Pulmonary Interstitial Glycogenosis

Pulmonary interstitial glycogenosis (PIG) is a rare non-lethal pediatric form of interstitial lung disease (ILD, see this term).

Epidemiology

Very few cases have been described to date but there appears to be male predominance.

Clinical description

Neonates present with respiratory distress syndrome shortly after birth. The characteristic histological feature of PIG is the accumulation of monoparticulate glycogen in interstitial cells on lung biopsy.

Etiology

The disease is thought to be the result of a maturation defect of interstitial cells leading to glycogen accumulation within the cytoplasm.

Prognosis

Prognosis is dependent on the type of lung growth abnormalities associated with the infiltrative disease.