Retroperitoneal fibrosis: case series of five patients and review of the literature

Shiber, Shacahf; Eliakim‐Raz, Noa; Molad, Yair

doi:10.1016/j.rbre.2014.09.006

Cited by 13 publications

(17 citation statements)

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“…The possibility of a genetic predisposition was studied by De Luca [18,19] who showed the presence of the histocompatibility antigen HLA B27 in 44% of the cases. Thus, the existence of cases of familial RPF is in favor of this genetic predisposition [20,21].…”

Section: Discussionmentioning

confidence: 99%

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Retroperitoneal Fibrosis: Case Series of 20 Patients

Said¹,

Ghoundale²,

Zakaria³

et al. 2017

SJPH

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Abstract:Retroperitoneal fibrosis (RPF) is a rare disease. The objective of this paper is to study the prevalence, clinical, biological and radiological aspects of the RPFand to specify its therapeutic modalities. This study provides descriptive evidence for a series of 20 patients with RPF. We conducted a retrospective study of cases of RPF diagnosed in the Urology and Internal Medicine departments of the Ibn Sina Military Hospital in Marrakech over a five-year period from July 2010 to December 2015. The positive diagnosis of RPF was retained either on the basis of a histological analysis from a biopsy fragment or, in the absence of histological evidence, by the detection on a scan of an infiltrate or "sleeve" Regular and homogeneous tissue density, perivascular topography, surrounding the abdominal aorta, more or less extended to the iliac vessels and able to take the contrast after injection. They were 15 men and 5 women with an average age of 53 years with extremes of 41 to 74 years. Almost all patients had mainly lumbar and abdominal pain. An inflammatory syndrome existed in all cases and renal insufficiency in 15 cases. Radiological investigations showed unilateral or bilateral hydronephrosis in 16 cases. RPF was idiopathic in 14 patients. Secondary forms were present in 6 patients (three atheromatous diseases, one gastric tumor, one case associated with Riedel's thyroiditis and one case of systemic fibrosis associating RPF, Riedel's thyroiditis and liver fibrosis). The treatment consisted of corticosteroids in 19 cases, surgery in 17 cases associated with corticosteroid therapy or even immunosuppressant in the case of steroid-dependence. Two deaths were observed in this series; one of these two patients had developed cervix carcinoma with pleural and peritoneal metastases after 5 years of immunosuppressive therapy. The second died due to the complications of his gastric neoplasm. The general characteristics of patients included are similar to the other series of the literature. The frequency of secondary forms seems to be underestimated, hence the value of a rigorous etiological investigation. The therapeutic strategy and the use of the cortisone saving treatments should be specified by randomized therapeutic trials. The prognosis is generally good; however, the possibility of recurrences, the incidence of which is greater during the first five years, requires prolonged clinical, biological and radiological monitoring.

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Section: Discussionmentioning

confidence: 99%

“…RPF is idiopathic in 70% of cases [4,19], when no etiology is found. Secondary forms account for only about 30% of cases.…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal Fibrosis: Case Series of 20 Patients

Said¹,

Ghoundale²,

Zakaria³

et al. 2017

SJPH

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“…Bilateral ureteral obstruction can present with uremic symptoms. Entrapment of the inferior vena cava can present as lower extremity edema, scrotal edema, or deep vein thrombophlebitis of the leg (1, 6, 13). …”

Section: Discussionmentioning

confidence: 99%

“…The treatment of idiopathic RPF is empirical and based on the use of corticosteroids (2, 6, 13). Induction therapy with high-dose prednisolone (1 mg/kg/day), at a maximum dose of 80 mg/kg, is recommended for 4 - 6 weeks with tapering in approximately 1 - 2 years.…”

Section: Discussionmentioning

confidence: 99%

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Retroperitoneal Fibrosis Due to Opium Abuse: A Case Series and Literature Review

2016

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Retroperitoneal fibrosis (RPF) is a rare condition with an unclear etiology, presenting with the development of aberrant chronic nonspecific fibroinflammatory tissue in the retroperitoneal space, which can result in entrapment and obstruction of the retroperitoneal structures. RPF is a subtype of chronic periaortitis, and can be divided into two types: primary (or idiopathic) and secondary. RPF is usually idiopathic, but can also be secondary to malignancies, certain drugs, infections, surgery, and trauma. The systemic clinical manifestations are nonspecific and include low-grade fever, fatigue, anorexia, weight loss, and myalgia. We report five patients admitted to our hospital with clinical, laboratory, imaging, and pathologic findings compatible with RPF, and we describe their treatment and follow-up. We were suspicious that the impurities of some types of opium have an important role in the pathogenesis of RPF. Some of our patients used opium again after the follow-up period; however, they used a different type with a different origin, and we were surprised to see that RPF did not form again.

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