Abstract
OBJECTIVES: Exercise-induced pulmonary hypertension (exercise PH) is instrumental in the early detection of pulmonary arterial hypertension. However, the prevalence and specific characteristics of exercise PH in systemic sclerosis (SSc), as well as factors that might facilitate its identification, remain inadequately understood. We investigated the clinical profiles of SSc patients diagnosed as PH, exercise PH, or non-PH. METHODS: This study included 50 patients with SSc and exercise intolerance. We compared the physical examination findings, biochemistry tests, echocardiography, respiratory function tests, and resting and exercise right heart catheterization outcomes of the PH, exercise PH, or non-PH groups. RESULTS: In a cohort of 50 patients with SSc and exercise intolerance, 30.0% were diagnosed with PH, and 44.0% exhibited exercise PH. Symptoms such as exertional dyspnea and palpitations were more prevalent in the exercise PH group compared to the non-PH group. Furthermore, a lower nailfold capillary density was observed in the exericse PH group. However, there was no difference between the two groups in terms of NT-proBNP level, serum uric acid, inflammatory findings, %DLco%, FVC/%DLco ratio, six-minute walking distance, or peak tricuspid regurgitant velocity. exercise PH was detected in 87.5% of patients presented all with exertional dyspnoea, exertional palpitations, and capillary density ≤ 7/mm. In many patients within the exercise PH group, the pulmonary artery wedge pressure/cardiac output slope exceeded 2 mmHg/L/min, indicating latent heart failure with preserved ejection fraction (HFpEF). CONCLUSION: In our study, 44.0% of patients with SSc and exercise intolerance exhibited exercise PH, with many potentially having HFpEF. Decreased nailfold capillary density, exertional dyspnea, and palpitations were indicative markers useful for exercise PH screening.