Table 2.
Incidence rates of severe clinical events (renal event, cardiac event, stroke or death) per 1000 patient years while on agalsidase β by history of pre-ERT event, age and sex (177 severe clinical events were observed during 5 years maximal follow-up)
| Years on agalsidase β | |||
|---|---|---|---|
| 0–0.5 year | >0.5–1 year | >1–5 years | |
| Pre-ERT event: yes (n=172) | |||
| Severe events, n | 13 | 7 | 29 |
| Incidence rate*(95% CI) | 166 (88 to 283) | 102 (41 to 210) | 102 (68 to 146) |
| Pre-ERT event: no (n=872) | |||
| Severe events, n | 41 | 13 | 74 |
| Incidence rate*(95% CI) | 101 (72 to 137) | 37 (19 to 62) | 44 (34 to 55) |
| Age ≥40 years at first ERT (n=518) | |||
| Severe events, n | 42 | 15 | 69 |
| Incidence rate*(95% CI) | 177 (128 to 239) | 74 (42 to 123) | 79 (61 to 99) |
| Age <40 years at first ERT (n=526) | |||
| Severe events, n | 12 | 5 | 34 |
| Incidence rate*(95% CI) | 48 (25 to 85) | 22 (7 to 52) | 31 (22 to 43) |
| Men (n=641) | |||
| Severe events, n | 37 | 13 | 70 |
| Incidence rate*(95% CI) | 124 (87 to 171) | 49 (26 to 85) | 54 (42 to 68) |
| Women (n=403) | |||
| Severe events, n | 17 | 7 | 33 |
| Incidence rate*(95% CI) | 91 (53 to 145) | 43 (17 to 88) | 49 (34 to 68) |
*Per 1000 patient-years.
ERT, enzyme replacement therapy.