Complex challenges of pulmonary hypertension

Abstract

It is now well appreciated that pulmonary hypertension (PH) is a frequent complication of scleroderma and that it occurs in both of the major disease subsets. However, despite a much greater understanding of the different forms of pulmonary hypertension and the availability of effective treatments for different forms of this disease, it remains one of the most feared complications of scleroderma. Compared with other forms fo PAH, that which occurs in scleroderma has a significantly worse outcome, and this is especially in comparison with idiopathic PAH. Thus, the strategies that are used for PAH may need to be modified in the context of scleroderma, and this is discussed below. In addition, there are other common causes for pulmonary hypertension in scleroderma, and it is likely that more than one process leading to elevated pulmonary pressures may operate in individual cases. Thus, it is necessary to consider the contribution of pulmonary arterial hypertension as well as left heart disease, interstitial lung disease and other mechanisms such as pulmonary veno-occlusive disease and thromboembolic disease. This chapter reviews the complexity of managing pulmonary hypertension in scleroderma, gives particular focus to the application of licensed therapies for PAH and the extent to which an admixture of underlying mechanisms may operate that justify consideration in managing this complication.