Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis

Abstract

The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide () <60% predicted.In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses.96 patients with a mean pulmonary arterial pressure (mPAP) <25 mmHg at baseline were followed for 2.95±0.7 years (median 3 years). Of these, 71 had a second RHC; 18 of these 71 patients (25.3%) developed PH, and five (7%) developed SSc-associated pulmonary arterial hypertension. For patients with an mPAP of 21-24 mmHg at baseline, the likelihood of presenting with PH as opposed to normal pressures on follow-up was significantly higher (p=0.026). Pulmonary vascular resistance, tricuspid regurgitation velocity, diffusion capacity and the size of the inferior vena cava at baseline were independent predictors for the development of PH during follow-up.In a selected cohort of SSc patients with a <60%, pulmonary pressures appeared to rise progressively during follow-up. In this population, it was possible to identify manifest PH in almost 25% of patients using prospective RHC during follow-up. Therefore, regular clinical assessment including RHC might be useful in patients with SSc.

Mildly increased pulmonary arterial pressure: a new disease entity or just a marker of poor prognosis?

“…Third, subjects with risk factors for PH referred for right heart catheterization and a mild elevation in mPAP tend to present with increasing mPAP values and haemodynamic deterioration during follow‐up compared to similar patients with normal mPAP …”

“…Another approach used a pre‐defined mPAP threshold set at the upper limit of normal (20 mmHg) and analysed if study subjects above this threshold, but < 25 mmHg, differed by clinical profile and prognosis compared with the mPAP ≤ 20 mmHg subgroup . A third method aimed to utilise unbiased methodologies for determining mPAP thresholds indicating worse prognosis .…”

“…The differentiation of these causes is important at point of care, as each implicates different management and therapeutic approaches. Their short description is provided in Table …”

Clinical case of delayed systemic sclerosis and pulmonary arterial hypertension diagnostics

“…Among them 3 % developed PH and 7 % developed SSc-PAH. PVR, tricuspid regurgitation velocity, diffusion capacity and the size of the inferior vena cava at baseline were independent predictors for the development of PH during follow-up [14].…”

Pulmonary hypertension and chronic lung disease: where are we headed?

“…During the 6th World Symposium on Pulmonary Hypertension, held in Nice in 2018, a new mPAP cut-off value was suggested, as an mPAP of 20 mmHg is widely above the upper limit of normal value [5]. Furthermore, an increased risk of disease progression has been found in patients affected by pulmonary vascular diseases with an mPAP between 21 and 24 mmHg [5][6][7]. To define precapillary PH, as in the case of PH-CLD, the presence of an mPAP value >20 mmHg has to be associated to a pulmonary artery wedge pressure ⩽15 mmHg and a pulmonary vascular resistance ⩾3 WU.…”