Table 3

Multivariable analysis of estimated slopes by categorised covariates

VariableLevelsFEV1 (z)P valueFEV1/FVC (z)P value
SexFemale−0.025 (−0.062, 0.013)−0.044 (−0.089, 0.001)
Male−0.052 (−0.082, –0.022)0.19−0.070 (−0.106, –0.028)0.26
SmokingNever smoker−0.025 (−0.055, 0.005)−0.059 (−0.099, –0.020)
Smoker−0.073 (−0.109, –0.037)0.022−0.063 (−0107, –0.019)0.86
Lyso-Gb3, nmol/L<8.6−0.020 (−0.078, 0.038)0.65−0.042 (−0.101, 0.017)0.54
8.6–21.2−0.034 (−0.079, 0.011)−0.027 (−0.077, 0.024)
≥21.3−0.049 (−0.085, –0.011)0.52−0.073 (−0.116, –0.030)0.098
MSSI, points<6−0.044 (−0.096, 0.008)0.79−0.067 (−0.126, –0.010)0.15
6–15.6−0.037 (−0.083, 0.008)−0.021 (−0.075, 0.032)
≥15.7−0.042 (−0.079, –0.006)0.73−0.069 (−0.113, –0.026)0.053
Age at ERT initiation, years<35−0.013 (−0.063, 0.037)0.041−0.091 (−0.154, 0.028)0.17
35–45−0.056 (−0.107, –0.005)−0.037 (−0.101, –0.028)
≥46−0.031 (−0.078, 0.016)0.42−0.004 (−0.054, 0.062)0.28
  • Values are estimated slopes (95% CI) and P values, respectively. For covariates with three categories, the ranges for each category are lowest, mid (highest minus lowest), highest. Median (IQR) spirometric follow-up time was 7.7 (3.7, 10.1) years.

  • ERT, enzyme replacement therapy; MSSI, Mainz Severity Score Index.