Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database
Background:Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether disease presentation varies within Europe.Aims and Methods:The baseline data of a...
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Veröffentlicht in: | Annals of the rheumatic diseases 2009-06, Vol.68 (6), p.856-862 |
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description | Background:Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether disease presentation varies within Europe.Aims and Methods:The baseline data of all SSc patients entered in the EULAR Scleroderma Trials and Research (EUSTAR) database up to April 2007 were analysed for geographical differences with regard to organ involvement, and geographical clusters with regard to clinical subsets (diffuse vs limited SSc) and autoantibodies (anticentromere vs anti-Scl70).Results:3661 patients from 79 centres in 62 cities and 23 countries were analysed. There was no clear trend between geographical coordinates and SSc subsets, although there appeared to be an increased prevalence of Scl70 in the more eastern centres. There was no association between geographical longitude or latitude and the age at the onset of Raynaud’s phenomenon or the onset of non-Raynaud’s symptoms. There was also a trend for the more eastern centres to care for patients with a higher prevalence of more severe organ manifestations (pulmonary arterial hypertension, cardiac involvement). Between different centres within one city there was a large variability in the frequency of organ complications.Conclusion:This analysis suggests that eastern centres care for more severe SSc manifestations in Europe. Large differences in patient referral account for a large local variability of SSc presentations and preclude the identification of genetic or environmental factors. |
doi_str_mv | 10.1136/ard.2008.091348 |
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The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether disease presentation varies within Europe.Aims and Methods:The baseline data of all SSc patients entered in the EULAR Scleroderma Trials and Research (EUSTAR) database up to April 2007 were analysed for geographical differences with regard to organ involvement, and geographical clusters with regard to clinical subsets (diffuse vs limited SSc) and autoantibodies (anticentromere vs anti-Scl70).Results:3661 patients from 79 centres in 62 cities and 23 countries were analysed. There was no clear trend between geographical coordinates and SSc subsets, although there appeared to be an increased prevalence of Scl70 in the more eastern centres. There was no association between geographical longitude or latitude and the age at the onset of Raynaud’s phenomenon or the onset of non-Raynaud’s symptoms. There was also a trend for the more eastern centres to care for patients with a higher prevalence of more severe organ manifestations (pulmonary arterial hypertension, cardiac involvement). Between different centres within one city there was a large variability in the frequency of organ complications.Conclusion:This analysis suggests that eastern centres care for more severe SSc manifestations in Europe. Large differences in patient referral account for a large local variability of SSc presentations and preclude the identification of genetic or environmental factors.</description><identifier>ISSN: 0003-4967</identifier><identifier>EISSN: 1468-2060</identifier><identifier>DOI: 10.1136/ard.2008.091348</identifier><identifier>PMID: 18625615</identifier><identifier>CODEN: ARDIAO</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd and European League Against Rheumatism</publisher><subject>Autoantibodies - blood ; Biological and medical sciences ; Cities ; Clinical Trials as Topic ; Cluster Analysis ; Databases, Factual ; Diseases of the osteoarticular system ; Europe - epidemiology ; Female ; Humans ; Male ; Medical sciences ; Middle Aged ; Patients ; Prevalence ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis ; Scleroderma ; Scleroderma, Systemic - epidemiology ; Scleroderma, Systemic - immunology ; Sex Factors ; Topography, Medical ; Twin studies ; Twins</subject><ispartof>Annals of the rheumatic diseases, 2009-06, Vol.68 (6), p.856-862</ispartof><rights>2009 BMJ Publishing Group and European League Against Rheumatism</rights><rights>2009 INIST-CNRS</rights><rights>Copyright: 2009 2009 BMJ Publishing Group and European League Against Rheumatism</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b426t-78f1aab258833aa111d47a4f46c76cc3ec6f5a53d94d212d0067e84c260cac333</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttp://ard.bmj.com/content/68/6/856.full.pdf$$EPDF$$P50$$Gbmj$$H</linktopdf><linktohtml>$$Uhttp://ard.bmj.com/content/68/6/856.full$$EHTML$$P50$$Gbmj$$H</linktohtml><link.rule.ids>114,115,314,780,784,3196,23571,27924,27925,77600,77631</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=21479719$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18625615$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Walker, U A</creatorcontrib><creatorcontrib>Tyndall, A</creatorcontrib><creatorcontrib>Czirják, L</creatorcontrib><creatorcontrib>Denton, C P</creatorcontrib><creatorcontrib>Farge-Bancel, D</creatorcontrib><creatorcontrib>Kowal-Bielecka, O</creatorcontrib><creatorcontrib>Müller-Ladner, U</creatorcontrib><creatorcontrib>Matucci-Cerinic, M</creatorcontrib><creatorcontrib>EUSTAR co-authors</creatorcontrib><creatorcontrib>and the EUSTAR co-authors</creatorcontrib><title>Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database</title><title>Annals of the rheumatic diseases</title><addtitle>Ann Rheum Dis</addtitle><description>Background:Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether disease presentation varies within Europe.Aims and Methods:The baseline data of all SSc patients entered in the EULAR Scleroderma Trials and Research (EUSTAR) database up to April 2007 were analysed for geographical differences with regard to organ involvement, and geographical clusters with regard to clinical subsets (diffuse vs limited SSc) and autoantibodies (anticentromere vs anti-Scl70).Results:3661 patients from 79 centres in 62 cities and 23 countries were analysed. There was no clear trend between geographical coordinates and SSc subsets, although there appeared to be an increased prevalence of Scl70 in the more eastern centres. There was no association between geographical longitude or latitude and the age at the onset of Raynaud’s phenomenon or the onset of non-Raynaud’s symptoms. There was also a trend for the more eastern centres to care for patients with a higher prevalence of more severe organ manifestations (pulmonary arterial hypertension, cardiac involvement). Between different centres within one city there was a large variability in the frequency of organ complications.Conclusion:This analysis suggests that eastern centres care for more severe SSc manifestations in Europe. Large differences in patient referral account for a large local variability of SSc presentations and preclude the identification of genetic or environmental factors.</description><subject>Autoantibodies - blood</subject><subject>Biological and medical sciences</subject><subject>Cities</subject><subject>Clinical Trials as Topic</subject><subject>Cluster Analysis</subject><subject>Databases, Factual</subject><subject>Diseases of the osteoarticular system</subject><subject>Europe - epidemiology</subject><subject>Female</subject><subject>Humans</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Patients</subject><subject>Prevalence</subject><subject>Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis</subject><subject>Scleroderma</subject><subject>Scleroderma, Systemic - epidemiology</subject><subject>Scleroderma, Systemic - immunology</subject><subject>Sex Factors</subject><subject>Topography, Medical</subject><subject>Twin studies</subject><subject>Twins</subject><issn>0003-4967</issn><issn>1468-2060</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqFkU1vEzEQhlcIREPhzA1ZQiBA2tRfa3u5pVEoiFBEmnC1Jl5v43S_sHcR_SX8XZxuVCQunCxrHr-emSdJnhM8JYSJM_DFlGKspjgnjKsHyYRwoVKKBX6YTDDGLOW5kCfJkxD28YoVUY-TE6IEzQTJJsnvC9tee-h2zkCFfoJ30Lu2QW2JChcsBItqaFxpQ39XCMg1KNyG3tbOoGAq69vgwnsEyNuu9T0qfVujfmfRYrOcrdDVHVJYXwNax_QqIGgKtLIx3JsderPYXK1nq7fo2rdDhwroYRt_fZo8KiNrnx3P02TzYbGef0yXXy8-zWfLdMup6FOpSgKwpZlSjAEQQgougZdcGCmMYdaIMoOMFTkvKKEFxkJaxQ0V2IBhjJ0mr8fczrc_hjilrl0wtqqgse0QtJCUEpHnEXz5D7hvB9_E3jSRUqpMZlxF6mykTFxL8LbUnXc1-FtNsD4Y09GYPhjTo7H44sUxd9jWtvjLHxVF4NURgBAdlR4a48I9RwmXuSSHBtORc1HOr_s6-Js4BJOZvvw-1-cr-u3zF3mpzyP_buS39f6_Xf4BQ427UQ</recordid><startdate>20090601</startdate><enddate>20090601</enddate><creator>Walker, U A</creator><creator>Tyndall, A</creator><creator>Czirják, L</creator><creator>Denton, C P</creator><creator>Farge-Bancel, D</creator><creator>Kowal-Bielecka, O</creator><creator>Müller-Ladner, U</creator><creator>Matucci-Cerinic, M</creator><general>BMJ Publishing Group Ltd and European League Against Rheumatism</general><general>BMJ Publishing Group</general><general>BMJ Publishing Group LTD</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88I</scope><scope>8AF</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9-</scope><scope>K9.</scope><scope>LK8</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>M7P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7X8</scope></search><sort><creationdate>20090601</creationdate><title>Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database</title><author>Walker, U A ; Tyndall, A ; Czirják, L ; Denton, C P ; Farge-Bancel, D ; Kowal-Bielecka, O ; Müller-Ladner, U ; Matucci-Cerinic, M</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b426t-78f1aab258833aa111d47a4f46c76cc3ec6f5a53d94d212d0067e84c260cac333</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Autoantibodies - blood</topic><topic>Biological and medical sciences</topic><topic>Cities</topic><topic>Clinical Trials as Topic</topic><topic>Cluster Analysis</topic><topic>Databases, Factual</topic><topic>Diseases of the osteoarticular system</topic><topic>Europe - epidemiology</topic><topic>Female</topic><topic>Humans</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Patients</topic><topic>Prevalence</topic><topic>Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis</topic><topic>Scleroderma</topic><topic>Scleroderma, Systemic - epidemiology</topic><topic>Scleroderma, Systemic - immunology</topic><topic>Sex Factors</topic><topic>Topography, Medical</topic><topic>Twin studies</topic><topic>Twins</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Walker, U A</creatorcontrib><creatorcontrib>Tyndall, A</creatorcontrib><creatorcontrib>Czirják, L</creatorcontrib><creatorcontrib>Denton, C P</creatorcontrib><creatorcontrib>Farge-Bancel, D</creatorcontrib><creatorcontrib>Kowal-Bielecka, O</creatorcontrib><creatorcontrib>Müller-Ladner, U</creatorcontrib><creatorcontrib>Matucci-Cerinic, M</creatorcontrib><creatorcontrib>EUSTAR co-authors</creatorcontrib><creatorcontrib>and the EUSTAR co-authors</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Science Database (Alumni Edition)</collection><collection>STEM Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>BMJ Journals</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>Consumer Health Database (Alumni Edition)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>ProQuest Biological Science Collection</collection><collection>Consumer Health Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Science Database</collection><collection>Biological Science Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><jtitle>Annals of the rheumatic diseases</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Walker, U A</au><au>Tyndall, A</au><au>Czirják, L</au><au>Denton, C P</au><au>Farge-Bancel, D</au><au>Kowal-Bielecka, O</au><au>Müller-Ladner, U</au><au>Matucci-Cerinic, M</au><aucorp>EUSTAR co-authors</aucorp><aucorp>and the EUSTAR co-authors</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database</atitle><jtitle>Annals of the rheumatic diseases</jtitle><addtitle>Ann Rheum Dis</addtitle><date>2009-06-01</date><risdate>2009</risdate><volume>68</volume><issue>6</issue><spage>856</spage><epage>862</epage><pages>856-862</pages><issn>0003-4967</issn><eissn>1468-2060</eissn><coden>ARDIAO</coden><abstract>Background:Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether disease presentation varies within Europe.Aims and Methods:The baseline data of all SSc patients entered in the EULAR Scleroderma Trials and Research (EUSTAR) database up to April 2007 were analysed for geographical differences with regard to organ involvement, and geographical clusters with regard to clinical subsets (diffuse vs limited SSc) and autoantibodies (anticentromere vs anti-Scl70).Results:3661 patients from 79 centres in 62 cities and 23 countries were analysed. There was no clear trend between geographical coordinates and SSc subsets, although there appeared to be an increased prevalence of Scl70 in the more eastern centres. There was no association between geographical longitude or latitude and the age at the onset of Raynaud’s phenomenon or the onset of non-Raynaud’s symptoms. There was also a trend for the more eastern centres to care for patients with a higher prevalence of more severe organ manifestations (pulmonary arterial hypertension, cardiac involvement). Between different centres within one city there was a large variability in the frequency of organ complications.Conclusion:This analysis suggests that eastern centres care for more severe SSc manifestations in Europe. Large differences in patient referral account for a large local variability of SSc presentations and preclude the identification of genetic or environmental factors.</abstract><cop>London</cop><pub>BMJ Publishing Group Ltd and European League Against Rheumatism</pub><pmid>18625615</pmid><doi>10.1136/ard.2008.091348</doi><tpages>7</tpages></addata></record> |
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subjects | Autoantibodies - blood Biological and medical sciences Cities Clinical Trials as Topic Cluster Analysis Databases, Factual Diseases of the osteoarticular system Europe - epidemiology Female Humans Male Medical sciences Middle Aged Patients Prevalence Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Scleroderma Scleroderma, Systemic - epidemiology Scleroderma, Systemic - immunology Sex Factors Topography, Medical Twin studies Twins |
title | Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database |
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