Table 1.

Clinical data for five patients with lymphangioleiomyomatosis (LAM) and recurrent pneumothorax treated with sirolimus

Characteristics	Case 1	Case 2	Case 3	Case 4	Case 5
Age (years)	33	23	31	38	30
VEGF-D (pg/ml)	6608	2385	1139	3901	2685.8
PaO2(mmHg)	51	92	96	45.5	98
Surgery	No	No	Yes	Yes	Yes
Chemical pleurodesis	No	No	Yes	No	No
Number of PTX episodes before sirolimus therapy	6	3	6	6	2
Sirolimus concentration (ng/ml)	5–7	4–5	6–10	3–8	5.28a
Number of PTX episodes during sirolimus therapy	0	0	0	1	0
Follow-up 6MWT results (m)	480	550	555	510	500

^aOnly examined once

VEGF-D vascular endothelial growth factor-D, PTX pneumothorax, PFT pulmonary function test, 6MWT 6-min walk test, FVC forced vital capacity, FEV1 forced expiratory volume in 1 s, DLCO diffusing capacity for carbon monoxide