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
VariablesLAM with PHControl LAM without PHp-value
Patients2072
Age mean yrs49±12 (33–73)44±19 (33–73)0.362
Post-menopausal10 (50)30 (71)0.612
Smoker0.644
 Current02
 Ex-smoker522
 Never smoked1548
LAM diagnosis1
 Definite1966
 Probable16
Lung biopsy with LAM13 (65)41 (57)0.612
Tuberous sclerosis complex2 (10)12 (17)0.726
Renal angiomyolipoma9 (45)32 (44)1
History of pneumothorax#8 (40)36 (50)0.460
History of chylothorax5 (25)13 (18)0.529
History of chylous ascites2 (10)5 (7)0.643
Lymphangioleiomyoma3 (15)20 (28)0.382
Lymph node involvement (pathology)1 (5)6 (8)1
Dyspnoea20 (100)53 (74)0.009
NYHA functional class<0.001
 I011
 II122
 III1018
 IV94
Haemoptysis2 (10)0 (0)0.045
Finger clubbing2 (10)NANA
History of right heart failure or lower limb oedema4 (20)0 (0)0.002
Syncope at exercise0 (0)0 (0)1
Body mass index21±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.