Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Anna Smukowska-Gorynia
* ,
Weronika Gościniak
,
Patrycja Woźniak
,
Sylwia Iwańczyk
ORCID logo ,
Karolina Jaxa-Kwiatkowska
,
Sylwia Sławek-Szmyt
,
Magdalena Janus
,
Jerzy Paluszkiewicz
,
Tatiana Mularek-Kubzdela
Version 1 : Received: 10 August 2023 / Approved: 11 August 2023 / Online: 11 August 2023 (13:25:08 CEST)

A peer-reviewed article of this Preprint also exists.

Smukowska-Gorynia, A.; Gościniak, W.; Woźniak, P.; Iwańczyk, S.; Jaxa-Kwiatkowska, K.; Sławek-Szmyt, S.; Janus, M.; Paluszkiewicz, J.; Mularek-Kubzdela, T. Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases. Pharmaceuticals 2023, 16, 1252. Smukowska-Gorynia, A.; Gościniak, W.; Woźniak, P.; Iwańczyk, S.; Jaxa-Kwiatkowska, K.; Sławek-Szmyt, S.; Janus, M.; Paluszkiewicz, J.; Mularek-Kubzdela, T. Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases. Pharmaceuticals 2023, 16, 1252.

Abstract

Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to different groups: 1) pulmonary arterial hypertension (PAH), 2) PH secondary to lung disease and/or hypoxia, 3) PH due to left heart disease, and 4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis, therefore the early diagnosis should be established A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase 5 inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs e.g. sotatercept are intensively investigated in the clinical trials. The aim of this paper is to review the literature on the recent advances in treatment strategy and prognosis of patients with PAH-CTD.

Keywords

pulmonary arterial hypertension associated with connective tissue diseases; drugs; novel drugs; therapy; treatment; prognosis

Subject

Medicine and Pharmacology, Medicine and Pharmacology

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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