CLINICAL AND DIAGNOSTIC FEATURES OF PULMONARY HYPERTENSION IN PATIENTS WITH SYSTEMIC SCLEROSIS

Pulmonary hypertension is one of the most severe complications of systemic sclerosis and is associated with progressive pulmonary vascular remodeling, right ventricular dysfunction, and increased mortality. This study evaluated the clinical and diagnostic characteristics of pulmonary hypertension in patients with systemic sclerosis through a systematic analysis of publications indexed in PubMed, Scopus, Web of Science, and Google Scholar between 2000 and 2025. A total of 218 scientific studies were analyzed. The reviewed data demonstrated that systemic sclerosis-associated pulmonary arterial hypertension represented the predominant hemodynamic phenotype and was characterized by elevated pulmonary vascular resistance, reduced DLCO, impaired exercise tolerance, and poor long-term survival. The most common clinical manifestations included exertional dyspnea, fatigue, peripheral edema, and progressive cardiopulmonary dysfunction. Doppler echocardiography, pulmonary function testing, NT-proBNP assessment, and high-resolution computed tomography showed high diagnostic value for early detection, whereas right heart catheterization remained the gold standard for definitive diagnosis and hemodynamic classification. Early multimodal screening significantly improved survival outcomes and reduced disease progression in systemic sclerosis-associated pulmonary hypertension patients.

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