J84.842
BillablePulmonary interstitial glycogenosis
HCC Category Mapping
V24HCC 112 — Fibrosis of Lung and Other Chronic Lung Disorders
0.268ESRDHCC 112 — Fibrosis of Lung and Other Chronic Lung Disorders
0.000RxHCCHCC 227 — Sarcoidosis, Pulmonary Alveolar Disorders
0.000What This Code Means
A rare lung disease in children where glycogen accumulates abnormally in the lung tissue, affecting the ability to breathe and exchange oxygen.
Coding Tips
- •This is a rare pediatric condition; verify patient age and confirm diagnosis with pulmonary specialist documentation
- •Often requires genetic testing confirmation; ensure supporting documentation is present in the medical record
Clinical Significance
Pulmonary interstitial glycogenosis is an extremely rare pediatric lung disease where mesenchymal cells laden with glycogen infiltrate the pulmonary interstitium, typically presenting in neonates and young infants with respiratory distress. This condition can occur in isolation or alongside other developmental lung abnormalities. Most cases improve with supportive care, but it requires accurate diagnosis to avoid inappropriate treatment.
Documentation Requirements
- ✓Lung biopsy confirming glycogen-laden mesenchymal cells in the pulmonary interstitium
- ✓Documentation of patient age (neonate or young infant)
- ✓Clinical presentation including respiratory distress, oxygen requirements
- ✓Imaging findings on chest CT or radiograph
- ✓Documentation of any associated lung developmental abnormalities (pulmonary hypoplasia, congenital heart disease)
Commonly Confused Codes
J84.841 — Neuroendocrine cell hyperplasia of infancy is a different pediatric ILD presenting similarly but with different pathologyJ84.843 — Alveolar capillary dysplasia is fatal in infancy unlike PIG which generally improvesJ84.848 — Other interstitial lung diseases of childhood should not be used when PIG is pathologically confirmedE74.09 — Other glycogen storage diseases involve different organs and pathophysiology