Pathogenetic features of pulmonary hypertension in systemic sclerosis

Systemic connective tissue diseases may be associated with various forms of pulmonary hypertension (PH). The prevalence of PH varies among different systemic connective tissue diseases, with systemic scleroderma (SSc) having the highest rate, ranging from 8 to 12 %. With SSc, PH is one of the severe and life-threatening manifestations, which necessitates the study of this problem. The pathogenesis of PH in SSc varies and depends on the predominant organ damage. PH can be represented by pulmonary arterial hypertension (PAH) (group 1) with isolated damage to the pulmonary vessels, or with more rare conditions such as veno-occlusive disease, portopulmonary hypertension, or drug toxicity. Venous PH can also develop due to damage to the left chambers of the heart, such as myocardial fibrosis, diastolic dysfunction of the left ventricle, or valve pathology (group 2). PH associated with lung damage may develop due to interstitial lung Systemic connective tissue diseases may be associated with various forms of pulmonary hypertension (PH). The prevalence of PH varies among different systemic connective tissue diseases, with systemic scleroderma (SSc) having the highest rate, ranging from 8 to 12 %. With SSc, PH is one of the severe and life-threatening manifestations, which necessitates the study of this problem. The pathogenesis of PH in SSc varies and depends on the predominant organ damage. PH can be represented by pulmonary arterial hypertension (PAH) (group 1) with isolated damage to the pulmonary vessels, or with more rare conditions such as veno-occlusive disease, portopulmonary hypertension, or drug toxicity. Venous PH can also develop due to damage to the left chambers of the heart, such as myocardial fibrosis, diastolic dysfunction of the left ventricle, or valve pathology (group 2). PH associated with lung damage may develop due to interstitial lung diseases, such as nonspecific and usual interstitial pneumonia (group 3). Patients with SSc are more susceptible to thrombosis and pulmonary embolism due to formation of antiphospholipid antibodies, which can lead to the development of chronic thromboembolic PH (group 4). A characteristic feature of SSc is that one patient may have several reasons for PH development. The variety of pathogenetic variants of PH in SSc requires personalized and comprehensive diagnostic approaches to each patient. Management of the patient and determination of indications for appointment of PAH-specific therapy depend on the variant and pathogenesis of PH.