THU0509 First 18 Month Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR)

ObjectivesThe term Auto-inflammatory diseases (AIDs) has been used to describe a group of illness characterized by attacks of seemingly unprovoked inflammation without a significant level of either autoantibodies or antigen-specific T cells more characteristic of autoimmune disease. Published data f...

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Veröffentlicht in:Annals of the rheumatic diseases 2015-06, Vol.74 (Suppl 2), p.384-384
Hauptverfasser: Mehregan, F., Ziaee, V., Moradinejad, M.H., Aghighi, Y., Raeeskarimi, S.R.
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container_end_page 384
container_issue Suppl 2
container_start_page 384
container_title Annals of the rheumatic diseases
container_volume 74
creator Mehregan, F.
Ziaee, V.
Moradinejad, M.H.
Aghighi, Y.
Raeeskarimi, S.R.
description ObjectivesThe term Auto-inflammatory diseases (AIDs) has been used to describe a group of illness characterized by attacks of seemingly unprovoked inflammation without a significant level of either autoantibodies or antigen-specific T cells more characteristic of autoimmune disease. Published data from Iran regarding autoinflammatory disease in children are scarce. In a multicenter effort, we set out to register genetic, epidemiological and clinical features as well as prognostic factors of these diseases by prospective longitudinal and long-term documentation, in order to define current and novel AIDs and to better understand treatment responses and outcome.MethodsThis study is a prospective cross sectional study of patients diagnosed with Auto-inflammatory diseases (AIDs) who was registered in the autoinflammatory computer database registration through periodic fever clinic, which was established for this reason in the medical center of children in 23.09.2012 in Tehran- Iran and data were collected with standardized forms. Inclusion criteria are disease-associated mutations for hereditary periodic fever syndromes [FMF, hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS), TNF receptor 1-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)], or, alternatively, clinically confirmed AID, and periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome with the unknown genetic background.ResultsDuring the first 18 months, 139 patients (93 males, 46 females) from 27 provinces and 11 ethnicities; age (newborn to 18 years) have been recorded and classified as FMF (n=102), PFAPA (n=29), HIDS (n=1), clinically confirmed AID (n=6) including TRAPS (n=1) and CAPS (n=2); CRMO (n=2), DIRA (n=1), and sweet syndrome (n=1). The most common AID in our study were FMF and then PFAPA, CRMO, CAPS. The most common ethnicity was Turkish and Kurdish people. In total 139 patients, genetic analysis was performed in 103 patients, 88.37% have genetically confirmed disease.ConclusionsRecruitment and follow-up of patients with AID will enable us to comprehensively address the correlation between clinical and epidemiological data, genetics.ReferencesINFEVERS: the Registry for FMF and hereditary inflammatory disorders mutations. Nucleic Acids Res, vol 31A retrospective review of autoinflammatory diseases in Saudi children at a rheumatology clinic. Ann Saudi Med 2012 Jan-Feb;32(1):43-8.Disclosure of InterestNone declared
doi_str_mv 10.1136/annrheumdis-2015-eular.6250
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Published data from Iran regarding autoinflammatory disease in children are scarce. In a multicenter effort, we set out to register genetic, epidemiological and clinical features as well as prognostic factors of these diseases by prospective longitudinal and long-term documentation, in order to define current and novel AIDs and to better understand treatment responses and outcome.MethodsThis study is a prospective cross sectional study of patients diagnosed with Auto-inflammatory diseases (AIDs) who was registered in the autoinflammatory computer database registration through periodic fever clinic, which was established for this reason in the medical center of children in 23.09.2012 in Tehran- Iran and data were collected with standardized forms. Inclusion criteria are disease-associated mutations for hereditary periodic fever syndromes [FMF, hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS), TNF receptor 1-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)], or, alternatively, clinically confirmed AID, and periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome with the unknown genetic background.ResultsDuring the first 18 months, 139 patients (93 males, 46 females) from 27 provinces and 11 ethnicities; age (newborn to 18 years) have been recorded and classified as FMF (n=102), PFAPA (n=29), HIDS (n=1), clinically confirmed AID (n=6) including TRAPS (n=1) and CAPS (n=2); CRMO (n=2), DIRA (n=1), and sweet syndrome (n=1). The most common AID in our study were FMF and then PFAPA, CRMO, CAPS. The most common ethnicity was Turkish and Kurdish people. In total 139 patients, genetic analysis was performed in 103 patients, 88.37% have genetically confirmed disease.ConclusionsRecruitment and follow-up of patients with AID will enable us to comprehensively address the correlation between clinical and epidemiological data, genetics.ReferencesINFEVERS: the Registry for FMF and hereditary inflammatory disorders mutations. Nucleic Acids Res, vol 31A retrospective review of autoinflammatory diseases in Saudi children at a rheumatology clinic. Ann Saudi Med 2012 Jan-Feb;32(1):43-8.Disclosure of InterestNone declared</description><identifier>ISSN: 0003-4967</identifier><identifier>EISSN: 1468-2060</identifier><identifier>DOI: 10.1136/annrheumdis-2015-eular.6250</identifier><identifier>CODEN: ARDIAO</identifier><language>eng</language><publisher>London: BMJ Publishing Group LTD</publisher><ispartof>Annals of the rheumatic diseases, 2015-06, Vol.74 (Suppl 2), p.384-384</ispartof><rights>2015, 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</rights><rights>Copyright: 2015 (c) 2015, 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</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttp://ard.bmj.com/content/74/Suppl_2/384.2.full.pdf$$EPDF$$P50$$Gbmj$$H</linktopdf><linktohtml>$$Uhttp://ard.bmj.com/content/74/Suppl_2/384.2.full$$EHTML$$P50$$Gbmj$$H</linktohtml><link.rule.ids>114,115,315,781,785,3197,23576,27929,27930,77605,77636</link.rule.ids></links><search><creatorcontrib>Mehregan, F.</creatorcontrib><creatorcontrib>Ziaee, V.</creatorcontrib><creatorcontrib>Moradinejad, M.H.</creatorcontrib><creatorcontrib>Aghighi, Y.</creatorcontrib><creatorcontrib>Raeeskarimi, S.R.</creatorcontrib><title>THU0509 First 18 Month Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR)</title><title>Annals of the rheumatic diseases</title><description>ObjectivesThe term Auto-inflammatory diseases (AIDs) has been used to describe a group of illness characterized by attacks of seemingly unprovoked inflammation without a significant level of either autoantibodies or antigen-specific T cells more characteristic of autoimmune disease. Published data from Iran regarding autoinflammatory disease in children are scarce. In a multicenter effort, we set out to register genetic, epidemiological and clinical features as well as prognostic factors of these diseases by prospective longitudinal and long-term documentation, in order to define current and novel AIDs and to better understand treatment responses and outcome.MethodsThis study is a prospective cross sectional study of patients diagnosed with Auto-inflammatory diseases (AIDs) who was registered in the autoinflammatory computer database registration through periodic fever clinic, which was established for this reason in the medical center of children in 23.09.2012 in Tehran- Iran and data were collected with standardized forms. Inclusion criteria are disease-associated mutations for hereditary periodic fever syndromes [FMF, hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS), TNF receptor 1-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)], or, alternatively, clinically confirmed AID, and periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome with the unknown genetic background.ResultsDuring the first 18 months, 139 patients (93 males, 46 females) from 27 provinces and 11 ethnicities; age (newborn to 18 years) have been recorded and classified as FMF (n=102), PFAPA (n=29), HIDS (n=1), clinically confirmed AID (n=6) including TRAPS (n=1) and CAPS (n=2); CRMO (n=2), DIRA (n=1), and sweet syndrome (n=1). The most common AID in our study were FMF and then PFAPA, CRMO, CAPS. The most common ethnicity was Turkish and Kurdish people. In total 139 patients, genetic analysis was performed in 103 patients, 88.37% have genetically confirmed disease.ConclusionsRecruitment and follow-up of patients with AID will enable us to comprehensively address the correlation between clinical and epidemiological data, genetics.ReferencesINFEVERS: the Registry for FMF and hereditary inflammatory disorders mutations. Nucleic Acids Res, vol 31A retrospective review of autoinflammatory diseases in Saudi children at a rheumatology clinic. Ann Saudi Med 2012 Jan-Feb;32(1):43-8.Disclosure of InterestNone declared</description><issn>0003-4967</issn><issn>1468-2060</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqVkL9OwzAQhy0EEqXwDpa6wJDiS2LHEVOFCI1URFW1s3GSC03VxMVOkLqx8KI8CSllYGW6P_p9d9JHyAjYGCAQt7pp7Bq7uqic5zPgHnZbbcfC5-yEDCAUsl8LdkoGjLHAC2MRnZML5zb9yCTIAXlZTleMs_jr4zOprGspSPpkmnZNF7gztqWmpKnVTaUbOkdbmaLKaYLvaKluCjrpWlM15VbXtW6N3ffUa-XavrlO58kkXdxckrNSbx1e_dYhWSUPy_upN3t-TO8nMy8DP-Je4ItY5DpHFoGUKHIIokCEGInY52WJeZ6FmjMAngkfIo59EHPEuJAYgBbBkIyOd3fWvHXoWrUxnW36lwpiBpL5oeR96u6Yyq1xzmKpdraqtd0rYOqgVP1Rqg5K1Y9SdVDa0-JIZ_XmX-A37FaBXA</recordid><startdate>201506</startdate><enddate>201506</enddate><creator>Mehregan, F.</creator><creator>Ziaee, V.</creator><creator>Moradinejad, M.H.</creator><creator>Aghighi, Y.</creator><creator>Raeeskarimi, S.R.</creator><general>BMJ Publishing Group LTD</general><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></search><sort><creationdate>201506</creationdate><title>THU0509 First 18 Month Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR)</title><author>Mehregan, F. ; Ziaee, V. ; Moradinejad, M.H. ; Aghighi, Y. ; Raeeskarimi, S.R.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b1275-32696cace07188e6c137364e76925ffeccb4a50115b62175ee07ecee9d8e31a63</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Mehregan, F.</creatorcontrib><creatorcontrib>Ziaee, V.</creatorcontrib><creatorcontrib>Moradinejad, M.H.</creatorcontrib><creatorcontrib>Aghighi, Y.</creatorcontrib><creatorcontrib>Raeeskarimi, S.R.</creatorcontrib><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health &amp; 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 &amp; Medical Complete (Alumni)</collection><collection>ProQuest Biological Science Collection</collection><collection>Consumer Health Database</collection><collection>Health &amp; 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><jtitle>Annals of the rheumatic diseases</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mehregan, F.</au><au>Ziaee, V.</au><au>Moradinejad, M.H.</au><au>Aghighi, Y.</au><au>Raeeskarimi, S.R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>THU0509 First 18 Month Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR)</atitle><jtitle>Annals of the rheumatic diseases</jtitle><date>2015-06</date><risdate>2015</risdate><volume>74</volume><issue>Suppl 2</issue><spage>384</spage><epage>384</epage><pages>384-384</pages><issn>0003-4967</issn><eissn>1468-2060</eissn><coden>ARDIAO</coden><abstract>ObjectivesThe term Auto-inflammatory diseases (AIDs) has been used to describe a group of illness characterized by attacks of seemingly unprovoked inflammation without a significant level of either autoantibodies or antigen-specific T cells more characteristic of autoimmune disease. Published data from Iran regarding autoinflammatory disease in children are scarce. In a multicenter effort, we set out to register genetic, epidemiological and clinical features as well as prognostic factors of these diseases by prospective longitudinal and long-term documentation, in order to define current and novel AIDs and to better understand treatment responses and outcome.MethodsThis study is a prospective cross sectional study of patients diagnosed with Auto-inflammatory diseases (AIDs) who was registered in the autoinflammatory computer database registration through periodic fever clinic, which was established for this reason in the medical center of children in 23.09.2012 in Tehran- Iran and data were collected with standardized forms. Inclusion criteria are disease-associated mutations for hereditary periodic fever syndromes [FMF, hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS), TNF receptor 1-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)], or, alternatively, clinically confirmed AID, and periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome with the unknown genetic background.ResultsDuring the first 18 months, 139 patients (93 males, 46 females) from 27 provinces and 11 ethnicities; age (newborn to 18 years) have been recorded and classified as FMF (n=102), PFAPA (n=29), HIDS (n=1), clinically confirmed AID (n=6) including TRAPS (n=1) and CAPS (n=2); CRMO (n=2), DIRA (n=1), and sweet syndrome (n=1). The most common AID in our study were FMF and then PFAPA, CRMO, CAPS. The most common ethnicity was Turkish and Kurdish people. In total 139 patients, genetic analysis was performed in 103 patients, 88.37% have genetically confirmed disease.ConclusionsRecruitment and follow-up of patients with AID will enable us to comprehensively address the correlation between clinical and epidemiological data, genetics.ReferencesINFEVERS: the Registry for FMF and hereditary inflammatory disorders mutations. Nucleic Acids Res, vol 31A retrospective review of autoinflammatory diseases in Saudi children at a rheumatology clinic. Ann Saudi Med 2012 Jan-Feb;32(1):43-8.Disclosure of InterestNone declared</abstract><cop>London</cop><pub>BMJ Publishing Group LTD</pub><doi>10.1136/annrheumdis-2015-eular.6250</doi><tpages>1</tpages></addata></record>
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