Two different enzyme preparations are used for the treatment of Fabry disease patients, agalsidase alfa (Replagal, Shire) and agalsidase beta (Fabrazyme, Genzyme). Therapeutic efficacy of both products has been variable probably due to differences in gender, severity, age and other patient characteristics. We studied the occurrence of
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-Gal A antibodies and their effect on urinary and plasma globotriaosylceramide (GL-3), plasma chitotriosidase and clinical outcome in 52 patients after 12 months of treatment with either 0.2 mg/kg agalsidase alfa (10 males, 8 females) or beta (8 males, 5 females) or 1.0 mg/kg agalsidase beta (10 males, 11 females). Antibodies were detected in 18/28 male patients after 6 months. None of the females developed antibodies. Following 12 months of 0.2 mg/kg treatment, urinary GL-3 decreased in antibody negative (AB−) but increased in antibody positive (AB+) patients.