Adult patients with confirmed diagnosis of Fabry disease, who are receiving enzyme replacement therapy with agalsidase beta

Inclusion Criteria:

• The patient must provide written, informed consent, and be ≥ 19 yrs of age.
• The patient is already receiving Fabrazyme® at 1 mg/kg every two weeks at the time of enrollment.
• Patient has confirmed Fabry disease (plasma αGAL activity of < 1.5 nmol/hr/mL, or leukocyte αGAL activity of < 4 nmol/hr/mg), or a known mutation compatible with Fabry disease.

• Patients with either:

  1. eGFRMDRD ≥ 20 and ≤ 60 ml/min/1.73 m2, and documented baseline urine protein/creatinine ratio > 0.5, based on the last value obtained before initiating ACEI/ARB therapy or obtained at screening before the first Evaluation Visit of the FAACET Study; or
  2. eGFRMDRD ≤ 125 ml/min/1.73 m2 and > 60 ml/min/1.73 m2 with documented baseline urine protein/creatinine ratio > 1, based on the last value obtained before initiating ACEI/ARB therapy or obtained at screening before the first Evaluation Visit of the FAACET Study.

Exclusion Criteria:

• The patient has undergone kidney transplantation or is currently on dialysis, or is planning on receiving a kidney transplant during the first year of the study.
• The patient has diabetic nephropathy or the presence of another, confounding kidney disease unless there is kidney biopsy confirmation that the patient does not have diabetic nephropathy or another, confounding kidney disease.
• The patient has a clinically significant organic disease, or other condition that in the opinion of the investigator would preclude participation in the full extent of the trial.
• The patient is unwilling to comply with the requirements of the protocol, including continuing on Fabrazyme® at 1 mg/kg body weight every two weeks.
• Patients who have documented allergies to ACE inhibitors and to ARBs are not eligible to participate in the FAACET Study.
• The patient is pregnant or intends to become pregnant during the course of the study.