pulmonary hypertension scleroderma

Pulmonary arterial hypertension (PAH) and systemic sclerosis are two interconnected diseases. Systemic sclerosis, also referred to as scleroderma, is a rare autoimmune disorder marked by the fibrosis of skin and visceral organs. PAH indicates a pathological increase in blood pressure within the pulmonary arteries, which can lead to an increased workload on the right ventricle and potentially result in heart failure. The co-occurrence of these diseases is common as systemic sclerosis can impact the small pulmonary arteries, causing them to narrow and become obstructed, thus precipitating PAH.

Among individuals with systemic sclerosis, the prevalence of PAH is notably elevated, with approximately 10% to 12% of these patients experiencing this complication. Symptoms associated with PAH may encompass shortness of breath, fatigue, chest pain, and fainting. As the condition advances, these symptoms can intensify, significantly impairing the patient's quality of life and life expectancy. Consequently, for patients with systemic sclerosis, it is crucial to regularly monitor pulmonary function and undergo imaging studies to identify and manage PAH at an early stage.

For those living with both PAH and systemic sclerosis, maintaining a healthy lifestyle is essential, including quitting smoking, moderating salt intake, and engaging in appropriate physical activity. Moreover, patients should adhere to their physician's recommendations, attend regular follow-ups and check-ups to track the disease's progression, and adjust treatment strategies accordingly. In terms of pharmacological management, patients must use medications under the supervision of a healthcare provider and avoid self-medicating to prevent unnecessary risks.