Although patients with scleroderma are known to be at risk of pulmonary arterial hypertension and interstitial lung disease, diagnosis of these lung complications is often delayed.
All patients with scleroderma are at risk of pulmonary arterial hypertension and interstitial lung disease.
Scleroderma (or systemic sclerosis) is characterised by vascular dysfunction, manifesting as Raynaud’s phenomenon, inflammation and eventually fibrosis, typically in the form of progressive skin thickening. Although the resultant digital ulceration and joint contractures cause significant morbidity, involvement of other organs such as the kidneys, gut and particularly lungs can have devastating effects on quality of life and life expectancy of affected patients. The most common lung manifestations, pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), which affect up to 15% and 80% of patients with scleroderma, respectively, are the leading cause of death in these patients.