Table 1 Clinical, functional and LHS data of patients with LAM

Age

39

39.5 ± 8.2

Smoking status- n (%)

39

- Former-smokers

- Current smokers

6 (15.4)

1 (2.5)

Dyspnea - n (%)

39

18 (46.1)

Pneumothorax - n (%)

39

16 (41.0)

Angiomyolipoma - n (%)

39

11 (28.2)

Tuberous Sclerosis - n (%)

39

4 (10.3)

Treatment at the time of surgery - n (%)

- Sirolimus

- Inhaled corticosteroids

- Long-acting bronchodilator

2 (5.1)

2 (5.1)

6 (15.4)

Pulmonary function test

FEV1 (L)

38

1.8 ± 0.8

FEV1 (% predicted)

38

62.4 ± 30.0

FVC (L)

38

2.8 ± 0.8

FVC (% predicted)

37

80.7 ± 24.7

FEV1/FVC

38

0.6 ± 0.2

DLCO (mL/min/mmHg)

33

16.2 ± 8.2

DLCO (% predicted)

34

62.5 ± 31.8

TLC (L)

33

5.2 ± 1.1

TLC (% predicted)

34

105.4 ± 17.7

RV (L)

33

2.2 ± 1.0

RV (% predicted)

34

153.7 ± 59.1

RV/TLC

33

0.4 ± 0.1

LAM histological score (LHS)

Up to 50%

38

22 (57.9%)

Over 50%

38

16 (42.1%)