Abstract

Background

Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by deposition of abnormal PAS positive lipoproteinaceous material in the alveolar spaces due to defective surfactant clearance by alveolar macrophages.  It is associated with alterations in the immune state and secondary chest infections. The radiological pattern of ‘crazy paving’ is thought to be quite characteristic of the disorder. Whole lung lavage (WLL) is the treatment of choice with increasing support for inhaled GMCSF.

Aims/Methods

To describe three cases of PAP with concomitant lung cysts and their potential implications in management.

Results

Three patients, 19, 22 and 26 year old, with autoimmune PAP and acute presentation with severe respiratory failure and typical ‘crazy paving’ demonstrated single lung cysts on imaging. Two of them grew Mycobacterium intracellulare but they were not considered to have active NTM infection.

The first patient, with a left upper lobe cyst, underwent right whole lung lavage (WLL) and fiberoptic segmental lavage of the lingula and left lower lobe with excellent response. The second patient, with a right upper lobe cyst, had uneventful bilateral WLL with subsequent reduction of the size of the cyst. The third patient presented with a right lower lobe cyst. WLL was delayed and inhaled GMCSF was initiated without success. Bilateral WLL was performed after resolution of the cyst following treatment with broad spectrum antibiotics.

Conclusion

Lung cysts in PAP are a rare phenomenon and can be associated with secondary infections or potentially fragility and destruction of the alveolar walls in more severe PAP cases. Their presence can have an impact on the patients’ management and choice of treatment.