Pulmonary hypertension phenotypes in patients with systemic sclerosis

Abstract

Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a heterogenous condition and several different forms can be associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to left heart disease and PH due to interstitial lung disease. The incidence of pulmonary veno-occlusive disease is also increased. Accurate and early diagnosis to allow optimal treatment is, therefore, essential. Recent changes to diagnostic haemodynamic criteria at the 6th World Symposium on Pulmonary Hypertension have resulted in therapeutic uncertainty regarding patients with borderline pulmonary haemodynamics. Furthermore, the optimal pulmonary vascular resistance threshold for diagnosing PAH and the role of exercise in identifying early disease require further elucidation. In this article we review the epidemiology, diagnosis, outcomes and treatment of the spectrum of pulmonary vascular phenotypes associated with SSc.

Cardiovascular Imaging for Systemic Sclerosis Monitoring and Management

“…SSc associated-PAH (SSc-PAH) is considered WHO Group 1 PH and may be the result of direct remodeling of the pre-capillary pulmonary arterioles, or rarely be attributable to pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH). It is a leading cause of mortality in SSc patients, with an incidence of 8–13% ( 12 , 13 ) and is more commonly associated with limited cutaneous systemic sclerosis (lcSSc) ( 14 ), Patients with SSc-PAH have a poor prognosis in comparison to SSc patients without PAH, with 3 year survival of only 52% ( 14 – 16 ). Several studies have demonstrated that the intrinsic RV dysfunction in SSc patients leads to worse outcomes in SSc-PAH in comparison to idiopathic PAH ( 2 , 13 , 17 ).…”

“…One year mortality for SSc-PAH has been found to be 30%, while one year mortality for those with idiopathic PAH is 15% ( 17 ). Mortality rates remain high despite therapy ( 14 ).…”

“…Rarely, patients with SSc may develop chronic thromboembolic pulmonary hypertension (CTEPH) and be classified as WHO Group 4 PH. Distinguishing among the different mechanisms of SSc associated PH is important as treatment and prognosis are impacted by a timely and accurate diagnosis ( 14 , 19 , 20 ).…”

“…Notably, STE is a novel way to detect RV dysfunction earlier and its role in SSc associated RV dysfunction and PH is currently being studied. It is important to screen patients for PH and RV dysfunction early, as studies have demonstrated improved survival and improved treatment response in SSc-PAH patients with mild hemodynamics and symptoms ( 14 , 21 ).…”

“…Although exercise-induced PH has clinical and prognostic significance in patients with cardiopulmonary conditions, there remains a lack of standard definition for exercise-induced PH and a lack of standard measures for how the RV and pulmonary vasculature respond to exercise ( 14 ). Further study is needed to determine the utility of exercise echocardiography in unmasking PH, as well as the use of exercise echocardiography in assessing RV and PV contractile reserve.…”