Table 2

Univariable analysis of estimated slopes by categorised covariates

Variable	Levels	FEV₁ (z)	P value	FEV₁/FVC (z)	P value
Phenotype	Classic	−0.011 (−0.031, 0.008)		−0.027 (−0.048, –0.005)
Phenotype	Later-Onset	−0.027 (−0.082, 0.027)	0.55	−0.001 (−0.057, 0.055)	0.37
Sex	Female	0.005 (−0.020, 0.030)		−0.007 (−0.034, 0.021)
Sex	Male	−0.030 (−0.056, –0.004)	0.048	−0.034 (−0.065, –0.003)	0.17
Smoking	Never smoker	0.002 (−0.022, 0.025)		−0.022 (−0.051, 0.007)
Smoking	Smoker	−0.039 (−0.070, –0.008)	0.037	−0.023 (−0.060, 0.014)	0.95
Cardiac involvement	Yes	−0.043 (−0.073, –0.013)		−0.025 (−0.061, 0.011)
Cardiac involvement	No	0.001 (−0.030, 0.031)	0.033	−0.034 (−0.070, 0.002)	0.72
Lyso-Gb3, ng/mL	<8.6	0.012 (−0.030, 0.053)	0.59	−0.012 (−0.050, 0.026)	0.5
	8.6–21.2	−0.001 (−0.034, 0.032)		0.004 (−0.029, 0.037)
	≥21.3	−0.032 (−0.064, 0.000)	0.14	−0.048 (−0.082, –0.013)	0.022
α-Gal activity, %	<9	−0.040 (−0.084, 0.004)	0.096	−0.034 (−0.082, 0.014)	0.36
	Sep-40	0.013 (−0.036, 0.062)		−0.003 (−0.052, 0.047)
	≥41	0.007 (−0.045, 0.059)	0.85	0.010 (−0.041, 0.060)	0.75
Age at diagnosis, years	<25	−0.007 (−0.054, 0.040)	0.07	−0.091 (−0.150, –0.033)	0.13
	25–41	−0.056 (−0.096, 0.016)		−0.053 (−0.101, –0.004)
	≥42	−0.039 (−0.081, 0.002)	0.5	−0.033 (−0.081, 0.016)	0.5
MSSI, points	<6	−0.009 (−0.053, 0.035)	0.67	−0.024 (−0.069, 0.021)	0.15
	6–15.6	0.002 (−0.035, 0.040)		0.016 (−0.023, 0.055)
	≥15.7	−0.018 (−0.051, 0.014)	0.35	−0.050 (−0.084, –0.016)	0.007
Age at ERT initiation, years	<35	0.004 (−0.046, 0.054)	0.029	−0.061 (−0.128, 0.005)	0.95
	35–45	−0.059 (−0.098, –0.019)		−0.064 (−0.117, –0.011)
	≥46	−0.037 (−0.079, 0.006)	0.38	−0.007 (−0.063, 0.049)	0.093

Values are estimated slopes of z-values (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.
α-Gal, α-galactosidase A enzyme; ERT, enzyme replacement therapy; MSSI, Mainz Severity Score Index.