Table 1– Characteristics, clinical manifestations and pulmonary function tests at diagnosis of pre-capillary pulmonary hypertension (PH) in 20 patients with lymphangioleiomyomatosis (LAM) compared with 72 patients with LAM without PH

Variables	LAM with PH	Control LAM without PH	p-value
Patients	20	72
Age mean yrs	49±12 (33–73)	44±19 (33–73)	0.362
Post-menopausal	10 (50)	30 (71)	0.612
Smoker			0.644
Current	0	2
Ex-smoker	5	22
Never smoked	15	48
LAM diagnosis			1
Definite	19	66
Probable	1	6
Lung biopsy with LAM	13 (65)	41 (57)	0.612
Tuberous sclerosis complex	2 (10)	12 (17)	0.726
Renal angiomyolipoma	9 (45)	32 (44)	1
History of pneumothorax^#	8 (40)	36 (50)	0.460
History of chylothorax^¶	5 (25)	13 (18)	0.529
History of chylous ascites	2 (10)	5 (7)	0.643
Lymphangioleiomyoma	3 (15)	20 (28)	0.382
Lymph node involvement (pathology)	1 (5)	6 (8)	1
Dyspnoea	20 (100)	53 (74)	0.009
NYHA functional class			<0.001
I	0	11
II	1	22
III	10	18
IV	9	4
Haemoptysis	2 (10)	0 (0)	0.045
Finger clubbing	2 (10)	NA	NA
History of right heart failure or lower limb oedema	4 (20)	0 (0)	0.002
Syncope at exercise	0 (0)	0 (0)	1
Body mass index	21±3 (14–30)	22±3 (16–37)	0.212

Data are presented as n, mean±sd (range) or n (%), unless otherwise stated. NYHA: New York Heart Association; NA: not available. ^#: bilateral in seven out of eight cases; ^¶: six other patients had a history of pleural effusion not otherwise specified.