This protocol covers the management of idiopathic retroperitoneal fibrosis in patients who do not meet criteria for IgG4-related disease and who present with ureteral obstruction requiring active intervention to protect renal function.
The patient has idiopathic retroperitoneal fibrosis confirmed to be in the absence of IgG4-related disease, complicated by ureteral obstruction. Long-term management is required to relieve obstruction and preserve kidney function.
In selected patients with this presentation, a surgical approach directed at the obstructed ureter may be undertaken. The precise indications, patient selection criteria, and management sequence are detailed in the full protocol.
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.
In contrast, aggressive surgical treatment, such as ureterolysis, can achieve the goal; however, the procedure is associated with high morbidity.
In contrast, ureterolysis, an aggressive surgical treatment, is carried out to relieve ureteral obstruction and salvage renal function in selected patients, but the optimal indication for it is controversial.
The goal of treatment for retroperitoneal fibrosis should be freedom from the stent/nephrostomy with withdrawal of the glucocorticoid in addition to salvage of renal function; however, conservative management does not always provide favorable outcomes.
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