[Pulmonary alveolar proteinosis: eleven-year follow-up in one case (author's transl)]

A case of pulmonary alveolar proteinosis followed-up for eleven years is presented and this poorly recognised affection discussed. It is a rare disease arising from filling of the alveoli by a non-surface-acting surfactant, either as a response to a non-specific aggression, experimentally reproducible, or spontaneously. The mechanism by which this accumulation occurs is still a controversial subject: increased production by the granular pneumocytes, deficiency of alveolar clearance, or loss of phagocytic activity of alveolar macrophages. The diagnosis, suggested by the only slightly specific clinical picture after elimination of more common aetiologies, is confirmed by electron microscopy of alveolar lavage fluid without, as in the past, the need for lung biopsy. The various treatments proposed have lacked efficacy and are not really necessary, except for symptomatic extensive pulmonary lavage, which should be reserved for severe cases and may need to be repeated.