Systemic sclerosis (SSc)

Systemic sclerosis or scleroderma is a complex connective tissue disease. It is characterized by chronic and recurrent inflammation, vasculopathy and excessive fibrosis. These processes manifest as a diverse range of tissue and organ-specific complications. One of the most common complications of the lung is pulmonary arterial hypertension, which develops in 8-13% of SSc patients and is the leading cause of death in patients with this disease.

Pulmonary arterial hypertension (PAH)

Pulmonary arterial hypertension is a progressive disease caused by constriction and narrowing of the pulmonary arteries. Due to an increasing pulmonary vascular resistance, blood flow is compromised and the right side of the heart becomes enlarged (hypertrophy and eventually dilatation), due to the increased strain involved in pumping blood. This strain on the heart, as well as the decrease in blood flow to the left heart and thus the systemic circulation (i.e. reduced cardiac output), lead to the common symptoms of PAH such as breathlessness and fatigue. Other typical PAH symptoms include dizziness, syncope, peripheral edema and chest pain. The non-specific nature of PAH symptoms represent a considerable challenge for the timely diagnosis of PAH. This frequently results in a considerable delay between the onset of symptoms and PAH diagnosis. As SSc patients are at greater risk of developing PAH than the general population, the possibility of screening these patients for PAH provides an opportunity to improve diagnosis of PAH in SSc patients. Extensive information about PAH can be found at