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Review
. 2019 Jun 29;30(2):90-93.
doi: 10.31138/mjr.30.2.90. eCollection 2019 Jun.

Pulmonary Arterial Hypertension in Connective Tissue Disorders: The emerging role of screening and early diagnosis. A position paper for Greek Rheumatologists

Eftychia Demerouti  1 , Iraklis Tsangaris  2 , Theodoros Dimitroulas  3 , George Giannakoulas  4 , Pelagia Katsimpri  5 , Ioanna Mitrouska  6 , Stylianos Orfanos  2 , Ioannis Skoularigkis  7 , Paraskevi Voulgari  8 , Petros Sfikakis  9
Affiliations
Review

Pulmonary Arterial Hypertension in Connective Tissue Disorders: The emerging role of screening and early diagnosis. A position paper for Greek Rheumatologists

Eftychia Demerouti et al. Mediterr J Rheumatol. .

Abstract

Pulmonary Arterial Hypertension is regarded as a devastating disease, complicating Connective Tissue Diseases. Although much progress has been achieved in the last 20 years, several unfulfilled needs in diagnosis and management of PAH in these patients may still be identified. After a systematic review of the literature and integrating results from the latest research articles, key clinical issues for the screening and diagnosis of Pulmonary Arterial Hypertension in Connective Tissue Disorder Patients and specifically Scleroderma patients are described in this article, allowing physicians to contribute to early diagnosis of patients with Scleroderma-associated Pulmonary Arterial Hypertension.

Keywords: Pulmonary arterial hypertension; connective tissue disorders; scleroderma.

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