Interstitial Lung Disease-Associated Pulmonary Hypertension in the Connective Tissue Disorders

Pulmonary hypertension (PH) is an incurable condition that is associated with high morbidity and morbidity. In most cases, PH eventually leads to right ventricular hypertrophy followed by dilation, right ventricular failure, and death. PH is common in connective tissue diseases (CTD) and portends a poor prognosis. Although CTD-associated pulmonary arterial hypertension (CTD–APAH) is an important subgroup within Group 1 [1] pulmonary arterial hypertension, it is important to recognize that all groups of PH can occur in the setting of connective tissue disease (Table 9.1). Since interstitial lung disease (ILD) is also a common contributor to morbidity and mortality in the CTDs, this chapter will focus on the challenge of Group 3 pulmonary hypertension, which carries the worst prognosis.