Retroperitoneal Fibrosis: Case Series of 20 Patients

Abstract

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.

Surviving Complications of Retroperitoneal Fibrosis with Intermittent Relapses over a Period of 20 years: Case report and literature Review

“…IRPF have long been associated with many auto-immune diseases, most common being Autoimmune Thyroiditis (Kaddouri et al, 2017). In a study, anti-thyroperoxidase antibodies were found in 24.7% of cases with evidence of thyroiditis on ultrasonography, 25% of them developed hypothyroidism requiring L-thyroxine.…”

“…Fibroblasts can be seen in stages of activation and transition into myofibroblasts (a smooth muscle actin expression), which form the major source of collagen. Though clonal proliferation was shown, mitoses occurred rarely (Vaglio & Moriatati, 2016, Kaddouri et al, 2017. Human Leukocyte antigen, HLA-DR (Human Leukocyte Antigen -DR isotype) may also be seen in many (Carmel et al, 2008).…”

“…With many possible hypotheses it has been seen to involve other structures either distant or adjacent (Carmel et al, 2008, Scheel et al, 2012. Some of the diverse manifestations may include mediastinitis, thyroiditis and sclerosing cholangitis and even ophthalmic tumors (Carmel et al, 2008, Kaddouri et al, 2017. Some investigators consider it to be part of IgG4-Related Disease (IgG4-RD) because of its association with many auto-immune disorders (Zhang et al, 2017, Hedgire et al, 2013, Khosroshahi et al, 2013, Subramani et al, 2019.…”