Diversity of borderline pulmonary arterial pressure associated with systemic sclerosis: 3 case series

Mean pulmonary arterial pressure of 21 to 24 mmHg, called borderline pulmonary arterial pressure (BoPAP), may represent a pre-pulmonary arterial hypertension (PAH) condition but has not so far been indicated for specific PAH therapy. Despite the recent progress in PAH-targeted therapy, the prognosis of patients with systemic sclerosis (SSc)-associated PAH still remains poor. One of the possible strategies to improve the prognosis of those patients is the early detection and therapeutic intervention of pre-PAH conditions. We present three different clinical courses of BoPAP associated with SSc, which include successful treatment with bosentan and tocilizumab, an improvement following the treatment of interstitial lung disease, and spontaneous progression to fatal PAH, and suggest the efficacy of frequent follow-up examinations and therapeutic intervention for BoPAP in SSc patients.