Lung involvement in systemic sclerosis

This code signifies the presence of pulmonary manifestations directly attributable to systemic sclerosis, also known as scleroderma. It encompasses various lung pathologies, such as interstitial lung disease (ILD), pulmonary hypertension, or pleural disease, occurring as a complication of the underlying autoimmune connective tissue disorder.

Use this code when documentation clearly indicates lung involvement as a direct consequence of systemic sclerosis. This typically applies to patients diagnosed with scleroderma who present with respiratory symptoms, abnormal imaging (e.g., HRCT showing fibrosis), or pulmonary function test abnormalities linked to their systemic disease.

• Always code the underlying systemic sclerosis first, followed by this code to specify the lung involvement.
• Distinguish from other causes of interstitial lung disease; documentation must explicitly link the lung condition to systemic sclerosis.
• Do not use for incidental lung findings not directly related to the systemic sclerosis.