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.
Inflammatory cells are usually absent, but if present usually indicate infection.
Ca 19-9 levels showed an inverse correlation to PaO 2. The 'gold standard' for the diagnosis of alveolar proteinosis is lung biopsy, showing the characteristic histological changes of alveolar proteinosis. The most effective means of treatment therefore remains whole lung bronchoalveolar lavage. Up to 44 litres of fluid may be required. Meticulous monitoring of fluid volumes, oxygen saturation, blood gases and haemodynamics are required.