ICD-10 Code J84.842 – Pulmonary interstitial glycogenosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code J84.842 – Pulmonary interstitial glycogenosis
What it is
Pulmonary interstitial glycogenosis is a rare interstitial lung disease, usually seen in infants, where the lung interstitium contains excess glycogen-filled cells. It can interfere with normal breathing and lung development.
Clinical signs
Clinical features vary; refer to documentation. Typical findings include early-life respiratory distress, tachypnea, hypoxemia, and diffuse interstitial changes on imaging, with diagnosis confirmed by lung biopsy.
When to use this code
Use this code when the provider documents pulmonary interstitial glycogenosis or confirms it as the diagnosis after pathology review. It may also apply when the record clearly identifies this specific interstitial lung disorder in an infant or child.
Do not code it for nonspecific neonatal respiratory distress, interstitial lung disease without a named diagnosis, or other causes of infant hypoxemia. Check documentation if the pathology report is incomplete or the diagnosis is uncertain.
Do not use for
Do not use this code for general interstitial lung disease, pulmonary fibrosis, or respiratory distress without documented pulmonary interstitial glycogenosis. Check documentation when another primary lung disorder is stated.
Coding tip
Code J84.842 only when the diagnosis is explicitly documented; a biopsy-supported note is often the clearest source.