Este protocolo aborda el manejo de la fibrosis retroperitoneal idiopática en pacientes que no cumplen los criterios de la enfermedad relacionada con IgG4 y que presentan obstrucción ureteral que requiere intervención activa para proteger la función renal.
El paciente presenta fibrosis retroperitoneal idiopática confirmada en ausencia de enfermedad relacionada con IgG4, complicada por obstrucción ureteral. Se requiere un manejo a largo plazo para aliviar la obstrucción y preservar la función renal.
En pacientes seleccionados con esta presentación, puede llevarse a cabo un abordaje quirúrgico dirigido al uréter obstruido. Las indicaciones precisas, los criterios de selección de pacientes y la secuencia de manejo se detallan en el protocolo completo.
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.
View source ↗