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Open Access 20-02-2024 | Systemic Sclerosis | IM - ORIGINAL

Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis

Authors: Mustafa Erdogan, Sinem Nihal Esatoglu, Burcak Kilickiran Avci, Gulen Hatemi

Published in: Internal and Emergency Medicine | Issue 3/2024

Abstract

The evidence for the treatment of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) mostly depends on subgroup or post hoc analysis of randomized controlled trials (RCTs). Thus, we performed a meta-analysis of RCTs that reported outcomes for CTD-PAH. PubMed and EMBASE were searched for CTD-PAH treatment. The selected outcomes were functional class (FC) change, survival rates, 6-min walk distance (6-MWD), clinical worsening (CW), N-terminal prohormone BNP (NT-proBNP), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), right atrial pressure (RAP), and cardiac index (CI). The meta-analysis was conducted according to the PRISMA guidelines and registered in PROSPERO (CRD42020153560). Twelve RCTs conducted with 1837 patients were included. The diagnoses were systemic sclerosis in 59%, SLE in 20%, and other CTDs in 21%. The pharmacological interventions were epoprostenol, treprostinil, sildenafil, tadalafil, bosentan, macitentan, ambrisentan, riociguat, and selexipag. There was a significant difference between interventions and placebo in FC, 6MWD, CW, PVR, RAP, and CI that favored intervention. Our analysis showed a 39% reduction in the CW risk with PAH treatment. The short-term survival rates and mean serum NT-proBNP changes were similar between the study and control groups. Treatment for CTD-PAH had favorable effects on clinical and hemodynamic outcomes but not on survival and NT-proBNP levels. Different from the previous meta-analyses that focused on 6-MWD, time to clinical worsening, and CW as outcomes, this meta-analysis additionally reports the pooled analysis of change in FC, hemodynamic measurements (RAP, PVR, CI), and NT-proBNP, some of which have prognostic value for PAH.

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Title: Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis
Authors: Mustafa Erdogan
Sinem Nihal Esatoglu
Burcak Kilickiran Avci
Gulen Hatemi
Publication date: 20-02-2024
Publisher: Springer International Publishing
Keywords: Systemic Sclerosis
Arterial Hypertension
Arterial Hypertension
Riociguat
Sildenafil
Systemic Lupus Erythematosus
Tadalafile
Ambrisentan
Bosentan
Published in: Internal and Emergency Medicine / Issue 3/2024
Print ISSN: 1828-0447
Electronic ISSN: 1970-9366
DOI: https://doi.org/10.1007/s11739-024-03539-1

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