Pulmonary interstitial glycogenosis

Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease associated with alveolar growth abnormalities. 

Patients may present in the neonatal period with disproportionate respiratory distress (neonatal respiratory distress).

It is histologically characterized by abundant glycogen-laden mesenchymal cells within the pulmonary interstitium 1.

Many cases many show concurrent extrapulmonary comorbidities with congenital cardiac disease considered the most common of these 3.

Features are nonspecific, including ground-glass opacification (considered most common 1), consolidation, linear opacities, and mosaic attenuation without a uniform pattern.


Article information

rID: 69106
Synonyms or Alternate Spellings:
  • Pulmonary interstitial glycogenosis (PIG)

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