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      Borderline pulmonary arterial pressure in systemic sclerosis patients: a post-hoc analysis of the DETECT study

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          Abstract

          Introduction

          Patients with mean pulmonary artery pressures (mPAP) of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)—a condition thought to represent early-stage pulmonary arterial vasculopathy. Based on the DETECT study, this post-hoc analysis examined patient characteristics of systemic sclerosis (SSc) patients with normal mPAP, BoPAP and elevated mPAP, fulfilling pulmonary arterial hypertension (PAH) criteria.

          Methods

          Adult patients with a duration of SSc more than 3 years, a diffusing capacity of the lung for carbon monoxide less than 60% predicted, and no previous diagnosis of any form of pulmonary hypertension (PH) underwent screening tests followed by right heart catheterization. Subjects were divided into three groups: normal mPAP, BoPAP, and PAH. Exploratory comparative and binary logistic regression analyses were performed for the BoPAP versus normal mPAP and PAH versus BoPAP groups.

          Results

          Of 244 patients evaluated, 148 (60%) had normal mPAP, 36 (15%) had BoPAP , and 60 (25%) had definite PAH. Univariable logistic regression (ULR) showed the mean tricuspid regurgitation velocity in patients with BoPAP to be intermediate between normal mPAP and PAH. In the ULR analyses BoPAP versus normal mPAP and PAH versus BoPAP, the statistically significant predictors were, amongst others: demographic, clinical, pulmonary function, echocardiographic and hemodynamic variables.

          Conclusions

          In this exploratory post-hoc analysis of the DETECT study population patients with BoPAP could be distinguished from patients with normal mPAP and PAH, and it appears that BoPAP may be an intermediate stage on the continuum between normal PA pressures and PAH.

          Electronic supplementary material

          The online version of this article (doi:10.1186/s13075-014-0493-1) contains supplementary material, which is available to authorized users.

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          Most cited references17

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          Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee.

          (1980)
          A multicenter, ongoing study of early-diagnosed cases of systemic sclerosis and comparison patients with systemic lupus erythematosus, polymyositis/dermatomyositis, and Raynaud's phenomenon was conducted in order to develop classification criteria for systemic sclerosis. Preliminary criteria are proposed namely, the finding of either the sole major criterion, i.e., proximal scleroderma, or two or more of the minor criteria, i.e., 1) sclerodactyly, 2) digital pitting scars of fingertips or loss of substance of the distal finger pad, and 3) bilateral basilar pulmonary fibrosis. When applied to the case and comparison patients included in this study, these proposed criteria had a 97% sensitivity for definite systemic sclerosis and 98% specificity.
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            ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association.

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              Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review.

              According to current guidelines, pulmonary arterial hypertension (PAH) is diagnosed when mean pulmonary arterial pressure (Ppa) exceeds 25 mmHg at rest or 30 mmHg during exercise. Issues that remain unclear are the classification of Ppa values 30 mmHg during exercise is always pathological. We performed a comprehensive literature review and analysed all accessible data obtained by right heart catheter studies from healthy individuals to determine normal Ppa at rest and during exercise. Data on 1,187 individuals from 47 studies in 13 countries were included. Data were stratified for sex, age, geographical origin, body position and exercise level. Ppa at rest was 14.0+/-3.3 mmHg and this value was independent of sex and ethnicity. Resting Ppa was slightly influenced by posture (supine 14.0+/-3.3 mmHg, upright 13.6+/-3.1 mmHg) and age ( or = 50 yrs: 14.7+/-4.0 mmHg). Ppa during exercise was dependent on exercise level and age. During mild exercise, Ppa was 19.4+/-4.8 mmHg in subjects aged or = 50 yrs (p<0.001). In conclusion, while Ppa at rest is virtually independent of age and rarely exceeds 20 mmHg, exercise Ppa is age-related and frequently exceeds 30 mmHg, especially in elderly individuals, which makes it difficult to define normal Ppa values during exercise.
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                Author and article information

                Contributors
                shv@med.umic.edu
                oliver.distler@usz.ch
                gerry.coghlan@nhs.net
                c.denton@ucl.ac.uk
                ekkehard.gruenig@med.uni-heidelberg.de
                diana.bonderman@meduniwien.ac.at
                u.mueller-ladner@kerckhoff-klinik.de
                janet.pope@sjhc.london.on.ca
                Madelon.Vonk@radboudumc.nl
                jamesrseibold@gmail.com
                jvtorres@syntaxfs.com
                martin.doelberg@actelion.com
                harbajan.chadha-boreham@actelion.com
                daniel.rosenberg@actelion.com
                vmclaugh@med.umich.edu
                khannad@med.umich.edu
                Journal
                Arthritis Res Ther
                Arthritis Research & Therapy
                BioMed Central (London )
                1478-6354
                1478-6362
                10 December 2014
                10 December 2014
                2014
                : 16
                : 6
                : 493
                Affiliations
                [ ]Division of Cardiology, Department of Medicine, University of Michigan, Ann Arbor, MI USA
                [ ]Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland
                [ ]Cardiology Department, Royal Free Hospital, London, UK
                [ ]Centre for Rheumatology, Royal Free Hospital, London, UK
                [ ]Centre for Pulmonary Hypertension, University Hospital, Heidelberg, Germany
                [ ]Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria
                [ ]Department of Rheumatology and Clinical Immunology, Justus-Liebig University, Giessen, Germany
                [ ]Department of Medicine, Division of Rheumatology, Western University of Canada, London, ON Canada
                [ ]Department of Rheumatology, Radboud University Medical Centre, Nijmegen, The Netherlands
                [ ]Scleroderma Research Consultants LLC, Litchfield, CT USA
                [ ]Actelion Pharmaceuticals Ltd, Allschwil, Switzerland
                [ ]Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan USA
                [ ]Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, 300 North Ingalls Street, Suite 7C27, Ann Arbor, MI 48109 USA
                Article
                493
                10.1186/s13075-014-0493-1
                4299685
                25491468
                0b59b7fe-92b6-4961-aa91-a1878f2a0524
                © Visovatti et al.; licensee BioMed Central. 2014

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 29 May 2014
                : 12 November 2014
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                © The Author(s) 2014

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