Long-term efficacy and safety of sirolimus therapy in patients with lymphangioleiomyomatosis

Table 2 Changes in pulmonary function and other indicators per year before and after sirolimus treatment

Variables	Before treatment		After treatment		Before vs. After
Variables	Mean change per year	^§P value	Mean change per year	^§P value	P value
FEV₁(ml)	-178 ± 36	< 0.001	− 10 ± 15	0.53	< 0.001
FVC (ml)	−72 ± 68	0.29	54 ± 22	0.016	0.017
FEV₁%pred	−7.71 ± 1.20	< 0.001	0.29 ± 0.48	0.42	< 0.001
FVC%pred	−4.11 ± 1.15	0.009	2.78 ± 0.72	< 0.001	0.008
FEV₁/FVC (%)	−7.34 ± 1.08	< 0.001	−1.40 ± 0.40	< 0.001	< 0.001
DLCO%pred	−4.12 ± 1.10	0.002	−0.32 ± 0.37	0.40	0.017
PaO₂ (mmHg)	−5.7 ± 1.3	< 0.001	1.8 ± 0.5	< 0.001	0.002
P(A-a)O₂ (mmHg)	4.5 ± 1.3	0.002	−1.3 ± 0.5	0.012	0.002
6MWD (m)	−21 ± 6	< 0.001	15 ± 3	< 0.001	< 0.001
SGRQ total score	3.29 ± 2.02	0.11	− 2.65 ± 0.68	< 0.001	< 0.001
VEGF-D (pg/ml)	− 233 ± 185	0.22	− 555 ± 96	< 0.001	< 0.001

Data: mean ± SD. Data were obtained by using mixed-effects models. ^§P was calculated against a slope = 0. ^¶P was calculated by the slope before sirolimus therapy versus the slope after sirolimus therapy

ISSN: 1750-1172