Pulmonary Arterial Hypertension in Thai Patients with Systemic Sclerosis

A recent consensus categorized pulmonary arterial hypertension (PAH) into multiple groups, including idiopathic PAH (IPAH), familial PAH (FPAH), and PAH associated with other diseases(1). The presence of PAH is associated with significant mortality and morbidity in patients with connective tissue disease, especially PAH associated with sys-temic sclerosis (PAH-SSc), which is the connective tissue disease mostly associated with PAH(2-4). Systemic sclerosis (SSc) is a systemic auto-immune disease, which involves mainly the skin, heart, renal, pulmonary, and gastrointestinal system. SSc is classified into two patterns; limited systemi