Retroperitoneal fibrosis: a clinical and outcome analysis of 58 cases and review of literature.
The purpose of the study was to investigate the clinical features and outcomes of retroperitoneal fibrosis (RPF). Fifty-eight RPF treatment cases in the First Affiliated Hospital of China Medical University were retrospectively analyzed, including clinical characteristics and laboratory data. RPF was found predominantly in elderly men with atypical clinical manifestations of back pain, abdominal pain, and lower limb edemas. In laboratory examinations, the acute-phase reactants such as erythrocyte sedimentation rate and C-reactive protein levels increased significantly. Renal function failure was frequently found in patients with urethral obstruction. All patients had retroperitoneal soft tissue shadows or urethral obstructions on computed tomography (CT) or magnetic resonance imaging (MRI), seven of which had histological diagnosis of idiopathic RPF. Forty-two patients received surgical interventions; 29 patients received medication treatment alone including corticosteroids, immunosuppressants, and tamoxifen; 17 patients received corticosteroids after surgical intervention. Surgery followed by medication was most effective for RPF. CT and MRI help to exclude secondary causes, but biopsy remains the gold standard for diagnosis. Long-term low-dose corticosteroids and immunosuppressants may prevent relapse of RPF.
Department of Rheumatology and Immunology, The 1st Affiliated Hospital of China Medical University, Shenyang, 110001, China, staronlyone@163.com.
Hang
H
Liu
Gejun
G
Zhang
Yimeng
Y
Niu
Nan
N
Jiang
Weiguo
W
Xiao
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002681:China / Q000453:epidemiology; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006403:Hematologic Tests; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012008:Recurrence; D012074:Remission Induction; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D012307:Risk Factors; D013514:Surgical Procedures, Operative; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents