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      Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study


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          Earlier detection of pulmonary arterial hypertension (PAH), a leading cause of death in systemic sclerosis (SSc), facilitates earlier treatment. The objective of this study was to develop the first evidence-based detection algorithm for PAH in SSc.


          In this cross-sectional, international study conducted in 62 experienced centres from North America, Europe and Asia, adults with SSc at increased risk of PAH (SSc for >3 years and predicted pulmonary diffusing capacity for carbon monoxide <60%) underwent a broad panel of non-invasive assessments followed by diagnostic right heart catheterisation (RHC). Univariable and multivariable analyses selected the best discriminatory variables for identifying PAH. After assessment for clinical plausibility and feasibility, these were incorporated into a two-step, internally validated detection algorithm. Nomograms for clinical practice use were developed.


          Of 466 SSc patients at increased risk of PAH, 87 (19%) had RHC-confirmed PAH. PAH was mild (64% in WHO functional class I/II). Six simple assessments in Step 1 of the algorithm determined referral to echocardiography. In Step 2, the Step 1 prediction score and two echocardiographic variables determined referral to RHC. The DETECT algorithm recommended RHC in 62% of patients (referral rate) and missed 4% of PAH patients (false negatives). By comparison, applying European Society of Cardiology/European Respiratory Society guidelines to these patients, 29% of diagnoses were missed while requiring an RHC referral rate of 40%.


          The novel, evidence-based DETECT algorithm for PAH detection in SSc is a sensitive, non-invasive tool which minimises missed diagnoses, identifies milder disease and addresses resource usage.

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          Most cited references19

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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.

          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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            Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry.

            The Registry to EValuate Early And Long-term pulmonary arterial hypertension disease management (REVEAL Registry) was established to provide updated characteristics of patients with pulmonary arterial hypertension (PAH) and to improve diagnosis, treatment, and management. Fifty-four US centers enrolled consecutively screened patients with World Health Organization group I PAH who met expanded hemodynamic criteria of mean pulmonary arterial pressure (PAP) > 25 mm Hg at rest (30 mm Hg with exercise), pulmonary capillary wedge pressure (PCWP) or= 240 dynes x s x cm(-5). Patients meeting the traditional hemodynamic definition (PCWP
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              Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers.

              This study sought to assess the risks associated with right heart catheter procedures in patients with pulmonary hypertension. Right heart catheterization, pulmonary vasoreactivity testing, and pulmonary angiography are established diagnostic tools in patients with pulmonary hypertension, but the risks associated with these procedures have not been systematically evaluated in a multicenter study. We performed a multicenter 5-year retrospective and 6-month prospective evaluation of serious adverse events related to right heart catheter procedures in patients with pulmonary hypertension, as defined by a mean pulmonary artery pressure >25 mm Hg at rest, undergoing right heart catheterization with or without pulmonary vasoreactivity testing or pulmonary angiography. During the retrospective period, 5,727 right heart catheter procedures were reported, and 1,491 were reported from the prospective period, for a total of 7,218 right heart catheter procedures performed. The results from the retrospective and the prospective analyses were almost identical. The overall number of serious adverse events was 76 (1.1%, 95% confidence interval 0.8% to 1.3%). The most frequent complications were related to venous access (e.g., hematoma, pneumothorax), followed by arrhythmias and hypotensive episodes related to vagal reactions or pulmonary vasoreactivity testing. The vast majority of these complications were mild to moderate in intensity and resolved either spontaneously or after appropriate intervention. Four fatal events were recorded in association with any of the catheter procedures, resulting in an overall procedure-related mortality of 0.055% (95% confidence interval 0.01% to 0.099%). When performed in experienced centers, right heart catheter procedures in patients with pulmonary hypertension are associated with low morbidity and mortality rates.

                Author and article information

                Ann Rheum Dis
                Ann. Rheum. Dis
                Annals of the Rheumatic Diseases
                BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )
                July 2014
                18 May 2013
                : 73
                : 7
                : 1340-1349
                [1 ]Cardiology Department, Royal Free Hospital , London, UK
                [2 ]Centre for Rheumatology, Royal Free Hospital , London, UK
                [3 ]Centre for Pulmonary Hypertension, University Hospital , Heidelberg, Germany
                [4 ]Medical University of Vienna, Department of Internal Medicine II, Division of Cardiology , Vienna, Austria
                [5 ]Department of Rheumatology, University Hospital Zurich , Zurich, Switzerland
                [6 ]Department of Internal Medicine, University of Michigan , Ann Arbor, Michigan, USA
                [7 ]Department of Rheumatology and Clinical Immunology, Justus-Liebig-University Giessen, Kerckhoff Clinic Bad Nauheim, Germany
                [8 ]Department of Medicine, Division of Rheumatology, Western University of Canada , London, Ontario, Canada
                [9 ]Department of Rheumatology, Radboud University Nijmegen Medical Centre , Nijmegen, The Netherlands
                [10 ]Global Medical Affairs, Actelion Pharmaceuticals Ltd, Allschwil , Switzerland
                [11 ]Clinical Development, Actelion Pharmaceuticals Ltd , Allschwil, Switzerland
                [12 ]Center for Medical Statistics, Informatics, and Intelligent Systems, Medical University of Vienna , Vienna, Austria
                [13 ]Scleroderma Research Consultants LLC , Avon, Connecticut, USA
                Author notes

                Handling editor Tore K Kvien

                [Correspondence to ] Dr J Gerry Coghlan, Cardiology Department, Royal Free Hospital, Pond Street, Hampstead, London NW3 2QG, UK; gerry.coghlan@ 123456nhs.net

                JGC, CPD and EK contributed equally.

                Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions

                This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 3.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/3.0/

                : 14 April 2013
                Clinical and Epidemiological Research
                Extended Report
                Custom metadata

                systemic sclerosis,arterial hypertension,epidemiology
                systemic sclerosis, arterial hypertension, epidemiology


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