Beyond Scleroderma: Pulmonary Arterial Hypertension in Patients with Other Connective Tissue Diseases

Pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD-PAH) is a devastating condition that may progress rapidly to cause right ventricular dysfunction, resulting in significant morbidity and mortality. The pathobiology, epidemiology, natural history, early diagnosis, and treatment response of PAH associated with scleroderma (SSc-PAH) have been the subjects of intense research efforts over the previous decade. The success of these efforts has resulted in increased awareness of SSc-PAH. Less is known about PAH associated with other connective tissue diseases. The aim of this chapter is to move beyond scleroderma in order to review what is known about PAH in other CTDs.