Sjögren Syndrome or Sjögren Disease? The Histological and Immunological Bias Caused by the 2002 Criteria
The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim...
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| Veröffentlicht in: | Clinical reviews in allergy & immunology 2010-04, Vol.38 (2-3), p.178-185 |
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| creator | Ramos-Casals, Manuel Brito-Zerón, Pilar Perez-De-Lis, Marta Jimenez, Iratxe Blanco, Maria-Jose Bove, Albert Soto, Maria-Jose Akasbi, Miriam Diaz, Candido Sentís, Juan Siso, Antoni |
| description | The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p |
| doi_str_mv | 10.1007/s12016-009-8152-z |
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The Histological and Immunological Bias Caused by the 2002 Criteria</title><source>MEDLINE</source><source>SpringerLink Journals - AutoHoldings</source><creator>Ramos-Casals, Manuel ; Brito-Zerón, Pilar ; Perez-De-Lis, Marta ; Jimenez, Iratxe ; Blanco, Maria-Jose ; Bove, Albert ; Soto, Maria-Jose ; Akasbi, Miriam ; Diaz, Candido ; Sentís, Juan ; Siso, Antoni</creator><creatorcontrib>Ramos-Casals, Manuel ; Brito-Zerón, Pilar ; Perez-De-Lis, Marta ; Jimenez, Iratxe ; Blanco, Maria-Jose ; Bove, Albert ; Soto, Maria-Jose ; Akasbi, Miriam ; Diaz, Candido ; Sentís, Juan ; Siso, Antoni</creatorcontrib><description>The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p < 0.001) and had a lower frequency of parotid enlargement (p = 0.002), fever (p = 0.041), arthritis (p = 0.041), vasculitis (p = 0.050), peripheral neuropathy (p = 0.002), cranial nerve involvement (p = 0.015), raised erythrocyte sedimentation rate ( ESR) levels (p < 0.001), anemia (p < 0.001), leukopenia (p = 0.037), hypergammaglobulinemia (p < 0.001), positive rheumatoid factor ( RF; p = 0.002), and cryoglobulinemia (p = 0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies , complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren “disease.” In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren “syndrome” seems more adequate.</description><identifier>ISSN: 1080-0549</identifier><identifier>EISSN: 1559-0267</identifier><identifier>DOI: 10.1007/s12016-009-8152-z</identifier><identifier>PMID: 19578996</identifier><language>eng</language><publisher>New York: New York : Humana Press Inc</publisher><subject>Allergology ; Anemia ; Antibodies, Antinuclear - immunology ; Antinuclear antibodies ; Arthritis ; Autoantibodies ; Autoantibodies - immunology ; Biopsy ; Cell survival ; Classification ; Cranial nerves ; Cryoglobulinemia ; double prime B-cell lymphoma ; Erythrocytes ; Female ; Fever ; Humans ; Hypergammaglobulinemia ; Immunology ; Internal Medicine ; Leukopenia ; Male ; Medicine ; Medicine & Public Health ; Middle Aged ; Peripheral neuropathy ; Practice Guidelines as Topic ; Reviews ; Rheumatoid factor ; Sedimentation ; Sjogren's syndrome ; Sjogren's Syndrome - classification ; Sjogren's Syndrome - diagnosis ; Sjogren's Syndrome - immunology ; Vasculitis</subject><ispartof>Clinical reviews in allergy & immunology, 2010-04, Vol.38 (2-3), p.178-185</ispartof><rights>Humana Press Inc. 2009</rights><rights>Springer Science+Business Media, LLC 2010</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c426t-195e5fdc3cf355080564e109457c27e7f908a5d6944c42f3aea075306ab456103</citedby><cites>FETCH-LOGICAL-c426t-195e5fdc3cf355080564e109457c27e7f908a5d6944c42f3aea075306ab456103</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s12016-009-8152-z$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s12016-009-8152-z$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19578996$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ramos-Casals, Manuel</creatorcontrib><creatorcontrib>Brito-Zerón, Pilar</creatorcontrib><creatorcontrib>Perez-De-Lis, Marta</creatorcontrib><creatorcontrib>Jimenez, Iratxe</creatorcontrib><creatorcontrib>Blanco, Maria-Jose</creatorcontrib><creatorcontrib>Bove, Albert</creatorcontrib><creatorcontrib>Soto, Maria-Jose</creatorcontrib><creatorcontrib>Akasbi, Miriam</creatorcontrib><creatorcontrib>Diaz, Candido</creatorcontrib><creatorcontrib>Sentís, Juan</creatorcontrib><creatorcontrib>Siso, Antoni</creatorcontrib><title>Sjögren Syndrome or Sjögren Disease? The Histological and Immunological Bias Caused by the 2002 Criteria</title><title>Clinical reviews in allergy & immunology</title><addtitle>Clinic Rev Allerg Immunol</addtitle><addtitle>Clin Rev Allergy Immunol</addtitle><description>The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p < 0.001) and had a lower frequency of parotid enlargement (p = 0.002), fever (p = 0.041), arthritis (p = 0.041), vasculitis (p = 0.050), peripheral neuropathy (p = 0.002), cranial nerve involvement (p = 0.015), raised erythrocyte sedimentation rate ( ESR) levels (p < 0.001), anemia (p < 0.001), leukopenia (p = 0.037), hypergammaglobulinemia (p < 0.001), positive rheumatoid factor ( RF; p = 0.002), and cryoglobulinemia (p = 0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies , complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren “disease.” In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren “syndrome” seems more adequate.</description><subject>Allergology</subject><subject>Anemia</subject><subject>Antibodies, Antinuclear - immunology</subject><subject>Antinuclear antibodies</subject><subject>Arthritis</subject><subject>Autoantibodies</subject><subject>Autoantibodies - immunology</subject><subject>Biopsy</subject><subject>Cell survival</subject><subject>Classification</subject><subject>Cranial nerves</subject><subject>Cryoglobulinemia</subject><subject>double prime B-cell lymphoma</subject><subject>Erythrocytes</subject><subject>Female</subject><subject>Fever</subject><subject>Humans</subject><subject>Hypergammaglobulinemia</subject><subject>Immunology</subject><subject>Internal Medicine</subject><subject>Leukopenia</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Middle Aged</subject><subject>Peripheral neuropathy</subject><subject>Practice Guidelines as Topic</subject><subject>Reviews</subject><subject>Rheumatoid factor</subject><subject>Sedimentation</subject><subject>Sjogren's syndrome</subject><subject>Sjogren's Syndrome - classification</subject><subject>Sjogren's Syndrome - diagnosis</subject><subject>Sjogren's Syndrome - immunology</subject><subject>Vasculitis</subject><issn>1080-0549</issn><issn>1559-0267</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNqFkctu1DAUhi0Eohd4ADZgsekqcGzHtxWC4dJKlVhMu7Y8ycngURIXO1lMH4wX4MXwKKNWYgELy9bx9__nHP2EvGLwjgHo95lxYKoCsJVhklf3T8gpk9JWwJV-Wt5goAJZ2xNylvMOgIMR9jk5YVZqY606Jbv17vevbcKRrvdjm-KANCb6UPwcMvqMH-jND6SXIU-xj9vQ-J76saVXwzCPD5VPwWe68nPGlm72dCoKXlrSVQoTpuBfkGed7zO-PN7n5Pbrl5vVZXX9_dvV6uN11dRcTVWZDWXXNqLphJRlA6lqZGBrqRuuUXcWjJetsnVdBJ3w6EFLAcpvaqkYiHNysfjepfhzxjy5IeQG-96PGOfsrKyVEcza_5JaCCF1aVzIt3-RuzinsazhLDBjmGEHiC1Qk2LOCTt3l8Lg094xcIfA3BKYK4G5Q2DuvmheH43nzYDto-KYUAH4AuTyNW4xPXb-l-ubRdT56Pw2hexu1wUSZVYueDl_AAtbqQ8</recordid><startdate>20100401</startdate><enddate>20100401</enddate><creator>Ramos-Casals, Manuel</creator><creator>Brito-Zerón, Pilar</creator><creator>Perez-De-Lis, Marta</creator><creator>Jimenez, Iratxe</creator><creator>Blanco, Maria-Jose</creator><creator>Bove, Albert</creator><creator>Soto, Maria-Jose</creator><creator>Akasbi, Miriam</creator><creator>Diaz, Candido</creator><creator>Sentís, Juan</creator><creator>Siso, Antoni</creator><general>New York : Humana Press Inc</general><general>Humana Press Inc</general><general>Springer Nature B.V</general><scope>FBQ</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>7T5</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</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>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20100401</creationdate><title>Sjögren Syndrome or Sjögren Disease? 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The Histological and Immunological Bias Caused by the 2002 Criteria</atitle><jtitle>Clinical reviews in allergy & immunology</jtitle><stitle>Clinic Rev Allerg Immunol</stitle><addtitle>Clin Rev Allergy Immunol</addtitle><date>2010-04-01</date><risdate>2010</risdate><volume>38</volume><issue>2-3</issue><spage>178</spage><epage>185</epage><pages>178-185</pages><issn>1080-0549</issn><eissn>1559-0267</eissn><abstract>The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p < 0.001) and had a lower frequency of parotid enlargement (p = 0.002), fever (p = 0.041), arthritis (p = 0.041), vasculitis (p = 0.050), peripheral neuropathy (p = 0.002), cranial nerve involvement (p = 0.015), raised erythrocyte sedimentation rate ( ESR) levels (p < 0.001), anemia (p < 0.001), leukopenia (p = 0.037), hypergammaglobulinemia (p < 0.001), positive rheumatoid factor ( RF; p = 0.002), and cryoglobulinemia (p = 0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies , complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren “disease.” In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren “syndrome” seems more adequate.</abstract><cop>New York</cop><pub>New York : Humana Press Inc</pub><pmid>19578996</pmid><doi>10.1007/s12016-009-8152-z</doi><tpages>8</tpages></addata></record> |
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| subjects | Allergology Anemia Antibodies, Antinuclear - immunology Antinuclear antibodies Arthritis Autoantibodies Autoantibodies - immunology Biopsy Cell survival Classification Cranial nerves Cryoglobulinemia double prime B-cell lymphoma Erythrocytes Female Fever Humans Hypergammaglobulinemia Immunology Internal Medicine Leukopenia Male Medicine Medicine & Public Health Middle Aged Peripheral neuropathy Practice Guidelines as Topic Reviews Rheumatoid factor Sedimentation Sjogren's syndrome Sjogren's Syndrome - classification Sjogren's Syndrome - diagnosis Sjogren's Syndrome - immunology Vasculitis |
| title | Sjögren Syndrome or Sjögren Disease? The Histological and Immunological Bias Caused by the 2002 Criteria |
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