Idiopathic Retroperitoneal Fibrosis: Long-term Risk and Predictors of Relapse

Raffiotta, Francesca; Escoli, Rachele; Quaglini, Silvana; Rognoni, Carla; Sacchi, Lucia; Binda, Valentina; Moroni, Gabriella

doi:10.1053/j.ajkd.2019.04.020

Cited by 20 publications

(14 citation statements)

References 37 publications

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“…On the other hand, non-IgG4-related patients with IRPF had a comparable prevalence of hydronephrosis and/or ureter obstruction in the same studies, 2–4 and IRPF patients with unknown relationship to IgG4 also had a comparable prevalence in other studies. 24–30 Moreover, a larger series of 53 patients with IRPF showed that the prevalence of hydro-ureteronephrosis was 55%. 17 Therefore, the prevalence of urinary tract obstruction is high in IRPF irrespective of IgG4-related status.…”

Section: Prevalence Of Renal Involvement In Rpfmentioning

confidence: 99%

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: Prevalence Of Renal Involvement In Rpfmentioning

confidence: 99%

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

Mizushima¹,

Kawano²

2021

IJNRD

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“…Recently, several studies have reported the importance of postoperative continuous monitoring in patients with IRF. 19 , 20 Our case is categorized into the low-risk group in accordance with the criteria advocated by Moriconi et al. 20 Nonetheless, the 15-year cumulative incidence rate of IRF recurrence was estimated to be as high as 48% in a previous study.…”

Section: Discussionmentioning

confidence: 99%

“… 20 Nonetheless, the 15-year cumulative incidence rate of IRF recurrence was estimated to be as high as 48% in a previous study. 19 Therefore, we will continue periodic surveillance for our patient.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic retroperitoneal fibrosis mimicking infiltrative malignancy: a case report

et al. 2022

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The treatment strategy for an idiopathic retroperitoneal mass has not yet been established. Additionally, differentiating between benign and malignant is a challenge. Herein, we report a case in which we performed partial resection of a mass in a symptomatic patient with idiopathic retroperitoneal fibrosis that mimicked malignancy. A 44-year-old woman with an unremarkable medical history other than gallstones presented with a 1-month history of abdominal pain and repetitive vomiting. Imaging studies identified a large, retroperitoneal mass compressing the duodenum that had grown acutely over the preceding 2 weeks. The possibility that the mass was malignant could not be excluded. Considering the invasiveness and potential curability, we performed partial resection of the mass, which involved partial colonic resection with reconstruction, to allow for pathological diagnosis and intestinal obstruction treatment. The final pathological findings revealed that the mass consisted of hemorrhagic and fibrotic tissue without a tumorous component. The patient’s postoperative course was unremarkable. She is alive 8 years postoperatively with no recurrence. In conclusion, a surgical approach, including biopsies, to idiopathic retroperitoneal fibrosis that mimics malignancy should be actively considered in symptomatic patients. Decisions regarding the required degree of surgical intervention call for sufficient, case-specific discussion.

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“…The clinical presentation of RPF depends on the impact of fibrotic changes on the surrounding tissues [5,6]. The most common presentation includes unilateral or bilateral flank pain, lower abdominal pain, and body fatigue.…”

Section: Introductionmentioning

confidence: 99%

Unique Presentation of Idiopathic Retroperitoneal Fibrosis in a Primary Care Setting

Khalil¹,

Selvarajah²,

Solanki³

et al. 2021

Cureus

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Patients with retroperitoneal fibrosis (RPF), a rare condition, present with nonspecific abdominal pain or flank pain that can be complicated by urologic obstruction and/or vascular compromise. Reporting rare entities that often elude prompt diagnosis will aid clinicians to consider the entity in their differential diagnosis and potentially lead to earlier recognition and appropriate management. Our case emphasizes the importance of not just the diagnosis and treatment of RPF but how to best monitor RPF patients to minimize complications from disease progression or treatment-related adverse events.

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Idiopathic Retroperitoneal Fibrosis: Long-term Risk and Predictors of Relapse

Cited by 20 publications

References 37 publications

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

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

Idiopathic retroperitoneal fibrosis mimicking infiltrative malignancy: a case report

Unique Presentation of Idiopathic Retroperitoneal Fibrosis in a Primary Care Setting

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