Pulmonary arterial hypertension associated with systemic sclerosis

Abstract

Systemic sclerosis (SSc) is commonly complicated by pulmonary arterial hypertension (PAH), which is a leading cause of death in the SSc patient population. Owing to the fact that the risk of developing pulmonary hypertension is high, screening is important, although the optimal modality remains to be defined. Furthermore, despite recent advances in therapy for PAH, the response to these interventions in patients with PAH with SSc has been discouraging. The lack of clinical response to these therapies may merely reflect the limitations of traditionally employed PAH outcome measures in SSc-PAH patients or highlight the heterogeneity of the disease manifestations within SSc. Importantly, since extrapulmonary involvement of the GI tract and kidneys by SSc limit candidacy for lung transplantation, new therapies that target abnormal cellular proliferation in the pulmonary vasculature are currently under investigation and may be particularly relevant to SSc-PAH.

Figure 1. Proposed diagnostic algorithm for pulmonary arterial hypertension in patients with systemic sclerosis employed by the Johns Hopkins Pulmonary Hypertension Program

†Elevated jugular venous pressure, increased second heart sound, RV tap or heave, peripheral edema. DL_CO: Diffusing capacity for carbon monoxide; FVC: Forced vital capacity; NT-proBNP: N-terminal pro-brain natriuretic peptide; PH: Pulmonary hypertension; RHC: Right heart catheterization; RV: Right ventricle; TAPSE: Tricuspid annular plane systolic excursion; V_A: Alveolar volume. Adapted from [137].

Figure 2. Clinical treatment algorithm for pulmonary arterial hypertension associated with systemic sclerosis employed by the Johns Hopkins Pulmonary Hypertension Program

6MWD: 6-min walk distance; 6MWT: 6-min walk test; AS: Atrial septostomy; ERA: Endothelin receptor antagonist; iv.: Intravenous; NYHA: New York Heart Association; PDE-5-I: Phosphodiesterase type-5 inhibitor; sc.: Subcutaneous. Reproduced from [138].