The clinical analysis of 9 patients with primary retroperitoneal fibrosis and literature review / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical features of primary retroperitoneal fibrosis (RPF), and to find out better method for diagnosis and therapy. Methods Nine patients with primary RPF in Peking Union Medical College Hospital since 1990 to 2003 were analyzed retrospectively. Results All patients had abnormal findings in CT or MRI, and 8 of them had chronic non-specific inflammation of the retroperitoneum in pathology. Six patients had renal involvement and 2 of them had severe renal failure. Eight of the patients had received therapy with corticosteroid or tamoxifene. Conclusions RPF is an uncommon collagen vascular disease characterized by a chronic non-specific inflammation of the retroperitoneum.Because of the atypical manifestations of RPF, awareness of the disease is important.CT and (or) open biopsy remains the gold standard for diagnosis.Management typically includes surgery and drug treatment.Corticosteroid therapy or more recently,tamoxifen has been used successfully. The clinical and radiographic improvement with drug treatment has been seen in several patients and confirmed the diagnosis,thereby early diagnosis and treatment can bring the patient more excellent renal and general outcome.