Skip to main content

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]