Management of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons.

Pulmonary arterial hypertension (PAH) is a severe disease characterised by a progressive thickening and obliteration of pulmonary vessels, resulting in increased vascular resistance, elevated pulmonary artery pressures, and right heart failure. Among the various conditions associated with PAH, systemic sclerosis (SSc) is the most common in Western countries. Compared to other forms of PAH, SSc-PAH presents with a more aggressive clinical course, poorer response to conventional therapies and a worse prognosis. However, despite these differences, the overall management of SSc-PAH remains close to idiopathic PAH; and therefore, there is a crucial need for treatment strategies dedicated to this disease. To help fill this gap, we assessed the level of evidence currently available on SSc-PAH management in a systematic literature review that compiled data regarding conventional therapies, immunosuppressants, nonconventional drugs and surgical/interventional procedures. For each study, we highlighted the results specific to the connective tissue disease or SSc subgroups, the haemodynamic characteristics of the patients, and their comorbidities. By doing so, we identified critical gaps in the field, consisting mostly of the lack of studies focusing on SSc-PAH, a substantial heterogeneity in haemodynamic severity (with notable scarcity of data for mild PAH) and the systematic exclusion of relevant comorbidities (such as interstitial lung disease). Building on these data and our cumulative experience, we provide pragmatic, experience-based suggestions tailored to the management of SSc-PAH, that tries to capture the full scope of clinical situations encountered in these patients and help clinicians manage difficult cases where robust data are lacking.