Pulmonary Alveolar Proteinosis Follow-up
- Author: Roger B Olade, MD, MPH; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more...
Further Outpatient Care
Patients usually improve dramatically with whole-lung lavage, but relapses may occur. Repeated lavage usually is necessary. Patients should have regular follow-up with a pulmonologist.
Further Inpatient Care
Inpatient care is uncommon in primary pulmonary alveolar proteinosis (PAP), except for concomitant superinfection or severe hypoxemia. Treatment of secondary PAP might require inpatient care and outpatient follow-up.
Patients prone to alveolar proteinosis related to inhalation of inorganic dusts or insecticides should avoid further exposure.
There has been some association between anti-GM-CSF autoantibodies and some cases of cryptococcal meningitis in otherwise immunocompetent patients.
The overall prognosis for primary PAP is very good, with achievement of complete remissions in many patients. Whole-lung lavage most often results in a dramatic response. Some patients require repeated lavages, and these patients usually progress to pulmonary fibrosis and have a poor outcome. Congenital PAP responds favorably to lung transplantation.
Mortality rates of as high as 30% within several years of disease onset have been reported previously, but the actual mortality rate may be less than 10%. Solitary pulmonary PAP is increasingly being seen and may resolve spontaneously over several months.
The natural history of secondary PAP depends on the underlying etiologic entity.
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