Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study
Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. Methods. At 3-year follow-up, vital status was obtained from i...
Gespeichert in:
| Veröffentlicht in: | Rheumatology 2009-03, Vol.48 (3), p.304-308 |
|---|---|
| Hauptverfasser: | , , , , , , , , , , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 308 |
|---|---|
| container_issue | 3 |
| container_start_page | 304 |
| container_title | Rheumatology |
| container_volume | 48 |
| creator | Hachulla, Eric Carpentier, Patrick Gressin, Virginie Diot, Elisabeth Allanore, Yannick Sibilia, Jean Launay, David Mouthon, Luc Jego, Patrick Cabane, Jean de Groote, Pascal Chabrol, Amélie Lazareth, Isabelle Guillevin, Loïc Clerson, Pierre Humbert, Marc |
| description | Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. Methods. At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan–Meier method. Multivariate survival analyses were conducted using the Cox model. Results. In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 ± 13.0 years and mean duration of SSc of 8.8 ± 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality. Conclusion. This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening. |
| doi_str_mv | 10.1093/rheumatology/ken488 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_2644045</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><oup_id>10.1093/rheumatology/ken488</oup_id><sourcerecordid>66939855</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4898-43cac4ee3416c6da77bda01ddc4b9af75788c7877496ac832ae29917b63542503</originalsourceid><addsrcrecordid>eNqNkt9u0zAUxiMEYmPwBEjIQoIrstnxv5gLpGqitKgaYhQJ7cZyHWfxmsTFTgq54IF4Dl4Ml1Rl7IorH8m_7_M5x1-SPEXwFEGBz3xl-kZ1rnbXw9natCTP7yXHiLAshRhn9w91Ro6SRyHcQAgpwvnD5AgJxAnl6Dj5cWnDGpRKd84HUDoPCqO6Cqi2AF1lAE4HozwIvd_araqBK8FGdda0XQDfbATDEDrTWA2Cro13wYbXf4RTb1pdgXln218__WR-mX7SdaxcYXxjDQhdXwyPkwelqoN5sj9Pks_Tt8vzWbr48G5-PlmkmuQiTwnWShNjMEFMs0JxvioUREWhyUqoklOe55rnnBPBlM5xpkwm4ogrhinJKMQnyZvRd9OvGlPo2L5Xtdx42yg_SKes_PemtZW8dluZMUIgodHg1WhQ3ZHNJgtp2xBnkhCSLKNEbFHEX-7f8-5rb0InGxu0qWvVGtcHyZjAIqc73-d3wBvX-zbuQiJBGaMC5hHCI6TjfoM35aEDBOUuCvJ2FOQYhah6dnvmv5r930fgxR5QQau69KrVNhy4DCHGmdgt73TkXL_5z5fTUWBjNL4fJMqvJeOYUzn7ciWXF8uPF--vqJzi36-L5Ek</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>195665908</pqid></control><display><type>article</type><title>Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study</title><source>Oxford University Press Journals All Titles (1996-Current)</source><source>MEDLINE</source><source>Alma/SFX Local Collection</source><creator>Hachulla, Eric ; Carpentier, Patrick ; Gressin, Virginie ; Diot, Elisabeth ; Allanore, Yannick ; Sibilia, Jean ; Launay, David ; Mouthon, Luc ; Jego, Patrick ; Cabane, Jean ; de Groote, Pascal ; Chabrol, Amélie ; Lazareth, Isabelle ; Guillevin, Loïc ; Clerson, Pierre ; Humbert, Marc</creator><creatorcontrib>Hachulla, Eric ; Carpentier, Patrick ; Gressin, Virginie ; Diot, Elisabeth ; Allanore, Yannick ; Sibilia, Jean ; Launay, David ; Mouthon, Luc ; Jego, Patrick ; Cabane, Jean ; de Groote, Pascal ; Chabrol, Amélie ; Lazareth, Isabelle ; Guillevin, Loïc ; Clerson, Pierre ; Humbert, Marc ; ItinérAIR-Sclérodermie Study Investigators ; the ItinerAIR-Sclerodermie Study Investigators</creatorcontrib><description>Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. Methods. At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan–Meier method. Multivariate survival analyses were conducted using the Cox model. Results. In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 ± 13.0 years and mean duration of SSc of 8.8 ± 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality. Conclusion. This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening.</description><identifier>ISSN: 1462-0324</identifier><identifier>EISSN: 1462-0332</identifier><identifier>EISSN: 1460-2172</identifier><identifier>DOI: 10.1093/rheumatology/ken488</identifier><identifier>PMID: 19174571</identifier><identifier>CODEN: BJRHDF</identifier><language>eng</language><publisher>Oxford: Oxford University Press</publisher><subject>Adult ; Age of Onset ; Aged ; Biochemistry, Molecular Biology ; Biological and medical sciences ; Clinical ; Diseases of the osteoarticular system ; Epidemiologic Methods ; Female ; France - epidemiology ; Humans ; Hypertension, Pulmonary - etiology ; Hypertension, Pulmonary - mortality ; Life Sciences ; Male ; Medical sciences ; Middle Aged ; Pneumology ; Prognosis ; Pulmonary arterial hypertension ; Pulmonary hypertension ; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis ; Scleroderma, Systemic - complications ; Scleroderma, Systemic - mortality ; Survival ; Systemic sclerosis ; Systemic sclerosis, Survival, Pulmonary arterial hypertension, Pulmonary hypertension ; Time Factors</subject><ispartof>Rheumatology, 2009-03, Vol.48 (3), p.304-308</ispartof><rights>2009 The Author(s) 2009</rights><rights>2009 INIST-CNRS</rights><rights>2009 The Author(s)</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4898-43cac4ee3416c6da77bda01ddc4b9af75788c7877496ac832ae29917b63542503</citedby><cites>FETCH-LOGICAL-c4898-43cac4ee3416c6da77bda01ddc4b9af75788c7877496ac832ae29917b63542503</cites><orcidid>0000-0001-6463-2160 ; 0000-0001-7432-847X ; 0000-0003-1840-1817 ; 0000-0003-0703-2892 ; 0000-0002-2152-6461 ; 0000-0002-9838-246X</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,776,780,881,1578,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=21167690$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19174571$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://inserm.hal.science/inserm-00422549$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Hachulla, Eric</creatorcontrib><creatorcontrib>Carpentier, Patrick</creatorcontrib><creatorcontrib>Gressin, Virginie</creatorcontrib><creatorcontrib>Diot, Elisabeth</creatorcontrib><creatorcontrib>Allanore, Yannick</creatorcontrib><creatorcontrib>Sibilia, Jean</creatorcontrib><creatorcontrib>Launay, David</creatorcontrib><creatorcontrib>Mouthon, Luc</creatorcontrib><creatorcontrib>Jego, Patrick</creatorcontrib><creatorcontrib>Cabane, Jean</creatorcontrib><creatorcontrib>de Groote, Pascal</creatorcontrib><creatorcontrib>Chabrol, Amélie</creatorcontrib><creatorcontrib>Lazareth, Isabelle</creatorcontrib><creatorcontrib>Guillevin, Loïc</creatorcontrib><creatorcontrib>Clerson, Pierre</creatorcontrib><creatorcontrib>Humbert, Marc</creatorcontrib><creatorcontrib>ItinérAIR-Sclérodermie Study Investigators</creatorcontrib><creatorcontrib>the ItinerAIR-Sclerodermie Study Investigators</creatorcontrib><title>Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study</title><title>Rheumatology</title><addtitle>Rheumatology (Oxford)</addtitle><description>Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. Methods. At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan–Meier method. Multivariate survival analyses were conducted using the Cox model. Results. In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 ± 13.0 years and mean duration of SSc of 8.8 ± 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality. Conclusion. This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening.</description><subject>Adult</subject><subject>Age of Onset</subject><subject>Aged</subject><subject>Biochemistry, Molecular Biology</subject><subject>Biological and medical sciences</subject><subject>Clinical</subject><subject>Diseases of the osteoarticular system</subject><subject>Epidemiologic Methods</subject><subject>Female</subject><subject>France - epidemiology</subject><subject>Humans</subject><subject>Hypertension, Pulmonary - etiology</subject><subject>Hypertension, Pulmonary - mortality</subject><subject>Life Sciences</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Pneumology</subject><subject>Prognosis</subject><subject>Pulmonary arterial hypertension</subject><subject>Pulmonary hypertension</subject><subject>Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases</subject><subject>Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis</subject><subject>Scleroderma, Systemic - complications</subject><subject>Scleroderma, Systemic - mortality</subject><subject>Survival</subject><subject>Systemic sclerosis</subject><subject>Systemic sclerosis, Survival, Pulmonary arterial hypertension, Pulmonary hypertension</subject><subject>Time Factors</subject><issn>1462-0324</issn><issn>1462-0332</issn><issn>1460-2172</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><sourceid>TOX</sourceid><sourceid>EIF</sourceid><recordid>eNqNkt9u0zAUxiMEYmPwBEjIQoIrstnxv5gLpGqitKgaYhQJ7cZyHWfxmsTFTgq54IF4Dl4Ml1Rl7IorH8m_7_M5x1-SPEXwFEGBz3xl-kZ1rnbXw9natCTP7yXHiLAshRhn9w91Ro6SRyHcQAgpwvnD5AgJxAnl6Dj5cWnDGpRKd84HUDoPCqO6Cqi2AF1lAE4HozwIvd_araqBK8FGdda0XQDfbATDEDrTWA2Cro13wYbXf4RTb1pdgXln218__WR-mX7SdaxcYXxjDQhdXwyPkwelqoN5sj9Pks_Tt8vzWbr48G5-PlmkmuQiTwnWShNjMEFMs0JxvioUREWhyUqoklOe55rnnBPBlM5xpkwm4ogrhinJKMQnyZvRd9OvGlPo2L5Xtdx42yg_SKes_PemtZW8dluZMUIgodHg1WhQ3ZHNJgtp2xBnkhCSLKNEbFHEX-7f8-5rb0InGxu0qWvVGtcHyZjAIqc73-d3wBvX-zbuQiJBGaMC5hHCI6TjfoM35aEDBOUuCvJ2FOQYhah6dnvmv5r930fgxR5QQau69KrVNhy4DCHGmdgt73TkXL_5z5fTUWBjNL4fJMqvJeOYUzn7ciWXF8uPF--vqJzi36-L5Ek</recordid><startdate>200903</startdate><enddate>200903</enddate><creator>Hachulla, Eric</creator><creator>Carpentier, Patrick</creator><creator>Gressin, Virginie</creator><creator>Diot, Elisabeth</creator><creator>Allanore, Yannick</creator><creator>Sibilia, Jean</creator><creator>Launay, David</creator><creator>Mouthon, Luc</creator><creator>Jego, Patrick</creator><creator>Cabane, Jean</creator><creator>de Groote, Pascal</creator><creator>Chabrol, Amélie</creator><creator>Lazareth, Isabelle</creator><creator>Guillevin, Loïc</creator><creator>Clerson, Pierre</creator><creator>Humbert, Marc</creator><general>Oxford University Press</general><general>Oxford Publishing Limited (England)</general><general>Oxford University Press (OUP)</general><scope>BSCLL</scope><scope>TOX</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>7QP</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope><scope>1XC</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-6463-2160</orcidid><orcidid>https://orcid.org/0000-0001-7432-847X</orcidid><orcidid>https://orcid.org/0000-0003-1840-1817</orcidid><orcidid>https://orcid.org/0000-0003-0703-2892</orcidid><orcidid>https://orcid.org/0000-0002-2152-6461</orcidid><orcidid>https://orcid.org/0000-0002-9838-246X</orcidid></search><sort><creationdate>200903</creationdate><title>Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study</title><author>Hachulla, Eric ; Carpentier, Patrick ; Gressin, Virginie ; Diot, Elisabeth ; Allanore, Yannick ; Sibilia, Jean ; Launay, David ; Mouthon, Luc ; Jego, Patrick ; Cabane, Jean ; de Groote, Pascal ; Chabrol, Amélie ; Lazareth, Isabelle ; Guillevin, Loïc ; Clerson, Pierre ; Humbert, Marc</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4898-43cac4ee3416c6da77bda01ddc4b9af75788c7877496ac832ae29917b63542503</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Adult</topic><topic>Age of Onset</topic><topic>Aged</topic><topic>Biochemistry, Molecular Biology</topic><topic>Biological and medical sciences</topic><topic>Clinical</topic><topic>Diseases of the osteoarticular system</topic><topic>Epidemiologic Methods</topic><topic>Female</topic><topic>France - epidemiology</topic><topic>Humans</topic><topic>Hypertension, Pulmonary - etiology</topic><topic>Hypertension, Pulmonary - mortality</topic><topic>Life Sciences</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Pneumology</topic><topic>Prognosis</topic><topic>Pulmonary arterial hypertension</topic><topic>Pulmonary hypertension</topic><topic>Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases</topic><topic>Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis</topic><topic>Scleroderma, Systemic - complications</topic><topic>Scleroderma, Systemic - mortality</topic><topic>Survival</topic><topic>Systemic sclerosis</topic><topic>Systemic sclerosis, Survival, Pulmonary arterial hypertension, Pulmonary hypertension</topic><topic>Time Factors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hachulla, Eric</creatorcontrib><creatorcontrib>Carpentier, Patrick</creatorcontrib><creatorcontrib>Gressin, Virginie</creatorcontrib><creatorcontrib>Diot, Elisabeth</creatorcontrib><creatorcontrib>Allanore, Yannick</creatorcontrib><creatorcontrib>Sibilia, Jean</creatorcontrib><creatorcontrib>Launay, David</creatorcontrib><creatorcontrib>Mouthon, Luc</creatorcontrib><creatorcontrib>Jego, Patrick</creatorcontrib><creatorcontrib>Cabane, Jean</creatorcontrib><creatorcontrib>de Groote, Pascal</creatorcontrib><creatorcontrib>Chabrol, Amélie</creatorcontrib><creatorcontrib>Lazareth, Isabelle</creatorcontrib><creatorcontrib>Guillevin, Loïc</creatorcontrib><creatorcontrib>Clerson, Pierre</creatorcontrib><creatorcontrib>Humbert, Marc</creatorcontrib><creatorcontrib>ItinérAIR-Sclérodermie Study Investigators</creatorcontrib><creatorcontrib>the ItinerAIR-Sclerodermie Study Investigators</creatorcontrib><collection>Istex</collection><collection>Oxford Journals Open Access Collection</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>Calcium & Calcified Tissue Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Rheumatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hachulla, Eric</au><au>Carpentier, Patrick</au><au>Gressin, Virginie</au><au>Diot, Elisabeth</au><au>Allanore, Yannick</au><au>Sibilia, Jean</au><au>Launay, David</au><au>Mouthon, Luc</au><au>Jego, Patrick</au><au>Cabane, Jean</au><au>de Groote, Pascal</au><au>Chabrol, Amélie</au><au>Lazareth, Isabelle</au><au>Guillevin, Loïc</au><au>Clerson, Pierre</au><au>Humbert, Marc</au><aucorp>ItinérAIR-Sclérodermie Study Investigators</aucorp><aucorp>the ItinerAIR-Sclerodermie Study Investigators</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study</atitle><jtitle>Rheumatology</jtitle><addtitle>Rheumatology (Oxford)</addtitle><date>2009-03</date><risdate>2009</risdate><volume>48</volume><issue>3</issue><spage>304</spage><epage>308</epage><pages>304-308</pages><issn>1462-0324</issn><eissn>1462-0332</eissn><eissn>1460-2172</eissn><coden>BJRHDF</coden><abstract>Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. Methods. At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan–Meier method. Multivariate survival analyses were conducted using the Cox model. Results. In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 ± 13.0 years and mean duration of SSc of 8.8 ± 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality. Conclusion. This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening.</abstract><cop>Oxford</cop><pub>Oxford University Press</pub><pmid>19174571</pmid><doi>10.1093/rheumatology/ken488</doi><tpages>5</tpages><orcidid>https://orcid.org/0000-0001-6463-2160</orcidid><orcidid>https://orcid.org/0000-0001-7432-847X</orcidid><orcidid>https://orcid.org/0000-0003-1840-1817</orcidid><orcidid>https://orcid.org/0000-0003-0703-2892</orcidid><orcidid>https://orcid.org/0000-0002-2152-6461</orcidid><orcidid>https://orcid.org/0000-0002-9838-246X</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1462-0324 |
| ispartof | Rheumatology, 2009-03, Vol.48 (3), p.304-308 |
| issn | 1462-0324 1462-0332 1460-2172 |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_2644045 |
| source | Oxford University Press Journals All Titles (1996-Current); MEDLINE; Alma/SFX Local Collection |
| subjects | Adult Age of Onset Aged Biochemistry, Molecular Biology Biological and medical sciences Clinical Diseases of the osteoarticular system Epidemiologic Methods Female France - epidemiology Humans Hypertension, Pulmonary - etiology Hypertension, Pulmonary - mortality Life Sciences Male Medical sciences Middle Aged Pneumology Prognosis Pulmonary arterial hypertension Pulmonary hypertension Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Scleroderma, Systemic - complications Scleroderma, Systemic - mortality Survival Systemic sclerosis Systemic sclerosis, Survival, Pulmonary arterial hypertension, Pulmonary hypertension Time Factors |
| title | Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-04T00%3A45%3A59IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Risk%20factors%20for%20death%20and%20the%203-year%20survival%20of%20patients%20with%20systemic%20sclerosis:%20the%20French%20Itin%C3%A9rAIR-Scl%C3%A9rodermie%20study&rft.jtitle=Rheumatology&rft.au=Hachulla,%20Eric&rft.aucorp=Itin%C3%A9rAIR-Scl%C3%A9rodermie%20Study%20Investigators&rft.date=2009-03&rft.volume=48&rft.issue=3&rft.spage=304&rft.epage=308&rft.pages=304-308&rft.issn=1462-0324&rft.eissn=1462-0332&rft.coden=BJRHDF&rft_id=info:doi/10.1093/rheumatology/ken488&rft_dat=%3Cproquest_pubme%3E66939855%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=195665908&rft_id=info:pmid/19174571&rft_oup_id=10.1093/rheumatology/ken488&rfr_iscdi=true |