid therapy, although the protocol is not standardized.
- After the introduction and subsequent tapering of the glucocorticoid, sufficient maintenance therapy with low-dose glucocorticoid might be necessary, because premature discontinuation is highly associated with relapse of disease, similarly to IgG4RD.