Coexisting Endogenous Lipoid Pneumonia, Cholesterol Granulomas, And Pulmonary Alveolar Proteinosis in a pediatric Population: A Clinical, Radiographic, and Pathologic Correlation

Abstract

Benign pulmonary diseases that have been associated with the accumulation of endogenous lipids within the alveoli, bronchioles, and interstitial tissues include endogenous lipoid pneumonia (ELP), pulmonary alveolar proteinosis (PAP), pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG), various xanthomatous lesions, and nulakoplakia. In ELP, fat-filled finely vacuolated macrophages fill the alveoli. In PAP, the aveoli become filled with cholesterol and periodic acid-Schiff (PAS)-positive acellular debris. In PICG, cholesterol esters are released from degenerating macrophages and, as organization occurs, the cholesterol is deposited in the form of acicular clefts within the interstitium. These morphologically distinct presentations of endogenous lipid deposition within the lung have long been thought to represent unique disease processes but several author now postulate a possible relationship between these entities. We report here on the clinical, radiographic, and morphologic findings in eight pediatric patients with diverse primary disease processes who were subsequently found to have varying and often coexisting degrees of EL8 PA8 and PICG.

Key Words:

• endogenous lipoid pneumonia
• gastroesophageal reflux with aspiration
• pulmonary alveolar proteinosis
• pulmonary cholesterol granulomas