pulmonary interstitial glycogenosis

Definition: Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory distress. The "pulmonary interstitial glycogenosis" of the neonate is a new entity to be differentiated from other forms of interstitial lung disease.

Despite its dramatic clinical presentation the diagnosis of PIG has a favorable prognosis with rare mortality in the absence of co-morbid conditions.

Because abundant glycogen is not normally found in pulmonary interstitial cells, we postulate an abnormality in lung cytodifferentiation involving interstitial mesenchymal cells.

Images

 Infant with respiratory distress - pulmonary interstitial glycogenosis, reactive to underlying injury.

• https://twitter.com/amy_treece13/status/873717842188914689

Clinical synopsis

 first month of life
 neonatal onset
 respiratory failure
 pulmonary hypertension
 tachypnea
 hypoxemia
 diffuse interstitial infiltrates
 overinflated lungs

Pathology synopsis

 expansion of the interstitium by spindle-shaped cells containing periodic acid-Schiff positive diastase labile material consistent with glycogen.

Immunohistochemical staining

 spindle-shaped cells

• vimentin positive
• negative for leucocyte common antigen, lysozyme, and other macrophage markers.

Electron microscopy

 primitive interstitial mesenchymal cells with few cytoplasmic organelles and abundant monoparticulate glycogen.
 Minimal or no glycogen seen in the alveolar lining cells.
 diffuse expansion of the alveolar interstitium by glycogenated mesenchymal cells.

Prognosis

 significant resolution of the disorder (19320535)

References

 Pulmonary interstitial glycogenosis. Lanfranchi M, Allbery SM, Wheelock L, Perry D. Pediatr Radiol. 2010 Mar;40(3):361-5. PMID: 19902199

 HISTOLOGIC RESOLUTION OF PULMONARY INTERSTITIAL GLYCOGENOSIS. Deutsch GH, Young LR. Pediatr Dev Pathol. 2009 Mar 25:1. PMID: 19320535

 Diffuse lung disease in infancy: a proposed classification applied to 259 diagnostic biopsies. Langston C, Dishop MK. Pediatr Dev Pathol. 2009 Nov-Dec;12(6):421-37. PMID: 19323600

 Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. Canakis AM, Cutz E, Manson D, O’Brodovich H. Am J Respir Crit Care Med. 2002 Jun 1;165(11):1557-65. PMID: 12045133 (Free)