TABLE 1

Demographic features of 554 patients with lymphangioleiomyomatosis (LAM)

DemographicsTSC–LAMSporadic LAM
Patients n94460
White79 (84)397 (86.3)
Black7 (7.4)26 (5.6)
Hispanic5 (5.3)10 (2.1)
Asian2 (2.1)25 (5.4)
Other1 (1.0)2 (0.4)
Age at LAM diagnosis years36.4±10.4*41.1±9.4
Age at first symptom years33.2±9.9*37.0±9.9
Age at first visit to NIH years39.1±10.2*43.9±9.2
Death during study14 (14.8)70 (15.2)
Lung transplantation during study4 (4.2)51 (11)
Initial symptoms
 Dyspnoea35 (37)222 (42)
 Cough019 (4)
 Pneumothorax38 (40)*13 (2.8)
 Haemoptysis3 (3)44 (10)
 Chylous effusions10 (11)33 (7)
 Abdominal, pelvic or back pain8 (9)59 (13)
 No respiratory symptoms19 (19)8 (2)
Extrapulmonary findings
 Lymphangioleiomyomas12 (12.8)177 (38)
 Angiomyolipomas90 (95.7)190 (41.3)
 Bilateral angiomyolipomas80 (85.1)61 (13.2)
 Chylous effusions, any time13 (13.8)93 (20)
Mode of diagnosis of LAM
 Tissue biopsy37 (39)308 (67)
 Clinical data and computed tomography57 (61)152 (33)
 Bronchodilator response107 (23)14 (15)
 Post-menopausal16 (17)105 (22.8)
Therapies received
 Progesterone/leuprolide47 (51)207 (45)
 Sirolimus8 (8.5)41 (8.9)