Management overview for pulmonary arterial hypertension in systemic sclerosis

Note: Pulmonary arterial hypertension occurs in 10 to 15% of people with systemic sclerosis, regardless of whether they have diffuse or limited disease.

For detailed information about pulmonary arterial hypertension (PAH) of all causes, see Pulmonary hypertension in the Cardiovascular guidelines. Pulmonary arterial hypertension occurs in 10 to 15% of people with systemic sclerosis, regardless of whether they have diffuse or limited disease. It can develop insidiously in the absence of clinically significant interstitial lung disease, but it may also occur secondary to interstitial lung disease with associated destruction of the pulmonary vascular bed.

Initial symptoms of pulmonary arterial hypertension can be subtle and easily misinterpreted as other symptoms of systemic sclerosis (eg mild breathlessness on exertion, dizziness, palpitations, generalised weakness). Pulmonary arterial hypertension and interstitial lung disease may be quite advanced by the time dyspnoea develops; annual echocardiography and pulmonary function tests are recommended for early identification of pulmonary arterial hypertension and interstitial lung disease.

Note: Annual echocardiography and pulmonary function tests are recommended for early identification of pulmonary arterial hypertension and interstitial lung disease associated with systemic sclerosis.