• Eur. J. Clin. Invest. · Feb 2018

    Review

    Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects.

    • Masaru Kato and Tatsuya Atsumi.
    • Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
    • Eur. J. Clin. Invest. 2018 Feb 1; 48 (2).

    AbstractRecent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH) has some distinctive clinical aspects from other PAH, such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre-PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD-PAH and garner attention to areas where further evidence is desired.© 2017 Stichting European Society for Clinical Investigation Journal Foundation.

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