Patients | 84 |
Age at LAM diagnosis years | 34.7±7.1 |
Age at first symptoms years | 32.0±7.2 |
History of pneumothorax | 48 (57) |
History of TSC | 18 (21) |
History of chylothorax | 19 (22) |
History of lymphangioleiomyomas | 26 (31) |
History of angiomyolipoma | 49 (58) |
History of anti-oestrogen therapy | 29 (34) |
History of pneumonia | 7 (8) |
Initial presentation | |
Dyspnoea | 25 (30) |
Pneumothorax | 33 (39) |
Haemoptysis | 7 (8) |
Abdominal pain | 5 (6) |
Cough | 4 (5) |
Chest pain | 3 (3) |
No symptoms | 7 (8) |
Mode of diagnosis | |
Lung biopsy | 43 (51) |
Abdominal mass biopsy | 6 (7) |
Characteristic CT and TSC | 10 (12) |
Characteristic CT and lymphangioleiomyoma | 8 (10) |
Characteristic CT and angiomyolipoma | 7 (8) |
Characteristic CT and chylous effusion | 1 (1) |
Characteristic CT scan and elevated serum VEGF-D levels | 2 (2) |
Data are presented as n, mean±sd or n (%). TSC: tuberous sclerosis complex; CT: computed tomography; VEGF: vascular endothelial growth factor.