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Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis

OBJECTIVE: To evaluate the independent contribution of several clinical and laboratory variables to the mortality of a cohort of Danish patients with systemic sclerosis (SSc). METHODS: A cohort of 174 patients with incident SSc was retrospectively identified using clinical charts and study records o...

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Published in:Journal of rheumatology 2001-11, Vol.28 (11), p.2454-2459
Main Authors: Jacobsen, S, Ullman, S, Shen, G Q, Wiik, A, Halberg, P
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Ullman, S
Shen, G Q
Wiik, A
Halberg, P
description OBJECTIVE: To evaluate the independent contribution of several clinical and laboratory variables to the mortality of a cohort of Danish patients with systemic sclerosis (SSc). METHODS: A cohort of 174 patients with incident SSc was retrospectively identified using clinical charts and study records of all new patients with SSc. Disease onset was defined as the time of onset of cutaneous sclerosis. Vital status and causes of death were determined at the end of the observation period. Data on clinical status and pulmonary function were obtained. Antitopoisomerase I (anti-topo I), anticentromere, anti-U1-RNP, anti-U3-RNP, anti-Th-RNP, and anti-RNA polymerase (anti-RNAP) antibodies were determined by means of double immunodiffusion, immunofluorescence, hemagglutination technique, radioactively labelled antisense riboprobes, and ELISA, respectively. RESULTS: Patients were followed for a mean period of 13.3 yrs; 16 died of an SSc related condition and 50 of other causes. Pulmonary fibrosis, DLCO reduction < 40% of the expected, diffuse cutaneous involvement, SSc nephropathy, cardiac disease, and anti-topo I and anti-RNAP antibody were related to decreased survival due to SSc. Variables that entered a Cox regression model of SSc related mortality were right heart failure (RR 12.4, 95% CI 2.5-60), diffuse SSc (RR 7.8, 95% CI 1.8-35), SSc nephropathy (RR 6.1, 95% CI 1.8-21), and DLCO < 40% (RR 4.8, 95% CI 1.1-20). The relative risk of developing right heart failure and diffuse SSc given the presence of anti-RNAP antibody was 14 (p = 0.0001) and 1.9 (p = 0.01), respectively. The corresponding figures for anti-topo I antibody were 4.6 (p = 0.02) and 2.0 (p = 0.01). CONCLUSION: SSc related mortality was associated with right heart failure and diffuse SSc, both of which were also associated with the presence of anti-topo I and anti-RNAP antibody. The prognostic value of these autoantibodies may lie in the early course of the disease when specific morbidity has not yet evolved.
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METHODS: A cohort of 174 patients with incident SSc was retrospectively identified using clinical charts and study records of all new patients with SSc. Disease onset was defined as the time of onset of cutaneous sclerosis. Vital status and causes of death were determined at the end of the observation period. Data on clinical status and pulmonary function were obtained. Antitopoisomerase I (anti-topo I), anticentromere, anti-U1-RNP, anti-U3-RNP, anti-Th-RNP, and anti-RNA polymerase (anti-RNAP) antibodies were determined by means of double immunodiffusion, immunofluorescence, hemagglutination technique, radioactively labelled antisense riboprobes, and ELISA, respectively. RESULTS: Patients were followed for a mean period of 13.3 yrs; 16 died of an SSc related condition and 50 of other causes. Pulmonary fibrosis, DLCO reduction &lt; 40% of the expected, diffuse cutaneous involvement, SSc nephropathy, cardiac disease, and anti-topo I and anti-RNAP antibody were related to decreased survival due to SSc. Variables that entered a Cox regression model of SSc related mortality were right heart failure (RR 12.4, 95% CI 2.5-60), diffuse SSc (RR 7.8, 95% CI 1.8-35), SSc nephropathy (RR 6.1, 95% CI 1.8-21), and DLCO &lt; 40% (RR 4.8, 95% CI 1.1-20). The relative risk of developing right heart failure and diffuse SSc given the presence of anti-RNAP antibody was 14 (p = 0.0001) and 1.9 (p = 0.01), respectively. The corresponding figures for anti-topo I antibody were 4.6 (p = 0.02) and 2.0 (p = 0.01). CONCLUSION: SSc related mortality was associated with right heart failure and diffuse SSc, both of which were also associated with the presence of anti-topo I and anti-RNAP antibody. The prognostic value of these autoantibodies may lie in the early course of the disease when specific morbidity has not yet evolved.</description><identifier>ISSN: 0315-162X</identifier><identifier>EISSN: 1499-2752</identifier><identifier>PMID: 11708418</identifier><identifier>CODEN: JRHUA9</identifier><language>eng</language><publisher>Toronto, ON: The Journal of Rheumatology</publisher><subject>Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antibodies, Antinuclear - blood ; Biological and medical sciences ; Cause of Death ; Child ; Cohort Studies ; Female ; Heart Diseases - etiology ; Heart Diseases - mortality ; Heart Diseases - pathology ; Humans ; Kidney Diseases - etiology ; Kidney Diseases - mortality ; Kidney Diseases - pathology ; Lung - pathology ; Lung - physiopathology ; Male ; Medical sciences ; Middle Aged ; Prognosis ; Prospective Studies ; Pulmonary Fibrosis - etiology ; Pulmonary Fibrosis - mortality ; Pulmonary Fibrosis - pathology ; Respiratory Function Tests ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis ; Scleroderma, Systemic - complications ; Scleroderma, Systemic - mortality ; Scleroderma, Systemic - pathology ; Survival Analysis ; Survival Rate</subject><ispartof>Journal of rheumatology, 2001-11, Vol.28 (11), p.2454-2459</ispartof><rights>2002 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=14113362$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11708418$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Jacobsen, S</creatorcontrib><creatorcontrib>Ullman, S</creatorcontrib><creatorcontrib>Shen, G Q</creatorcontrib><creatorcontrib>Wiik, A</creatorcontrib><creatorcontrib>Halberg, P</creatorcontrib><title>Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis</title><title>Journal of rheumatology</title><addtitle>J Rheumatol</addtitle><description>OBJECTIVE: To evaluate the independent contribution of several clinical and laboratory variables to the mortality of a cohort of Danish patients with systemic sclerosis (SSc). METHODS: A cohort of 174 patients with incident SSc was retrospectively identified using clinical charts and study records of all new patients with SSc. Disease onset was defined as the time of onset of cutaneous sclerosis. Vital status and causes of death were determined at the end of the observation period. Data on clinical status and pulmonary function were obtained. Antitopoisomerase I (anti-topo I), anticentromere, anti-U1-RNP, anti-U3-RNP, anti-Th-RNP, and anti-RNA polymerase (anti-RNAP) antibodies were determined by means of double immunodiffusion, immunofluorescence, hemagglutination technique, radioactively labelled antisense riboprobes, and ELISA, respectively. RESULTS: Patients were followed for a mean period of 13.3 yrs; 16 died of an SSc related condition and 50 of other causes. Pulmonary fibrosis, DLCO reduction &lt; 40% of the expected, diffuse cutaneous involvement, SSc nephropathy, cardiac disease, and anti-topo I and anti-RNAP antibody were related to decreased survival due to SSc. Variables that entered a Cox regression model of SSc related mortality were right heart failure (RR 12.4, 95% CI 2.5-60), diffuse SSc (RR 7.8, 95% CI 1.8-35), SSc nephropathy (RR 6.1, 95% CI 1.8-21), and DLCO &lt; 40% (RR 4.8, 95% CI 1.1-20). The relative risk of developing right heart failure and diffuse SSc given the presence of anti-RNAP antibody was 14 (p = 0.0001) and 1.9 (p = 0.01), respectively. The corresponding figures for anti-topo I antibody were 4.6 (p = 0.02) and 2.0 (p = 0.01). CONCLUSION: SSc related mortality was associated with right heart failure and diffuse SSc, both of which were also associated with the presence of anti-topo I and anti-RNAP antibody. The prognostic value of these autoantibodies may lie in the early course of the disease when specific morbidity has not yet evolved.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Antibodies, Antinuclear - blood</subject><subject>Biological and medical sciences</subject><subject>Cause of Death</subject><subject>Child</subject><subject>Cohort Studies</subject><subject>Female</subject><subject>Heart Diseases - etiology</subject><subject>Heart Diseases - mortality</subject><subject>Heart Diseases - pathology</subject><subject>Humans</subject><subject>Kidney Diseases - etiology</subject><subject>Kidney Diseases - mortality</subject><subject>Kidney Diseases - pathology</subject><subject>Lung - pathology</subject><subject>Lung - physiopathology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Prognosis</subject><subject>Prospective Studies</subject><subject>Pulmonary Fibrosis - etiology</subject><subject>Pulmonary Fibrosis - mortality</subject><subject>Pulmonary Fibrosis - pathology</subject><subject>Respiratory Function Tests</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>Scleroderma, Systemic - pathology</subject><subject>Survival Analysis</subject><subject>Survival Rate</subject><issn>0315-162X</issn><issn>1499-2752</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><recordid>eNpF0EtLxDAQwPEiiq6PryC5qBcLzWPb9CjiCwQvCt5Kmk7cSJqumcTiya9u1BVhIAP58T_MVrGgom1L1izZdrGoOF2WtGbPe8U-4mtV0VrUcrfYo7SppKByUXzeeeMSeA1kMkQ7661WjhhQMQXAc4IQ0kiUj9Yn7UCFn72fBvv9q_xAXPIvxCSvo508yYMpvNv3XMnFtYoWfEQy27gi-IERRqsJ5lSY0OJhsWOUQzjavAfF0_XV4-Vtef9wc3d5cV-uWN3GktNG86FVTNLGNA2AMZQxVZmeail1LTVAbwz0apCccS76ijV0qWpumOip5AfF6W93Haa3BBi70aIG55SHKWHXsOxbLjI83sDUjzB062BHFT66v5NlcLIBCvOpTFBeW_x3glLOa5bd2a9b2ZfVbAN0OCrncpZ38zwzmZMdE0vBvwAKPIZ4</recordid><startdate>20011101</startdate><enddate>20011101</enddate><creator>Jacobsen, S</creator><creator>Ullman, S</creator><creator>Shen, G Q</creator><creator>Wiik, A</creator><creator>Halberg, P</creator><general>The Journal of Rheumatology</general><general>Journal of Rheumatology Publishing</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>20011101</creationdate><title>Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis</title><author>Jacobsen, S ; Ullman, S ; Shen, G Q ; Wiik, A ; Halberg, P</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-h269t-317c3d9a2817f77eeff122a0fb1c88c68ceebffebad832334b02715a63f24b183</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Antibodies, Antinuclear - blood</topic><topic>Biological and medical sciences</topic><topic>Cause of Death</topic><topic>Child</topic><topic>Cohort Studies</topic><topic>Female</topic><topic>Heart Diseases - etiology</topic><topic>Heart Diseases - mortality</topic><topic>Heart Diseases - pathology</topic><topic>Humans</topic><topic>Kidney Diseases - etiology</topic><topic>Kidney Diseases - mortality</topic><topic>Kidney Diseases - pathology</topic><topic>Lung - pathology</topic><topic>Lung - physiopathology</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Prognosis</topic><topic>Prospective Studies</topic><topic>Pulmonary Fibrosis - etiology</topic><topic>Pulmonary Fibrosis - mortality</topic><topic>Pulmonary Fibrosis - pathology</topic><topic>Respiratory Function Tests</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>Scleroderma, Systemic - pathology</topic><topic>Survival Analysis</topic><topic>Survival Rate</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Jacobsen, S</creatorcontrib><creatorcontrib>Ullman, S</creatorcontrib><creatorcontrib>Shen, G Q</creatorcontrib><creatorcontrib>Wiik, A</creatorcontrib><creatorcontrib>Halberg, P</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of rheumatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Jacobsen, S</au><au>Ullman, S</au><au>Shen, G Q</au><au>Wiik, A</au><au>Halberg, P</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis</atitle><jtitle>Journal of rheumatology</jtitle><addtitle>J Rheumatol</addtitle><date>2001-11-01</date><risdate>2001</risdate><volume>28</volume><issue>11</issue><spage>2454</spage><epage>2459</epage><pages>2454-2459</pages><issn>0315-162X</issn><eissn>1499-2752</eissn><coden>JRHUA9</coden><abstract>OBJECTIVE: To evaluate the independent contribution of several clinical and laboratory variables to the mortality of a cohort of Danish patients with systemic sclerosis (SSc). METHODS: A cohort of 174 patients with incident SSc was retrospectively identified using clinical charts and study records of all new patients with SSc. Disease onset was defined as the time of onset of cutaneous sclerosis. Vital status and causes of death were determined at the end of the observation period. Data on clinical status and pulmonary function were obtained. Antitopoisomerase I (anti-topo I), anticentromere, anti-U1-RNP, anti-U3-RNP, anti-Th-RNP, and anti-RNA polymerase (anti-RNAP) antibodies were determined by means of double immunodiffusion, immunofluorescence, hemagglutination technique, radioactively labelled antisense riboprobes, and ELISA, respectively. RESULTS: Patients were followed for a mean period of 13.3 yrs; 16 died of an SSc related condition and 50 of other causes. Pulmonary fibrosis, DLCO reduction &lt; 40% of the expected, diffuse cutaneous involvement, SSc nephropathy, cardiac disease, and anti-topo I and anti-RNAP antibody were related to decreased survival due to SSc. Variables that entered a Cox regression model of SSc related mortality were right heart failure (RR 12.4, 95% CI 2.5-60), diffuse SSc (RR 7.8, 95% CI 1.8-35), SSc nephropathy (RR 6.1, 95% CI 1.8-21), and DLCO &lt; 40% (RR 4.8, 95% CI 1.1-20). The relative risk of developing right heart failure and diffuse SSc given the presence of anti-RNAP antibody was 14 (p = 0.0001) and 1.9 (p = 0.01), respectively. The corresponding figures for anti-topo I antibody were 4.6 (p = 0.02) and 2.0 (p = 0.01). CONCLUSION: SSc related mortality was associated with right heart failure and diffuse SSc, both of which were also associated with the presence of anti-topo I and anti-RNAP antibody. The prognostic value of these autoantibodies may lie in the early course of the disease when specific morbidity has not yet evolved.</abstract><cop>Toronto, ON</cop><pub>The Journal of Rheumatology</pub><pmid>11708418</pmid><tpages>6</tpages></addata></record>
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source Medical Journals
subjects Adolescent
Adult
Aged
Aged, 80 and over
Antibodies, Antinuclear - blood
Biological and medical sciences
Cause of Death
Child
Cohort Studies
Female
Heart Diseases - etiology
Heart Diseases - mortality
Heart Diseases - pathology
Humans
Kidney Diseases - etiology
Kidney Diseases - mortality
Kidney Diseases - pathology
Lung - pathology
Lung - physiopathology
Male
Medical sciences
Middle Aged
Prognosis
Prospective Studies
Pulmonary Fibrosis - etiology
Pulmonary Fibrosis - mortality
Pulmonary Fibrosis - pathology
Respiratory Function Tests
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Scleroderma, Systemic - complications
Scleroderma, Systemic - mortality
Scleroderma, Systemic - pathology
Survival Analysis
Survival Rate
title Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis
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