Pulmonary Function in Familial Amyloidosis with Polyneuropathy

Abstract

Pulmonary function tests were performed in 14 patients with familial amyloidosis with polyneuropathy. Low maximum expiratory and inspiratory pressures were found in all patients with advanced polyneuropathy; the neuropathy in this disease evidently involves not only the extremities but also the respiratory muscles. The spirometric findings were consistent with a slight restrictive pattern in several patients, but in 2 of them the expiratory and inspiratory flows were low. The intrapulmonary gas distribution was uniform in all. The diffusing capacity was reduced in patients who had the disease for a long time, suggesting alveolocapillary block caused by amyloid deposits. The arterial O2 tension at rest was essentially normal in these patients.