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Table 1 Baseline demographic and clinical features of patients with lymphangioleiomyomatosis

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

Demographics

Numbers

Percentage or value

Total sample size

142

100%

Age (years)

 

38 ± 9

Sex

 Female

142

100%

Former smoker

0

0%

Complications

 Renal angiomyolipomas

33/142

23.2%

 Pneumothorax

40/142

28.2%

 Chylothorax

48/142

33.8%

 Chyloperitoneum

16/142

11.3%

CT gradinga

 I

7/142

4.9%

 II

9/142

6.3%

 III

119/142

83.8%

Pulmonary function

 FEV1 (ml)

114

1622 ± 712

 FVC (ml)

114

2760 ± 645

 FEV1% pred

114

58.5 ± 25

 FVC% pred

114

85 ± 20.6

 FEV1/FVC (%)

114

57.5 ± 17.9

 RV% pred

107

161.3 ± 79.3

 TLC% pred

107

116.8 ± 103.8

 RV/TLC (%)

107

46.25 ± 13.67

 DLCO% pred (N = 107)

107

40.7 ± 21.2

Arterial blood gas analysis

 PaO2 (mmHg)

122

72.3 ± 13.4

 P(A-a)O2 (mmHg)

114

43.4 ± 51.1

 Borg dyspnea index

132

2.5 ± 2.1

 6MWD (m)

133

422 ± 113

 SGRQ

132

 

 Symptoms score

132

40.4 ± 24.2

 Activity score

132

54.2 ± 24.9

 Impacts score

132

40.5 ± 25.8

 Total score

132

44.8 ± 23.5

Serum VEGF-D level (pg/ml)

140

3318 ± 2578

  1. Data: mean ± SD
  2. Abbreviations: 6MWD 6-min walking distance, DLCO Diffusion capacity for carbon monoxide, FEV1 Forced expiratory volume in 1 second; FVC Forced vital capacity, PaO2 Partial pressure of oxygen; P(A-a)O2 Alveolo-arterial oxygen partial pressure difference, RV Residual volume, SGRQ St.George’s Respiratory Questionnaire, TLC Total lung capacity, VEGF-D Vascular endothelial growth factor–D. a: According to the degree of lung involvement, CT was classified as I, II and III grades. Grade I was less than 1/3 of the whole lung field, Grade III was more than 2/3, Grade II was between 1/3 and 2/3 [16]