Interstit lung dis w progr fibrotic phenotype dis classd e

This diagnosis code signifies a specific type of interstitial lung disease (ILD) that exhibits a progressive fibrotic phenotype, meaning the lung scarring is worsening over time. It is used when this fibrotic progression occurs as a complication or manifestation of another underlying disease process, rather than being an idiopathic ILD. This indicates a severe and often irreversible form of lung damage.

Use this code when documentation clearly states a diagnosis of interstitial lung disease with a progressive fibrotic phenotype, and explicitly links it to an underlying systemic or autoimmune disease (e.g., rheumatoid arthritis, systemic sclerosis, inflammatory myositis). The physician's notes should confirm the progressive nature of the fibrosis, often supported by imaging (e.g., HRCT) and pulmonary function tests showing decline.

• Always sequence the underlying systemic disease first, as this code indicates a manifestation in diseases classified elsewhere.
• Ensure documentation specifies "progressive fibrotic phenotype" and not just "fibrosis" or "interstitial lung disease."
• Do not use for idiopathic pulmonary fibrosis (IPF) or other ILDs without an identified underlying cause; those have their own specific codes.