• Vivek Thakkar and Edmund M T Lau.
• Department of Rheumatology, Liverpool Hospital, Sydney, New South Wales, Australia; School of Medicine, Western Sydney University, Campbelltown, New South Wales, Australia; South Western Sydney Clinical School, University of New South Wales, Liverpool, Australia; Ingham Institute, Liverpool, Australia. Electronic address: V.Thakkar@westernsydney.edu.au.
• Best Pract Res Clin Rheumatol. 2016 Feb 1; 30 (1): 22-38.

AbstractOver the past two decades, there have been several advances in the assessment and management of connective tissue disease-related pulmonary arterial hypertension (CTD-PAH) that improved outcomes of the treatment of this lethal disease, and this will be the focus of this study. Systemic sclerosis is the leading cause of CTD-PAH, followed by systemic lupus erythematosus, mixed connective tissue disease, idiopathic inflammatory myositis, rheumatoid arthritis, and Sjogren's syndrome. Clinical registries have been invaluable in informing about the burden of disease, risk and prognostic factors, and temporal trends with respect to treatment and outcome in CTD-PAH. The major advances have centered on improved disease classification and diagnostic criteria, screening and early diagnosis, the emergence of evidence-based therapies including combination goal-orientated treatment strategies, and the establishment of centers with expertise in PAH.Crown Copyright © 2016. Published by Elsevier Ltd. All rights reserved.

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