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Published 1 September 2009
Cite this as: BMJ Case Reports 2009 [doi:10.1136/bcr.04.2009.1796]
Copyright © 2009 by the BMJ Publishing Group Ltd.

Rare disease

A case report of pulmonary alveolar proteinosis

Helen McDermott1, Frances Aitchison2, Nazim Nathani2, Anjali Bhatnager2

1 The University of Birmingham Medical School, Edgbaston, Birmingham, West Midlands. B15 2TT
2 City Hospital, Dudley Road, Birmingham, B18 7QH, UK

Correspondence to:
Helen Mcdermott, Hem507{at}bham.ac.uk

SUMMARY

This is a case of pulmonary alveolar proteinosis that presented in an inner city hospital in Birmingham, UK. The patient was a previously well 42-year-old man, who went on to experience unusual cerebral complications of the disease. The presentation, imaging findings and diagnostic histology findings are described. Pulmonary alveolar proteinosis is a rare but important diagnosis. Characteristic high-resolution CT findings include diffuse ground glass density with superimposed interlobular septal thickening, which is described as the "crazy paving" pattern. Diagnosis is made by bronchoalveolar lavage. Pathologically the disease is characterised by alveolar filling with a lipid rich, proteinaceous material (positive to periodic acid–Schiff stain) while the lung interstitium remains relatively normal. Morbidity and mortality can be improved by treatment with whole lung lavage.


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