ABSTRACT
Lung involvement is primarily due to interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). The survival of systemic sclerosis patients with SSc-ILD has been reported to be between 29 and 69% at 10 years. Risk factors for a worse outcome following lung transplantation including gastroesophageal reflux disease, malabsorption and low body mass index. Median survival following lung transplantation is approximately 5 years, which is similar to that seen in patients undergoing transplantation for PAH. Disease progression despite these measures should trigger consideration of suitability for lung transplantation and consideration of involvement of palliative care services. Pulmonary hypertension in SSc is most commonly due to a vasculopathy but may also occur in the context of lung disease such as ILD or as a consequence of LV diastolic or systolic dysfunction. Imaging may be helpful when assessing the likelihood of pulmonary hypertension and is required to assess for coexisting lung disease, left-heart disease, and thromboembolic pulmonary hypertension.
