open access

Online first
Expert opinion
Published online: 2021-07-01
Get Citation

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

Tatiana Mularek-Kubzdela, Michał Ciurzyński, Otylia Kowal Bielecka, Jarosław D Kasprzak, Grzegorz Kopeć, Katarzyna Mizia-Stec, Ewa Mroczek, Ewa Lewicka, Ilona Skoczylas, Marek Grabka, Michał Furdal, Michał Florczyk, Marek Brzosko, Bogdan Batko, Hanna Przepiera-Będzak, Włodzimierz Samborski, Eugeniusz J Kucharz
DOI: 10.33963/KP.a2021.0055
·
Pubmed: 34227677

open access

Online first
Expert opinion and position paper
Published online: 2021-07-01

Abstract

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%–12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Abstract

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%–12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Get Citation

Keywords

pulmonary hypertension, connective tissue disease, autoimmune disease, systemic sclerosis, systemic lupus erythematosus

Supp./Additional Files (1)
Supplementary material
Download
629KB
About this article
Title

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Online first

Article type

Expert opinion

Published online

2021-07-01

DOI

10.33963/KP.a2021.0055

Pubmed

34227677

Keywords

pulmonary hypertension
connective tissue disease
autoimmune disease
systemic sclerosis
systemic lupus erythematosus

Authors

Tatiana Mularek-Kubzdela
Michał Ciurzyński
Otylia Kowal Bielecka
Jarosław D Kasprzak
Grzegorz Kopeć
Katarzyna Mizia-Stec
Ewa Mroczek
Ewa Lewicka
Ilona Skoczylas
Marek Grabka
Michał Furdal
Michał Florczyk
Marek Brzosko
Bogdan Batko
Hanna Przepiera-Będzak
Włodzimierz Samborski
Eugeniusz J Kucharz

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

By VM Media Sp. z o.o. VM Group Sp.k., ul. Świętokrzyska 73 , 80–180 Gdańsk, Poland

phone:+48 58 320 94 94, fax:+48 58 320 94 60, e-mail: viamedica@viamedica.pl