[Pulmonary arterial hypertension and systemic sclerosis]

Area of Science:

• Cardiology
• Rheumatology
• Pulmonology

Background:

• Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc), significantly increasing mortality.
• An estimated 10-15% of SSc patients develop PAH, often with a poorer prognosis than idiopathic PAH.
• Current screening guidelines recommend annual echocardiography for SSc patients.

Purpose of the Study:

• To review the prevalence, diagnosis, and treatment of PAH in SSc patients.
• To discuss the efficacy of current therapeutic options for SSc-related PAH.
• To highlight the challenges in managing SSc-associated PAH.

Main Methods:

• Literature review of recent publications on SSc-related PAH.
• Analysis of diagnostic criteria and screening recommendations (echocardiography, right heart catheterization).
• Evaluation of therapeutic strategies including prostacyclin therapy, prostacyclin analogs, endothelin receptor antagonists, and PDE5 inhibitors.

Main Results:

• SSc-related PAH has a high prevalence (10-15%) and poor prognosis.
• Many SSc patients present with advanced WHO functional class III or IV symptoms.
• Effective treatments include intravenous epoprostenol, prostacyclin analogs (treprostinil, iloprost), bosentan, and sildenafil.

Conclusions:

• Early detection and intervention are critical for improving outcomes in SSc-related PAH.
• While several treatments show efficacy, long-term data, especially for newer agents like sildenafil in scleroderma, is still needed.
• Multidisciplinary management is essential for patients with this complex condition.