PULMONARY ALVEOLAR PROTEINOSIS: REPORT OF THREE CASES

Abstract

Three new cases of pulmonary alveolar proteinosis are reported. Cough and dyspnea were the principal complaints of these patients. In 2 of the 3 cases, the onset of the disease was marked by the signs and symptoms of an acute respiratory illness. Roentgeno-grams suggestive of atypical sarcoidosis without hilar lymph node involvement, or of atypical pulmonary edema were obtained in the early stages of the disease. In 2 of the patients, the degree of disability was not great, although the roentgenograms demonstrated extensive bilateral pulmonary involvement. This (paradox or) disproportion between the clinical signs and symptoms and the X-ray findings may be a clue to the clinical diagnosis of this disease. Laboratory studies were non-contributory. The diagnosis was established in each case after lung biopsy. Histopathologically, the findings were similar in all cases. The characteristic lesions showed the deposition of granular and amorphour eosinophilic material in the air spaces. This material is periodic acid-Schiff positive. These and other histologica findings are so striking and uniform that they lend weight to the hypothesis that this is a new disease rather than an old one that has previously escaped detection. The etiology of pulmonary alveolar proteinosis remains obscure.