Early detection of pulmonary arterial hypertension in systemic sclerosis: A French nationwide prospective multicenter study

Abstract

Objective. Screening allows for early management of pulmonary arterial hypertension (PAH), a severe complication of systemic sclerosis (SSc). Since no consensus has been reached on the method and criteria for optimal screening, we sought to develop an algorithm based on symptoms, Doppler echocardiography, and right heart catheterization (RHC) for application to a nationwide multicenter SSc population in France.Methods. This prospective study was conducted Conclusion. This screening algorithm, based on dyspnea, Doppler echocardiographic evaluation of VTR, and RHC, enabled early detection of PAH at a mild stage. Whether mild PAH will evolve to severe PAH in reported cases and whether this early diagnosis translates into improved prognosis for patients with mild PAH will be evaluated in the ongoing 3-year followup of this cohort.Pulmonary arterial hypertension (PAH) is a disease of the small pulmonary arteries, characterized by a progressive increase in pulmonary vascular resistance, ultimately causing right ventricular failure and death. PAH is defined as a mean pulmonary artery pressure (mPAP) Ն25 mm Hg at rest or Ն30 mm Hg during Supported by a research grant from Actelion Pharmaceuticals France for the logistical support, monitoring, project management, data management, and statistical analysis of this study.

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

“…Based on right heart catheterization, the prevalence of resting PAH in SSc is likely 7.9-20% (7,17). However, histopathologic evidence of pulmonary arteriopathy has been documented in up to 72% of patients with limited scleroderma, suggesting discordance between clinical findings and pathologic diagnosis (18).…”

Guidelines for the diagnosis and treatment of pulmonary hypertension

“…The prevalence of haemodynamically proven PAH in large cohorts of patients with systemic sclerosis is between 7 and 12% [2,114]. In these patients, PAH may occur in association with interstitial fibrosis or as a result of an isolated pulmonary arteriopathy.…”

Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group

“…Out of 124 articles identified, 87 were excluded based on predefined inclusion/exclusion criteria, two studies could not be retrieved for full text review, and 35/124 articles were included for further analysis [references [1][2][3][4]. The search strategy of the systematic literature research including reasons for exclusions is presented in Figure 1.…”

“…Indeed, significant lung disease, which might lead to pulmonary hypertension (PH) due to hypoxemia, was found in up to 30-75% of SSc patients with elevated PAP [1][2]. Left heart dysfunction, which might cause postcapillary/venous PH, was found in up to 13%-19% of SSc patients suspected of having PAH [3][4].…”