[Is there an evidence-based management of idiopathic retroperitoneal fibrosis?].
BACKGROUND
Nowadays it is quite easy to diagnose idiopathic retroperitoneal fibrosis (IRF), particularly with the help of medical imaging. However there is no guideline about the treatment.


OBJECTIVE
Looking for data about an evidence-based management.


METHODS
Screening of the database Medline. Titles and abstracts of articles published between 01/01/1985 and 31/12/2004 have been read to identify clinical trials and series about more than ten patients.


RESULTS
No record of any therapeutic trials has been found. Eight series in total, which included 177 patients, were identified. Two of the patients have been treated by an ureteral desobstruction only (endoscopy or nephrostomy), 45 by surgery (ureterolysis), 65 by corticotherapy and 64 both by surgery and steroids. For 38 patients, immunosuppressive drugs were combined with corticotherapy (azathioprine, cyclophosphamide or D-penicillamine). According to the authors, doses and duration of corticotherapy varied. Median follow-up lasted 56 months. The outcome is satisfactory in 73% for surgery alone, 86% for medical treatment alone and 73% for both. The association between steroids therapy and immunosuppressive drugs is efficient in 97% of the cases. No clear data about side effects was mentioned.


CONCLUSIONS
Treatment of the IRF is still empirical, based on surgery and corticotherapy. There is no guideline about the treatment strategy. Although tamoxifen has been proposed, efficacy evidence is lacking. Prospective multicenter studies will help us to progress in the management of the IRF.
Maillart
E
E
Service de médecine interne, hôpital Ambroise-Paré, université Versailles Saint-Quentin-en-Yvelines, Assistance publique-Hôpitaux de Paris, 92100 Boulogne-Billancourt, France.
Lauerière
L
L
Kassis
S
S
Moulonguet-Doleris
L
L
Prinseau
J
J
Baglin
A
A
Hanslik
T
T
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D019317:Evidence-Based Medicine; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012185:Retroperitoneal Fibrosis / Q000150:complications / Q000628:therapy*; D016896:Treatment Outcome; D014517:Ureteral Obstruction / Q000209:etiology / Q000601:surgery
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents