Clinical and Physiological Considerations in Pulmonary Muscular Hyperplasia

Abstract

Although localized muscular hyperplasia in the lung has frequently been noted in many types of chronic pulmonary disease, a generalized muscular hypertrophy is far more rare. Biopsies were performed in 6 cases, and the histo-logical sections were entirely comparable. Although some degree of interstitial fibrosis was uniformly present, the most striking feature was the degree of smooth muscle hyperplasia. Physiologically all subjects demonstrated a restrictive ventilatory pattern accompanied by the alveolar capillary block syndrome. Hyperventilation was marked, and exaggerated by exercise. Clinically, the presenting complaint in all cases is dyspnea. Cyanosis and clubbing are frequent, as is a distressing nonproductive cough. Right heart failure is a common sequel. The course of the disease is one of slowly but progressively unrelenting disability. The correlation between the pathological findings and the physiological data are discussed, as is the possible relationship of this syndrome to carcinoma of the lung.