Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study

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-functions^a
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 manifestations^b	22 (22.4)
Renal angiomyolipoma	4 (4.1)
Early treatment^c	4 (4.1)
Unknown reasons^d	3 (3.1)
Types of sirolimus used^e
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

ISSN: 1750-1172