Clinical analysis on 31 patients with idiopathic retroperitoneal fibrosis and literature review

Abstract

Objective To investigate and analyze the clinical features of patients with idiopathic retroperitoneal fibrosis (IRF) for improving clinical diagnosis and treatment. Methods The medical records of 31 cases diagnosed as IRF in Shanxi Dayi Hospital from 2011 to 2017 were reviewed retrospectively and summarized with recent relevant literatures. Results The average age of patients was 48 years old, ranging from 25 to 72 years old, with 19 males and 12 females. Among them, 3 men were defined as IgG4 related retroperitoneal fibrosis. Hydronephrosis to some extent was found in 28 patients, of which 23 patients were implanted with 37 uieteral stents (D-J tubes), 14 cases were with bilateral hydronephrosis, 9 cases were with unilateral hydronephrosis (two on the right and seven on the left). The reexamination by ultrasound or CT showed that the hydronephrosis was reduced or disappeared after surgery at 3 months, 6 months and 1 year after treatment. Five patients refused implanting D-J tube, of which three cases underwent reexamination of ultrasound for aggravated syndrome suggesting exacerbation of hydronephrosis, two human beings received remedial D-J tube implanting and the result was favorable, one case suffered secondary deep venous thrombosis and was given immunosuppressive agents for having no chance to implant tube, and the patient was lost after leaving hospital. It was only three cases who got complications such as lower fever and urinary tract irritative syndroms during the 1-year follow-up. After been diagnosed with urinary infection by bacterial culture, the 3 patients took sensitive antibiotics orally and then recovered. Thirty-one patients were treated with corticosteroids, of which 27 patients were treated by corticosteroidst associated with tamoxifen, 19 patients were treated with immunosuppressive agents; the D-J tubes were exchanged or extubated after half a year; twenty-eight tubes showed stone formulated on the wall of ureteral stents. No condition of extubating difficultly occured. On the basis of the drug therapy, the extubated rate at 6 months and 1 year was 16%(4/25) and 80%(20/25), the diffience was statistially significant(P<0.05). Conclusions IRF is rare in clinic and its prognosis is relatively good. The diagnosis of IRF is primarily based on histopathologic study. Glucocorticoid and immunosuppressive therapy are the basic treatments for IRF. It is convenient and safe to implant the D-J tube for patients with hydronephrosis in time, by which the incidence of complications are not increased, such as infection, hematuresis and so on, and it has good tolerability. The patients defined as IgG4 related retroperitoneal fibrosis are recommended to perfect the imageological examination of the whole body, lest missing foci. Key words: Ureteral stent; Retroperitoneal fibrosis; Immunoglobulin G