FATAL CASE OF PULMONARY ALVEOLAR PROTEINOSIS BY R. L. RAY AND R. SALM From Tehidy Chest Hospital, Camborne, and Camborne-Redruth Hospital, Redruth, Cornwall

Rosen, Castleman, and Liebow (1958) defined a " new " syndrome which they called pulmonary alveolar proteinosis, though it is surprising that the malady should have escaped the notice of competent workers of the past. They based their observations on a series of 27 cases (25 of which had been referred to them for opinion), 24 from the United States, and one each from Canada, Great Britain, and Italy. According to these authors the disease occurred mainly in adults between the ages of 20 and 50 years; their youngest patient was aged just over 2 years, their oldest 57 years. Males predominated in a proportion of 2.5: 1. The illness frequently began suddenly with a pneumonia-like attack, which occasionally recurred, though sometimes the onset was gradual and insidious. After the initial attack the patient usually remained apyrexial. The main symptom was a progressive and ultimately crippling dyspnoea, though anorexia, lassitude, chest pain, cyanosis, and loss of weight were commonly present. Cough was noticed frequently, often unproductive, but sometimes-with a little white or yellowish sputum described as " chunky." Clubbing of the fingers was occasionally seen. Pulmonary function tests, though not normal, showed only moderate restrictive changes for most of the course, as well as evidence of an alveolar-capillary block. Polycythaemia with haemoglobin values above 100% was almost always found, occasionally accompanied by a polymorphic leucocytosis. The plasma lipids and proteins were within normal limits, electrophoresis results did not show any definite pattern, and serum electrolytes were normal. Chest radiographs showed fine diffuse feathery or vaguely nodular opacities of moderate radio-opacity, which tended to spread outwards from the hila not unlike pulmonary oedema and, when resolution occurred, regressed in the opposite direction. Pulmonary calcification and hilar glandular enlargement were never seen. All investigations for the presence of bacterial, fungal, viral, and protozoal infections and of chemical irritants, including radioactive material, were negative. The illness extended over months or years, and, of the original series of 27, one-third improved, one-third remained stationary, and one-third had died within five years as the result of slowly progressive pulmonary insufficiency. Superadded infections such as nocardiosis and cryptococcosis were infrequent complications. Various forms of therapy were tried, but all proved ineffective. At necropsy the lungs were the only diseased organs. They were bulky and heavy, weighing together between 2,950 and 3,500 g., due to numerous yellowish-grey, confluent nodular consolidations. The intervening lung tissue tended to be …