Retroperitoneal fibrosis: Single-centre experience from 1992 to 2010, current status of knowledge and review of the international literature

García, I. Gómez; Castaño, Ángel; Molina, Marta Romero; Hidalgo, Emilio Rubio; Betancourth, Natanael García; Gonzalez, R. Sigüenza; Crespo, Antonio Sampietro; Fernández, Fernando Álvarez; Herrero, Ángel Flores; Rodríguez, A. Gómez

doi:10.3109/00365599.2012.747564

Cited by 9 publications

(2 citation statements)

References 27 publications

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“…Its frequency ranges from 33 to 71% of cases. 17 , 19 , 20 , 25–29 IRPF patients with hydronephrosis had significantly higher serum creatinine levels than those without hydronephrosis. 17 In patients with IgG4-RD, those with RPF showed significantly higher serum creatinine levels compared with those without RPF although the influence of hydronephrosis on renal function was not evaluated.…”

Section: Laboratory Findingsmentioning

confidence: 84%

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Mizushima¹,

Kawano²

2021

IJNRD

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Retroperitoneal fibrosis (RPF) is a rare disorder consisting of idiopathic and various secondary forms and characterized by chronic inflammatory infiltrates and marked fibrosis in the retroperitoneal space. In idiopathic RPF (IRPF), 35–60% of cases have been reported to be IgG4-related RPF, the retroperitoneal lesions of IgG4-related disease (IgG4-RD). IRPF can frequently lead to renal insufficiency mediated by urinary tract obstruction and hydronephrosis irrespective of being IgG4-related or not. Clinical pictures, laboratory and imaging findings, and location of the urinary tract obstruction are generally similar in IgG4-related and non-IgG4-related IRPF although multiple organ involvement and serum IgG4 elevation may be characteristic of the IgG4-related forms. Periaortic/periarterial lesions are the most frequent cause of renal insufficiency. Although the response to glucocorticoids is generally good, relapse does occur in a considerable proportion of patients, and may require an additional immunosuppressive agent and/or urological intervention in cases with multiple relapses or refractory obstructive uropathy. In general, the prognosis of patients with IRPF is good, but careful attention needs to be paid to chronic kidney disease as a major complication and rupture of the affected aorta/artery as a life-threatening one. Further studies are necessary to better understand the pathogenesis of the disease and to establish the optimal diagnostic and therapeutic strategies for it.

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Section: Laboratory Findingsmentioning

confidence: 84%

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Mizushima¹,

Kawano²

2021

IJNRD

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“…A Finnish retrospective case-control study estimated an age-standardized incidence of 0.10 cases/100 000 person-years and a prevalence of 1.4 affected/100 000 inhabitants [7]. The disease occurs more frequently between the fifth and the seventh decade of life, and shows male predominance with a male-to-female ratio varying between 2:1 and 3:1 [8][9][10].…”

Section: E P I D E M I O L O G Ymentioning

confidence: 99%

Idiopathic retroperitoneal fibrosis: an update for nephrologists

Raglianti

Rossi

Vaglio

2020

Nephrology Dialysis Transplantation

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Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications of IRF, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. IRF may be isolated or develop in association with autoimmune diseases (e.g. Hashimoto’s thyroiditis and psoriasis) and other fibro-inflammatory disorders (often within the spectrum of immunoglobulin G4-related disease), which suggests that it should be considered as a potentially systemic condition. IRF is an immune-mediated disease: genetic variants (e.g. human leukocyte antigen (HLA)-DRB1*03) and environmental agents (mainly exposure to asbestos and smoking) are strongly associated with an increased risk of developing the disease, while a complex network of chemokines (e.g. CXCL12 and C-C moti chemokine 11 (CCL11)) and cytokines [e.g. interleukin (IL)-6, IL-12 and IL-13] is likely to orchestrate the inflammatory response and simultaneously promote fibrosis. Glucocorticoids, alone or in combination with traditional immunosuppressants such as methotrexate and mycophenolate mofetil, are usually efficacious and promptly induce disease remission; however, up to 50% of patients relapse, thus requiring repeat immunosuppressive courses. Biologic drugs, namely rituximab, are being explored for the treatment of IRF. In addition to medical therapies, interventional procedures (mainly ureteral stenting) are required to relieve ureteral obstruction, whereas surgical ureterolysis is generally reserved to refractory cases. If appropriately treated, then the overall and renal prognosis of IRF are good, with <5% patients developing end-stage renal disease.

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Idiopathic retroperitoneal fibrosis: a clinicopathological study in 24 Spanish cases

et al. 2013

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Idiopathic retroperitoneal fibrosis (IRPF) is a rare condition of unknown aetiology characterized by chronic non-specific inflammation of the retroperitoneum. The aim of this study is to describe, for the first time, the features of Spanish patients with IRPF. In this retrospective study, a clinical description was performed to examine the histopathological features, radiologic findings, laboratory data and treatment of a cohort of 24 IRPF Spanish patients who were admitted to Vall d'Hebron Hospital in Barcelona (Spain) between 1982 and 2009. Patients with secondary retroperitoneal fibrosis were excluded. Nineteen patients (79.1 %) were male, whereas 5 were female. The mean age at diagnosis was 51.8 ± 16.4 years. Pain (79.1 %) and hydronephrosis (70.8 %) were the most common clinical manifestations. Abdominal computed tomography and ultrasonography were performed on all cases. Acute-phase reactants were elevated in most patients. Thirteen surgical biopsies were performed, and all were consistent with retroperitoneal fibrosis. Steroid therapy was given in 19 cases, and eight patients (42.1 %) required urethral catheterisation. Chronic renal failure (CRF) rate after 2 years of follow-up was 42.8 %, more frequently in patients with higher serum creatinine at diagnosis. IRPF in Spain is a rare condition affecting mainly middle-aged males, with pain and hydronephrosis as the most frequent manifestations. Steroids are the mainstay treatment, and CRF is the main sequel. An earlier diagnosis and uniform treatment protocols could prompt lower CRF rates.

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Retroperitoneal fibrosis: Single-centre experience from 1992 to 2010, current status of knowledge and review of the international literature

Cited by 9 publications

References 27 publications

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Idiopathic retroperitoneal fibrosis: an update for nephrologists

Idiopathic retroperitoneal fibrosis: a clinicopathological study in 24 Spanish cases

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