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Table 5 Comparison of clinical course between the sirolimus and non-sirolimus groups among patients with LAM

From: Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis

Characteristics

Sirolimus

Non-sirolimus

P-value

Number of patients

22

22

 

Death or lung transplantation

0 (0.0)

3 (13.6)

0.233

Disease progression

4/21 (19.0)

6/22 (27.3)

0.721

Lung function decline, % predicted/year

N = 17

N = 19

 

 FEV1, % predicted/year

0.5 ± 3.0

− 0.3 ± 4.7

0.452

 FVC, % predicted/year

0.7 ± 3.1

0.0 ± 4.1

> 0.999

 DLCO, % predicted/year

− 1.2 ± 3.9

0.6 ± 5.5

0.271

 RV, % predicted/year

0.1 ± 5.8

− 3.4 ± 15.3

0.485

 FEF25-75%, % predicted/year

− 1.3 ± 4.1

− 5.7 ± 13.9

0.808

Median follow-up period, year

8.5 (3.8–11.5)

6.1 (2.4–9.5)

0.213

  1. Data are expressed as the mean ± standard deviation, a number (%) or the median (interquartile range), unless otherwise indicated
  2. DLCO diffusing capacity of the lung for carbon monoxide, FEV1 forced expiratory volume in one second, FVC forced vital capacity, RV residual volume, FEF25–75% forced expiratory flow between 25 and 75% of FVC, IQR interquartile range, LAM lymphangioleiomyomatosis