Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment

Didem Saygin,1 Robyn T Domsic2 1Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA; 2Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USACorrespondence: Robyn T DomsicDivision of Rheumatology and Clinical...

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Autores principales:	Saygin D, Domsic RT
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2019
Materias:	scleroderma pulmonary hypertension screening Diseases of the musculoskeletal system RC925-935
Acceso en línea:	https://doaj.org/article/86b5fef6ae60474eafe068dafbdf52b1
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spelling	oai:doaj.org-article:86b5fef6ae60474eafe068dafbdf52b12021-12-02T04:14:45ZPulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment1179-156Xhttps://doaj.org/article/86b5fef6ae60474eafe068dafbdf52b12019-12-01T00:00:00Zhttps://www.dovepress.com/pulmonary-arterial-hypertension-in-systemic-sclerosis-challenges-in-di-peer-reviewed-article-OARRRhttps://doaj.org/toc/1179-156XDidem Saygin,1 Robyn T Domsic2 1Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA; 2Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USACorrespondence: Robyn T DomsicDivision of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, S706 BST, 200 Lothrop Street, Pittsburgh, PA 15213, USATel +1 412 383 8000Fax +1 412 383 8765Email rtd4@pitt.eduAbstract: Systemic sclerosis (SSc) is a chronic, multisystem autoimmune disease characterized by vasculopathy, fibrosis and immune system activation. Pulmonary hypertension and interstitial lung disease account for majority of SSc-related deaths. Diagnosis of SSc-PAH can be challenging due to nonspecific clinical presentation which can lead to delayed diagnosis. Many screening algorithms have been developed to detect SSc-associated pulmonary arterial hypertension (SSc-PAH) in early stages. Currently used PAH-specific medications are largely extrapolated from IPAH studies due to smaller number of patients with SSc-PAH. In this review, we discuss the current state of knowledge in epidemiology and risk factors for development of SSc-PAH, and challenges and potential solutions in the diagnosis, screening and management of SSc-PAH.Keywords: scleroderma, pulmonary hypertension, screeningSaygin DDomsic RTDove Medical Pressarticlesclerodermapulmonary hypertensionscreeningDiseases of the musculoskeletal systemRC925-935ENOpen Access Rheumatology: Research and Reviews, Vol Volume 11, Pp 323-333 (2019)
institution	DOAJ
collection	DOAJ
language	EN
topic	scleroderma pulmonary hypertension screening Diseases of the musculoskeletal system RC925-935
spellingShingle	scleroderma pulmonary hypertension screening Diseases of the musculoskeletal system RC925-935 Saygin D Domsic RT Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment
description	Didem Saygin,1 Robyn T Domsic2 1Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA; 2Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USACorrespondence: Robyn T DomsicDivision of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, S706 BST, 200 Lothrop Street, Pittsburgh, PA 15213, USATel +1 412 383 8000Fax +1 412 383 8765Email rtd4@pitt.eduAbstract: Systemic sclerosis (SSc) is a chronic, multisystem autoimmune disease characterized by vasculopathy, fibrosis and immune system activation. Pulmonary hypertension and interstitial lung disease account for majority of SSc-related deaths. Diagnosis of SSc-PAH can be challenging due to nonspecific clinical presentation which can lead to delayed diagnosis. Many screening algorithms have been developed to detect SSc-associated pulmonary arterial hypertension (SSc-PAH) in early stages. Currently used PAH-specific medications are largely extrapolated from IPAH studies due to smaller number of patients with SSc-PAH. In this review, we discuss the current state of knowledge in epidemiology and risk factors for development of SSc-PAH, and challenges and potential solutions in the diagnosis, screening and management of SSc-PAH.Keywords: scleroderma, pulmonary hypertension, screening
format	article
author	Saygin D Domsic RT
author_facet	Saygin D Domsic RT
author_sort	Saygin D
title	Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment
title_short	Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment
title_full	Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment
title_fullStr	Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment
title_full_unstemmed	Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment
title_sort	pulmonary arterial hypertension in systemic sclerosis: challenges in diagnosis, screening and treatment
publisher	Dove Medical Press
publishDate	2019
url	https://doaj.org/article/86b5fef6ae60474eafe068dafbdf52b1
work_keys_str_mv	AT saygind pulmonaryarterialhypertensioninsystemicsclerosischallengesindiagnosisscreeningandtreatment AT domsicrt pulmonaryarterialhypertensioninsystemicsclerosischallengesindiagnosisscreeningandtreatment
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Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment

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