Demographic, physiological and radiological findings in 40 patients with tuberous sclerosis complex (TSC) and sporadic lymphangioleiomyomatosis (LAM) and 40 patients with sporadic LAM

DemographicsTSC–LAMSporadic LAM
Age years40.1±9.939.9±8.1
Follow-up time years6.5±2.76.5±3.2
Deaths2 (5)3 (7.5)
Lung function
 FEV1 L2.6±0.52.6±0.5
 FEV1 % predicted96.8±18.993.8±15.9
DLCO mL·min-1·mmHg-120.4±3.920.5±4.2
DLCO % predicted95.4±19.194.8±16.7
 Decline in FEV1 % predicted1.9±2.71.9±1.9
 Decline in DLCO % predicted2.1±2.81.9±2.7
Cardiopulmonary exercise testing
 Work rate % predicted99±25109±24
 Peak heart rate % predicted89±891±8
VEmax % predicted57±1662±16
 Breathing reserve %44±1638±20
VO2max % predicted80±1586±21
VE/VCO2 at anaerobic threshold32±734±6
Volume occupied by cysts
 Initial cyst scores %5.3±5.95.6±5.2
 Final cyst scores %11.8±9.6#15.3±9.8#
 Yearly change in cyst scores %1.0±1.31.4±1.7