Abstract
A patient with mixed connective tissue disease (MCTD) presented the typical signs of respiratory muscle weakness and marked diaphragm dysfunction: reduced maximal ex- and inspirator) transrespiratory pressures (measured at FRO, maximal transdlaphragmatic pressures and transpulmonary pressures at TLC, FRC and RV. This Insufficiency of the diaphragm was also responsible for the restrictive ventilatory defect and the decreased static lung compliance with high diffusing capacity per unit lung volume.