Adult Onset Still Disease Associated with Endogenous Lipoid Pneumonia

Abstract

Cholesterol pneumonia or endogenous lipid pneumonia (ELP) is a rare disease that can occur in the context of a systemic disease or following a bronchial obstruction. It is characterized by a wide range of diverse symptoms and various disease course. The present report introduces a young woman diagnosed with adult onset still disease three years ago, who has been referred with macrophage activation syndrome (MAS). She underwent biopsy due to dyspnea and a crazy paving pattern in HRCT of the lungs, leading to the diagnosis of lipoid pneumonia based on the interstitial lymphocytic inflammation and cholesterol granulomas. So far, there has been no report indicating MAS associated with cholesterol pneumonia. This is the second case reporting ELP in the adult onset still disease.

Exogenous Lipoid Pneumonia Induced by Nasal Instillation of Paraffin Oil

“…Basing on the source of the lipids exposure, lipoid pneumonia has been classified into endogenous and exogenous [1]. The pathogenesis of the endogenous form is complex, the source of lipids is the lung tissue itself, the disease may occur as a result of infection (mainly in the obstructed bronchus), repetitive episodes of fungal infections or due to the non-infectious process such as pulmonary alveolar proteinosis and lipid-storage disorders (Niemann-Pick disease) [1,3,6,7]. The exogenous type of lipoid pneumonia occurs as a result of exposure to various fatty substances (mostly mineral oils), which may be inhaled or ingested.…”