Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis

Respiratory Research

Table 4 Comparison of baseline characteristics between the sirolimus and the non-sirolimus groups among patients with LAM

Characteristic	Sirolimus	Non-sirolimus	P-value
Number of patients	22	22
Age, years	42.4 ± 3.2	40.8 ± 9.2	0.723
Female sex	22 (100)	22 (100)	> 0.999
Ever-smoker	1 (4.5)	3 (13.6)	0.607
TSC	2 (9.1)	3 (13.6)	> 0.999
Pneumothorax	12 (54.5)	9 (40.9)	0.365
Chylothorax	0 (0.0)	2 (9.1)	0.488
Extrapulmonary manifestations	7 (31.8)	10 (45.5)	0.537
Angiomyolipoma	7 (31.8)	7 (31.8)	> 0.999
Lymphangioleiomyoma	1 (4.5)	2 (9.1)	> 0.999
Lung function, % predicted
FEV₁	75.0 ± 18.0	78.1 ± 20.1	0.655
FVC	85.9 ± 12.9	87.1 ± 13.6	0.796
DLCO	60.9 ± 18.0	62.2 ± 15.5	0.655
TLC	97.5 ± 11.9	96.0 ± 12.5	0.879
FEV₁/FVC	87.5 ± 16.5	90.3 ± 20.0	0.348
RV	98.6 ± 23.3	101.4 ± 22.3	0.702
FEF_25–75%	75.4 ± 42.8	76.7 ± 47.2	0.930
6MWT
Distance, m	513.4 ± 79.6	508.0 ± 54.2	0.496
Lowest SpO₂, %	93.9 ± 4.0	95.7 ± 5.0	0.047

Data are expressed as the mean ± standard deviation or as a number (%), unless otherwise indicated. The index date was set as the date of the first prescription of sirolimus in the sirolimus group and the date of LAM diagnosis in the non-sirolimus group
DLCO diffusing capacity of the lung for carbon monoxide, FEV₁ forced expiratory volume in one second, FVC forced vital capacity, RV residual volume, FEF_25–75% forced expiratory flow between 25 and 75% of FVC, LAM lymphangioleiomyomatosis, TSC tuberous sclerosis complex, TLC total lung capacity, SpO₂ oxygen saturation, 6MWT 6-min walk test

ISSN: 1465-993X