Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis

Respiratory Research

Table 3 Prognostic factors for mortality in patients with LAM assessed using the Cox proportional analysis

Characteristics	Unadjusted		Multivariable model-1		Multivariable model-2
Characteristics	HR (95% CI)	P-value	HR (95% CI)	P-value	HR (95% CI)	P-value
Age	1.046 (0.993–1.102)	0.090	1.136 (1.016–1.269)	0.025	1.167 (1.036–1.314)	0.011
Ever-smoker	1.388 (0.172–11.178)	0.758
TSC	0.949 (0.117–7.689)	0.961
Pneumothorax	0.920 (0.246–3.439)	0.902
Angiomyolipoma	1.149 (0.285–4.627)	0.845
FEV₁	0.956 (0.927–0.984)	0.003	0.956 (0.919–0.995)	0.026
FVC	1.000 (0.954–1.048)	0.996
DLCO	0.946 (0.914–0.979)	0.001			0.914 (0.861–0.969)	0.003
FEV₁/FVC ratio	0.947 (0.919–0.976)	< 0.001
RV	1.007 (0.996–1.019)	0.214
FEF_25–75	0.946 (0.936–0.993)	0.014
6MWD	0.992 (0.987–0.996)	< 0.001	0.993 (0.987–0.999)	0.020	0.997 (0.992–1.002)	0.247
6MWT, the lowest SpO₂, %	0.921 (0.868–0.977)	0.006	1.007 (0.908–1.117)	0.890	0.994 (0.902–1.095)	0.905
Sirolimus therapy	0.584 (0.155–2.195)	0.426

FEV₁ and DLCO were separately included in the multivariable models due to the high correlation between them (r = 0.705). FEV₁/FVC (r = 0.803), and FEF_25-75 (r = 0.767) were not included in the multivariable models due to their correlation with FEV₁
CI confidence interval, DLCO diffusing capacity of the lung for carbon monoxide, FEV₁ forced expiratory volume in 1 s, FVC forced vital capacity, RV residual volume, FEF_25–75% forced expiratory flow between 25 and 75% of FVC, HR hazard ratio, LAM lymphangioleiomyomatosis, SpO₂ oxygen saturation, TLC total lung capacity, TSC tuberous sclerosis complex, 6MWD 6-min walk distance, 6MWT 6-min walk test

ISSN: 1465-993X