Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of a large amount of both components of surfactant, proteins and phospholipids in alveoli and terminal bronchioles. This cumulation may lead to impaired gas exchange and result in respiratory insufficiency. The disease may develop in neonatal age or later in adult age. Two congenital forms are known. The disease occurs however also in people with neoplastic disease, during cytostatic treatment, du- ring inhalation of inorganic dusts. A dominant symptom is progressing dyspnoea. Asymptomatic forms exist also. The most effective therapeutic method is overall pulmonary lavage. The authors implemented in 1999 the first overall pulmonary lavage in the Czech Republic in the General Faculty Hospital in Prague in a 60-year-old female patient.

        Key words: Pulmonary alveolar proteinosis - Overall pulmonary lavage