scleroderma pulmonary hypertension

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a rare but severe complication, often presenting with symptoms such as shortness of breath, fatigue, and syncope. This condition is linked to the narrowing and obstruction of small pulmonary arteries due to systemic sclerosis, an autoimmune disease, which leads to elevated pulmonary artery pressure.

In the early stages of SSc-PAH, symptoms like mild shortness of breath and fatigue may be overlooked as common tiredness or a normal part of aging. As the disease advances, patients may experience more pronounced shortness of breath, particularly with exertion, and paroxysmal nocturnal dyspnea. The heart's increased effort to overcome the high pulmonary artery pressure can result in palpitations and chest pain. Severe cases of pulmonary arterial hypertension can lead to right ventricular failure, characterized by peripheral edema and ascites.

For individuals with SSc-PAH, it is crucial to avoid strenuous exercise and high-altitude environments to prevent exacerbation of breathing difficulties. Maintaining a balanced diet and engaging in moderate physical activity can contribute to better health. In terms of treatment, adherence to medical guidance is paramount, including taking prescribed medications as directed and attending regular check-ups for timely adjustments to the treatment plan. It is imperative not to alter medication dosages or discontinue medication without consulting a healthcare professional to prevent disease progression.