PULMONARY MECHANICS IN LYMPHANGIOLEIOMYOMATOSIS

Standard pulmonary function and mechanics studies were done in eight female patients with lymphangioleiomyomatosis diagnosed by open-lung biopsy. Five patients were studied before hormone treatment. The mean age of the patients was 40 +/- 3 (SEM) years. Two of the eight had a smoking history of 10 pack-years or more, but all had quit smoking several years before evaluation. There was a trend toward increased total lung capacity (114 +/- 7% expressed as mean of percentage predicted +/- SEM) and increased residual volume (207 +/- 24%). Of the eight patients seven had expiratory obstruction as evidenced by the reduced forced expiratory volume in 1 s/forced vital capacity ratio ([FEV1/FVC] 61 +/- 6%). Steady-state diffusing capacity for carbon monoxide was reduced in seven of the patients (57 +/- 12%). Pulmonary mechanics studies performed in a body plethysmograph revealed a modest reduction in retractive force both at total lung capacity and at 90% total lung capacity (67 +/- 10 and 59 +/- 9%, respectively). Static compliance tended to be increased (128 +/- 19%). Pulmonary flow resistance was markedly elevated (266 +/- 46%). Maximal flow-static recoil curves revealed that in the seven patients with expiratory obstruction the cause was predominantly airway narrowing rather than loss of lung elastic forces. We conclude that in this group of patients with lymphagioleiomyomatosis there was no evidence for significant restriction. Although there was some decrease in retractive force consistent with emphysema, expiratory flows were reduced predominantly because of airway narrowing or obstruction rather than loss of pulmonary elastic recoil forces.