Table 1.
Baseline demographic and clinical characteristics of patients with lymphangioleiomyomatosis
| Characteristics | Numbers (%) |
|---|---|
| Patients numbers | 98 |
| Age-year | 21–62 |
| Gender | |
| Female | 98 (100) |
| Race | |
| Asian | 98 (100) |
| Diagnosis | |
| Definite LAM | 78 (79.6) |
| Probable LAM | 20 (20.4) |
| Clinical features | |
| Postmenopause | 5 (5.1) |
| Previous pregnancy | 76 (77.6) |
| Former smoker | 0 (0) |
| Renal angiomyolipomas | 19 (19.4) |
| Retroperitoneal angiomyolipomas | 2 (2.0) |
| History of pneumothorax | 42 (42.8) |
| History of chylothorax | 35 (35.7) |
| Pulmonary-functionsa | |
| FEV1 (L) | 1.51 ± 0.74 |
| FEV1/pred (%) | 49.03 ± 24.64 |
| FVC (L) | 2.55 ± 0.74 |
| FVC/pred (%) | 76.31 ± 22.99 |
| FEV1/FVC (%) | 51.22 ± 16.53 |
| PaO2 on room air (mmHg) | 65.26 ± 11.39 |
| Reasons for sirolimus-No. (%) | |
| FEV1 less than 70% predicted | 65 (66.3) |
| Chylothorax or other lymphatic manifestationsb | 22 (22.4) |
| Renal angiomyolipoma | 4 (4.1) |
| Early treatmentc | 4 (4.1) |
| Unknown reasonsd | 3 (3.1) |
| Types of sirolimus usede | |
| Rapamune | 76 (77.6) |
| Yixinke | 21 (21.4) |
| Saimosi | 1 (1.0) |
Abbreviations: LAM lymphangioleiomyomatosis, FEV1 forced expiratory volume in 1 s
aSample size for spirometry and blood gas analysis was 71 and 81 respectively
bIncluding measurable lymphangiomyomas, lymphangiectasis and lymphedema
cIncluding 3 patients with FEV1 annual decreases of more than 90 mL and 1 patient with normal and stable FEV1
dReason for sirolimus could not be identified because of insufficient data
eRapamune, Pfizer Pharmaceuticals Ltd.; Yixinke, North China Pharmaceuticals Co., Ltd.; Saimosi, Hangzhou Sino-American Pharmaceuticals Ltd