Pulmonary interstitial glycogenosis
ICD-10 J84.842 is a billable code used to indicate a diagnosis of pulmonary interstitial glycogenosis.
Pulmonary interstitial glycogenosis (PIG) is a rare lung disease characterized by the accumulation of glycogen within the interstitial spaces of the lungs. Clinically, it presents with respiratory distress, cough, and hypoxemia, often in infants or young children, although it can also occur in adults. The disease primarily affects the alveolar interstitium, leading to impaired gas exchange and progressive respiratory failure. The pathophysiology involves abnormal glycogen metabolism, which may be associated with genetic disorders or environmental factors. Diagnosis is typically made through imaging studies such as high-resolution CT scans, which reveal ground-glass opacities and reticular patterns, alongside lung biopsy for histological confirmation. Disease progression can vary, with some patients experiencing chronic symptoms while others may have acute exacerbations. Early recognition and management are crucial to improve outcomes, as the condition can lead to significant morbidity if left untreated.
Standard ICD-10-CM documentation requirements apply
Various clinical presentations within this specialty area
Follow specialty-specific billing guidelines
Standard ICD-10-CM documentation requirements apply
Various clinical presentations within this specialty area
Follow specialty-specific billing guidelines
J84.842 specifically covers pulmonary interstitial glycogenosis, which is characterized by the accumulation of glycogen in the lung interstitium, leading to respiratory symptoms. It is important to differentiate it from other interstitial lung diseases, such as pulmonary fibrosis or sarcoidosis.
J84.842 should be used when the clinical presentation and diagnostic findings specifically indicate pulmonary interstitial glycogenosis. It is crucial to ensure that the diagnosis is confirmed through appropriate imaging and histological evaluation to avoid misclassification.
Documentation for J84.842 should include a comprehensive clinical history, results from imaging studies (such as CT scans), and histopathological findings from lung biopsies. Additionally, any underlying metabolic disorders should be documented to support the diagnosis.
