By A. Vaglio

 

The optimal treatment for ECD has not been established, since the therapeutic options are based on anecdotal experience. Corticosteroids have often been used as first-line therapy, and they are usually given in association with other immunosuppressive drugs (e.g. cyclophosphamide) 4,8. In some cases, chemotherapeutic agents like vinblastine have been used, but they are often reserved to patients who do not respond to first-line treatments 9. Tamoxifen is an anti-estrogen agent often used in patients with idiopathic retroperitoneal fibrosis and in different types of fibromatoses, but its use has never been reported in patients with ECD. Finally, radiation therapy has also been described in ECD, but in our patient it was not a feasible option on the basis of the widespread diffusion of the disease.

Thus, oral treatment with prednisone (1 mg/kg/day) and cyclophosphamide (2 mg/kg/day) was started in our patient; one month later, his clinical conditions considerably improved, the leg edema resolved and ESR and CRP levels normalised.

 

References
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Author for Correspondence:
Dr A. Vaglio
Department of Clinical Medicine, Nephrology and Health Science
University of Parma
Via Gramsci 14
43100 Parma
Italy
E-mail: bucamail@unipr.it