[Pulmonary alveolar proteinosis].

ABSTRACT

Pulmonary alveolar proteinosis is a rare disease of unknown etiology characterized by alveoli filled with PAS-positive material high in protein and lipid. A 30-year-old man was referred because of respiratory failure. 2 years previously exertional dyspnea and productive cough appeared and gradually worsened. A year later he sought medical advice but defaulted on follow-up. After further deterioration he returned for treatment. On examination he had tachypnea and cyanosis. Diffuse crackles were audible in both lungs. X-ray revealed diffuse bilateral airspace consolidation. Arterial blood gases on air showed PaO2 41 mm Hg, PaCO2 35 mm Hg, and pH 7.46. There was a restrictive pattern on pulmonary function testing. Fiberoptic bronchoscopy with broncho-alveolar lavage and transbronchial biopsy established the diagnosis of pulmonary alveolar proteinosis. Whole lung lavage lead to gradual improvement over the next few weeks. However, chest X-ray, arterial blood gases and pulmonary function tests failed to revert completely to normal. Such severe manifestations and such an aggressive course as in this case might have been prevented had the diagnosis been made and treatment instituted earlier in the course of the disease.