Idiopathic pulmonary fibrosis

This code identifies a chronic, progressive lung disease characterized by the irreversible scarring and thickening of lung tissue without an identifiable cause. The fibrosis leads to impaired gas exchange, resulting in progressive shortness of breath and a restrictive lung pattern. It is a specific form of interstitial lung disease.

Assign this code when documentation clearly states a diagnosis of idiopathic pulmonary fibrosis (IPF). This diagnosis is typically made by a pulmonologist based on clinical presentation, high-resolution computed tomography (HRCT) findings, and often lung biopsy, after excluding other known causes of interstitial lung disease.

• Ensure the "idiopathic" nature of the fibrosis is explicitly documented to differentiate from secondary pulmonary fibrosis.
• Do not use this code for other forms of interstitial lung disease or pulmonary fibrosis with a known etiology.
• Consider co-morbidities such as pulmonary hypertension (I27.2x) or respiratory failure (J96.xx) and code them appropriately.