Pulmonary interstitial glycogenosis

This code identifies a rare, diffuse lung disease primarily affecting infants, characterized by the abnormal accumulation of glycogen within the interstitial spaces of the lung parenchyma. It presents as persistent tachypnea and hypoxemia, often requiring respiratory support, and is a distinct entity from other interstitial lung diseases of infancy.

Apply this code when documentation explicitly states a diagnosis of pulmonary interstitial glycogenosis (PIG). This diagnosis is typically confirmed by lung biopsy showing characteristic glycogen deposits in interstitial fibroblasts. It is often considered in infants with unexplained chronic respiratory distress and diffuse interstitial changes on imaging.

• Ensure the documentation clearly differentiates PIG from other pediatric interstitial lung diseases, such as neuroendocrine cell hyperplasia of infancy (NEHI) or diffuse developmental disorders.
• Do not confuse this with glycogen storage diseases affecting other organs; this code is specific to pulmonary involvement.
• Code any associated respiratory failure or other complications separately.