Coexisting Endogenous and Exogenous Llpold Pneumonia and Pulmonary Alveolar Proteinosis in a Patient with Neurodevelopmental Disease

Abstract

We report a unique case of coexistingexogenous lipoid pneumonia, endogenous lipoid pneumonia (EU), and pulmonary alveolar proteinosis (PAP) in a 5-year-old patient with severe neurodevelop mental disease. The patient presented with gastroesophageal reflux and presumed chronic lung disease resulting from recurrent aspiration pneumonias and succumbed to respiratory failure. The autopsy showed lipid-laden macrophages and periodic acid-SchiJf9ositive granular matm'al i n alveolar spaces and multilamellated structures within both alveolar macrophages and extracellular debris.

These findings were similar to those in previous reports of coexisting E I P and PAP in the setting of gastroesophageal refux [I]. However, the present case diJfered by the presence of scattered large osmiophilic extracellular lipid vacuob. Besides strengthening the association between EIP and PAP and their relationship to gastroesophageal refux, this case suggests that t h q may arise together with exogenous lipoid pneumonia, through related mechanisms, in the setting of neurodevelopmental disease.

Key Words:

• exogenous lipoid pneumonia
• endogenous lipoid pneumonia
• pulmonary alveolar proteinosis