Diffuse progressive pulmonary interstitial and intra-alveolar cholesterol granulomas in childhood

We describe an 18 year old male with pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG), which developed to severe respiratory failure over 15 yrs. The histological diagnosis was made on the basis of open lung biopsy findings at the age of 3 yrs and autopsy at 18 yrs of age. Although the pathological features of the lung were similar to that of paediatric patients with lipoid pneumonia coexisting with pulmonary alveolar proteinosis (PAP), gastro-oesophageal reflux (GOR) and a diverse group of severe primary diseases, the patient lacked evidence for any of these. We believe the present case provides a new example of a diffuse-type of lipoid pneumonia coexisting with pulmonary alveolar proteinosis, which we call cholesterol granulomas.