Related Experiment Videos
[Idiopathic retroperitoneal fibrosis: clinico-pathological characteristics]
A Rodríguez Jornet1, F J Andreu Navarro, R Orellana Fernández
1Servicio de Nefrología, Corporación Sanitaria Parc Taulí, Sabadell. arodriguez@tauli.cat
Abstract:
Idiopathic retroperitoneal fibrosis is a rare disease often causing obstructive uropathy because the fibrosis entraps the ureters. The retroperitoneal tissue is constituted by a fibrous component and a chronic inflammatory infiltrate with the former characterized by miofibroblasts. The infiltrate displayed perivascular. Last years, the immunosuppressive therapy and the decompression of obstructive renal failure have improved the prognostic. We reported 9 patients with Idiopathic retroperitoneal fibrosis in a Centre. We evaluate clinical symptoms at the presentation with the signs of renal and non-renal involvement. We evaluate the achieved therapy and the follow-up. All 9 patients had a radiological and/or histological diagnosis in the absence of malignancy, previous radiotherapy or gadolinium's administration with severe renal failure. The patients were treated with 1 mg/kg/day of prednisone three months and possibly the insertion of ureteral catheters. 89% patients have a high rate of initial success without renal failure, despite frequent disease relapse. A patient developed progression of the fibrosis.
Insights
Idiopathic retroperitoneal fibrosis can cause obstructive uropathy. Immunosuppressive therapy with prednisone showed high initial success in managing patients, though relapses occurred.
Area of Science:
- Nephrology
- Urology
- Pathology
Context:
- Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by excessive fibrous tissue growth in the retroperitoneum.
- This fibrosis often leads to obstructive uropathy by entrapping the ureters, causing severe renal failure.
- Recent advancements include immunosuppressive therapy and surgical decompression to improve patient outcomes.
Purpose:
- To evaluate the clinical presentation, treatment, and outcomes of patients with idiopathic retroperitoneal fibrosis.
- To assess the efficacy of immunosuppressive therapy in managing IRF and its associated obstructive uropathy.
- To analyze the long-term follow-up and relapse rates in patients treated for IRF.
Summary:
- Nine patients diagnosed with idiopathic retroperitoneal fibrosis were retrospectively analyzed.
- Patients presented with varying degrees of renal and non-renal involvement, often with severe renal failure.
- Treatment involved prednisone (1 mg/kg/day for three months) and ureteral catheterization when necessary, achieving an 89% initial success rate in preventing renal failure, despite frequent relapses.
Impact:
- Highlights the effectiveness of immunosuppressive therapy in managing idiopathic retroperitoneal fibrosis and preventing renal failure.
- Emphasizes the need for long-term monitoring due to the high rate of disease relapse.
- Provides valuable data for clinicians managing this rare condition, informing treatment strategies and prognostic expectations.
Related Concept Videos
Pericarditis I: Introduction
Chronic Inflammation: Introduction
Chronic Pancreatitis I: Introduction
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
Chronic Pancreatitis I: Introduction
Nephrotic Syndrome I : Introduction
Rheumatic Heart Disease I: Introduction