Table 3.

Multivariable analysis of estimated slopes by categorised covariates

Variable	Levels	FEV1 (z)	P value	FEV1/FVC (z)	P value
Sex	Female	−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
Smoking	Never 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.