Successful treatment of Wegener granulomatosis is not without a price

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  • Successful treatment of Wegener granulomatosis is not without a price

    THERAPY

    The efficacy of cyclophosphamide and prednisone in the treatment of Wegener granulomatosis has been confirmed in a study by Dr Hoffman and colleagues from the National Institutes of Health, Maryland, USA.

    The study reported on over 24 years of experience in treating 158 patients with Wegener granulomatosis. Although treatment of this previously fatal condition with cyclophosphamide and prednisone ' ... hIlS been life-saving, extended follow-up hIlS led to recognition of a greater frequency of disease relapse, morbidity, and drug toxicity than had been previously recognized'.

    Complete remission was achieved in 75% of patients who received cyclophosphamide 2mgJkg and prednisone 1 mgJkg (with dosage adjustments as required); however, relapse occurred in 50% of these patients after 3 months to 16 years. Marked improvement or partial remission was achieved in 91 % of patients; the median time to remission was 12 months.

    Morbidity which could be attributed to the disease and/or treatment included chronic sinus dysfunction (47% of patients) and pulmonary insufficiency (17%). Solely treatment-induced adverse effects included cystitis (43% of patients), cataracts (21 %), fractures (11 %), diabetes mellitus (8%), bladder cancer (3%), and myelodysplasia (2%).

    13% of patients died during follow-up as a result of the disease or complications of therapy. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, et aI. Wegener granulomatosis: an analysis of 158 patients. Annals of Internal Medicine 116: 488-498, 15 Mar 1992 1019

    ISSN 0156-2703/92/0328-0017/$1.00/0 Adis Internatiollal lid

    L7

    INPHARMA. 28 Mar 1992

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