Description

Vaglio et al proposed an algorithm for the management of a patient with retroperitoneal fibrosis. The authors are from the University of Parma and Arcispedale S Maria Nuova in Italy,


 

Parameters affecting management decisions:

(1) probable precipitating condition

(2) evidence of inflammation contributing to the process

(3) ureteral involvement

(4) course of the disease

 

Precipitating cause:

(1) If secondary to drug, then discontinue drug.

(2) If secondary to tumor, then treat the tumor.

(3) If secondary to infection, then treat with antibiotics.

(4) If due to a systemic automimmune disease, then treat with cytotoxic drugs or immunosuppression.

(5) If idiopathic, then manage based on the following parameters.

 

If there is evidence that inflammation is contributing to the process, then treat the patient with corticosteroids.

 

If there is ureteral involvement, then a ureteral stent or nephrostomy tube is placed at first. If the disease continues to progress then consider surgical ureterolysis.

 

If the disease remits, then therapy (immunosuppression, steroids, stent, etc) is withdrawn. If the disease persists or progresses then switch to immunosuppression or tamoxifen.

 


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