Table 3.

Paired comparisons of functional tests from baseline to various time points after sirolimus

Variable	6 month			12 months			≥15 months
	Baseline	After sirolimus	P value	Baseline	After sirolimus	P value	Baseline	After sirolimus	P value
Pulmonary function	(N = 14)			(N = 9)			(N = 9)
FEV1 (L)	1.00 ± 0.42	1.21 ± 0.47	0.002	1.56 ± 0.85	1.70 ± 0.66	0.272	0.99 ± 0.45	1.20 ± 0.59	0.007
FEV1/pred (%)	36.7 ± 16.8	44.5 ± 18.1	0.003	53.1 ± 29.0	58.5 ± 22.5	0.237	34.7 ± 14.2	43.3 ± 19.3	0.002
FVC/pred (%)	68.5 ± 26.0	83.0 ± 25.0	0.001	72.4 ± 20.7	82.6 ± 12.5	0.059	62.8 ± 16.6	84.7 ± 17.3	0.007
Arterial blood gas	(N = 21)			(N = 16)			(N = 14)
PaO2 (mmHg)	63.8 ± 9.2	70.1 ± 14.0	0.017	65.6 ± 13.0	67.9 ± 15.0	0.595	62.1 ± 8.7	71.4 ± 11.2	0.001
PaCO2 (mmHg)	34.2 ± 3.1	34.5 ± 3.5	0.980	32.9 ± 3.7	31.6 ± 7.3	0.970	33.3 ± 4.3	34.5 ± 4.3	0.238
P(A-a)O2 (mmHg)	43.1 ± 10.1	43.1 ± 10.1	0.293	45.1 ± 12.7	39.1 ± 16.6	0.083	48.4 ± 10.5	39.5 ± 15.0	0.011
6-min walk test	(N = 22)			(N = 18)			(N = 13)
6MWD (m)	352.5 ± 143.5	442.2 ± 103.9	< 0.001	367.3 ± 135.5	448.7 ± 100.9	0.001	320.7 ± 138.0	423.3 ± 127.1	0.016
St. George’s Respiratory Questionnaire	(N = 27)			(N = 20)			(N = 16)
Symptoms score	52.7 ± 23.7	37.6 ± 25.4	0.001	48.1 ± 24.8	32.0 ± 26.7	< 0.001	51.4 ± 21.1	29.4 ± 17.1	< 0.001
Activity score	68.1 ± 26.0	56.1 ± 25.4	< 0.001	63.8 ± 25.3	51.6 ± 24.7	0.009	70.9 ± 23.2	53.7 ± 25.0	0.001
Impacts score	53.1 ± 27.0	37.2 ± 27.0	< 0.001	48.4 ± 25.3	32.5 ± 22.9	0.001	55.8 ± 23.7	33.2 ± 21.7	0.001
Total score	57.6 ± 24.8	43.0 ± 23.7	< 0.001	53.2 ± 23.9	38.2 ± 23.0	< 0.001	59.8 ± 21.9	38.8 ± 19.6	< 0.001

Abbreviations: FEV₁ forced expiratory volume in 1 s, FVC forced vital capacity, P_aO₂ partial pressure of oxygen in arterial blood, P_aCO₂ partial pressure of carbon dioxide in arterial blood, P_(A-a)O₂ alveolar-arterial oxygen gradient, 6MWD six-minute walking distance

Data are presented as means±SD