Obstructive pulmonary disease is not a feature. Hypoxemia may be present at rest or, if disease is mild, only with mild to moderate exercise. Prognosis and Treatment Disability from respiratory insufficiency is common, but death rarely occurs if those who need treatment with bronchopulmonary lavage receive it. Secondary infections should be promptly identified and treated. Patients with minimal or no symptoms do not require treatment but should be observed for exacerbations that may lead to respiratory failure.
Treatment is indicated only for patients with significant symptoms and hypoxemia.
With the patient under general anesthesia, the lungs are usually lavaged one at a time with 3 to 5 days between lavages. Systemic corticosteroids have been unsuccessful and may increase the possibility of secondary infection. Any therapeutic regimen is difficult to evaluate because spontaneous remissions occur and because the number of cases available for study by one investigator is limited. All rights reserved.