Clinical Analysis Of 29 Patients With Retroperitoneal Fibrosis

Objective:To analyze and summarize the clinical characteristics,diagnosis,treatment and prognosis of 29 patients with retroperitoneal fibrosis(RPF),so as to provide clinical data for the identification and therapy of RPF.Methods:The clinical data were collected retrospectively from 29patients with established diagnosis of RPF,in Second Hospital of Hebei Medical University,including clinical manifestations,laboratory examination,radiological features,pathological changes,diagnosis and treatment.Meanwhile their prognosis was followed up.Results:1. The majority of patients in this study was in middle and old age,with a median age of 56 years,among which there 17 males and 12 females,and the average disease duration is 23 months.Lumbar and abdominal pain was the most common clinical manifestation.Ureteral obstruction occurred in 19patients.And there were 10 cases of bilateral hydronephrosis and 9 cases of unilateral hydronephrosis.2. All patients done abdominal computed tomography(CT),which showed homogeneous tissue with isodensity with muscle surrounding the abdominal aorta and iliac arteries,and different degrees of enhancement could be detected after contrast.2 patients’mesenteric artery were involved.PET-CT examination was performed in 5 patients,showing high metabolic soft tissue density,and the maximum standard uptake value is 4～8.Four patients took tissue biopsy,and the pathological changes were mainly fibrous tissue hyperplasia and inflammatory cells infiltration,Ig G4~+plasma cells accounted for more than 40%of Ig G~+plasma cells,meanwhile their serum Ig G4 was elevated.3. Six patients had malignant tumors,and the remaining 23 were considered as idiopathic retroperitoneal fibrosis(IRF),of which 4 patients were diagnosed with Ig G4-related RPF.Treatment methods of IRF included glucocorticoids combined with cyclophosphamide,tamoxifen or other medicine,indwelling ureteral stents(double J tubes)and so on.And most of them removed their ureteral stents within 1～6 months during the 5 years follow-up,and glucocorticoids were tapered and stopped within 2 years.However,a few patients developed into chronic renal failure.Patients with malignant tumor had a shorter survival period of about 3 months to 1 year.No recurrence was found.Conclusions:RPF can occur in both male and female,and the first symptom is mainly lumbar and abdominal pain,and the ureter often involved.Lesion Ig G4 detection of serum and pathological tissue,together with abdominal CT are important investigations.PET-CT can help to distinguish the lesion range,benign or malignant nature.Except the patients secondary to malignancy,most of patients with RPF have a good prognosis after early diagnosis and timely treatment.The combination treatment of glucocorticoids and cyclophosphamide,tamoxifen or other drugs could possiably reduce the recurrence rate of the disease.