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.