It was later established that this same diagnosis had been made in 1989 at an admission at Baragwanath Hospital in Soweto. The patient refused bronchopulmonary lavage and was discharged to be followed up at the Respiratory Clinic. The pathogenesis of alveolar proteinosis is at present incompletely understood. One of the abnormalities described is abnormal in situ degradation of surfactant in patients with alveolar proteinosis, possibly explaining some of the subtle differences between fluid found from patients with alveolar proteinosis and normal surfactant. This was corrected by replacing the normal GM-CSF gene in the respiratory epithelium in the offspring.
Controversy exists about the role of macrophages in the pathogenesis of alveolar proteinosis.
This appeared to be related to an unrecognised component of fluid obtained by BAL from patients with alveolar proteinosis. Primary alveolar proteinosis is characterised by a diffuse disease process with a uniform staining for surfactant-specific apoprotein. Opportunistic infections are sometimes described preceding the development of secondary alveolar proteinosis, but are believed to be a reflection of the underlying disease process rather than a cause of alveolar proteinosis. Diseases associated with secondary alveolar proteinosis HIV infection 2 Haematological malignancies 2 Hypogammaglobulinaemia 2 Dust exposure 2 including silica, aluminium and titanium. In children, a nodular and miliary pattern may occur.