Systemic sclerosis with lung involvement

This code signifies systemic sclerosis (scleroderma) that has manifested with pulmonary complications. It indicates the presence of fibrotic changes or other disease-related pathology within the lungs as a direct result of the underlying autoimmune connective tissue disorder.

Apply this code when documentation clearly states a diagnosis of systemic sclerosis and specifies lung involvement, such as interstitial lung disease (ILD), pulmonary hypertension secondary to scleroderma, or other scleroderma-related lung pathology. Supporting documentation would include physician notes, imaging reports (e.g., HRCT chest), and pulmonary function tests confirming lung compromise due to systemic sclerosis.

• Always sequence the systemic sclerosis code first, as it is the underlying condition.
• Do not use this code for localized scleroderma (morphea) without systemic manifestations.
• Ensure documentation specifies lung involvement and not just a general diagnosis of systemic sclerosis.