TABLE 1

Demographic features of 84 premenopausal lymphangioleiomyomatosis (LAM) patients

Patients84
Age at LAM diagnosis years34.7±7.1
Age at first symptoms years32.0±7.2
History of pneumothorax48 (57)
History of TSC18 (21)
History of chylothorax19 (22)
History of lymphangioleiomyomas26 (31)
History of angiomyolipoma49 (58)
History of anti-oestrogen therapy29 (34)
History of pneumonia7 (8)
Initial presentation
 Dyspnoea25 (30)
 Pneumothorax33 (39)
 Haemoptysis7 (8)
 Abdominal pain5 (6)
 Cough4 (5)
 Chest pain3 (3)
 No symptoms7 (8)
Mode of diagnosis
 Lung biopsy43 (51)
 Abdominal mass biopsy6 (7)
 Characteristic CT and TSC10 (12)
 Characteristic CT and lymphangioleiomyoma8 (10)
 Characteristic CT and angiomyolipoma7 (8)
 Characteristic CT and chylous effusion1 (1)
 Characteristic CT scan and elevated serum VEGF-D levels2 (2)

Data are presented as n, mean±sd or n (%). TSC: tuberous sclerosis complex; CT: computed tomography; VEGF: vascular endothelial growth factor.