{"id":"https://openalex.org/W3003157228","doi":"https://doi.org/10.4081/reumatismo.2019.1192","title":"Adult-onset systemic autoinflammatory disorders: a clinical approach","display_name":"Adult-onset systemic autoinflammatory disorders: a clinical approach","publication_year":2020,"publication_date":"2020-01-28","ids":{"openalex":"https://openalex.org/W3003157228","doi":"https://doi.org/10.4081/reumatismo.2019.1192","mag":"3003157228","pmid":"https://pubmed.ncbi.nlm.nih.gov/31995956"},"language":"en","primary_location":{"id":"doi:10.4081/reumatismo.2019.1192","doi":"https://doi.org/10.4081/reumatismo.2019.1192","is_oa":true,"landing_page_url":"https://doi.org/10.4081/reumatismo.2019.1192","pdf_url":"https://reumatismo.org/index.php/reuma/article/download/1192/855","source":{"id":"https://openalex.org/S2738672850","display_name":"Reumatismo","issn_l":"0048-7449","issn":["0048-7449","2240-2683"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","ddh","doaj","medline","poland-40","russia-white-list-3"],"host_organization":"https://openalex.org/P4310319913","host_organization_name":"PAGEPress (Italy)","host_organization_lineage":["https://openalex.org/P4310319913"],"host_organization_lineage_names":["PAGEPress (Italy)"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Reumatismo","raw_type":"journal-article"},"parent_works":[],"lineage":["https://openalex.org/W3003157228"],"child_works_count":0,"type":"review","is_traditional":true,"indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"diamond","oa_url":"https://reumatismo.org/index.php/reuma/article/download/1192/855","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5004404511","display_name":"Tiago Borges","orcid":"https://orcid.org/0000-0002-8271-9865","observed_orcids":["https://orcid.org/0000-0002-8271-9865"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"T. Borges","raw_affiliation_strings":["Department of Internal Medicine, Trofa Sa\u00fade Hospital Privado de Gaia, Vila Nova de Gaia. mtiagoborges@gmail.com","Department of Internal Medicine, Trofa Sa\u00fade Hospital Privado de Gaia, Vila Nova de Gaia"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Internal Medicine, Trofa Sa\u00fade Hospital Privado de Gaia, Vila Nova de Gaia. mtiagoborges@gmail.com","institution_ids":[]},{"raw_affiliation_string":"Department of Internal Medicine, Trofa Sa\u00fade Hospital Privado de Gaia, Vila Nova de Gaia","institution_ids":[]}]},{"author_position":"middle","author":{"id":null,"display_name":"A. Barbosa","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I3018176973","display_name":"Hospital de S\u00e3o Jo\u00e3o","ror":"https://ror.org/04qsnc772","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I3018176973"]}],"countries":["PT"],"is_corresponding":true,"raw_author_name":"A. Barbosa","raw_affiliation_strings":["Department of Internal Medicine, Centro Hospitalar de S\u00e3o Jo\u00e3o, Porto"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Internal Medicine, Centro Hospitalar de S\u00e3o Jo\u00e3o, Porto","institution_ids":["https://openalex.org/I3018176973"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5007375100","display_name":"S. Silva","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"S. Silva","raw_affiliation_strings":["Department of Internal Medicine, Trofa Sa\u00fade Hospital Privado de Gaia, Vila Nova de Gaia"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Internal Medicine, Trofa Sa\u00fade Hospital Privado de Gaia, Vila Nova de Gaia","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5004404511","https://openalex.org/A5007375100"],"corresponding_institution_ids":["https://openalex.org/I3018176973"],"apc_list":null,"apc_paid":null,"fwci":0.1627,"has_fulltext":true,"cited_by_count":11,"citation_normalized_percentile":{"value":0.10942813,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":87,"max":96},"biblio":{"volume":"71","issue":"4","first_page":"177","last_page":"188"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11078","display_name":"Inflammasome and immune disorders","score":0.9839000105857849,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11078","display_name":"Inflammasome and immune disorders","score":0.9839000105857849,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","score":0.014999999664723873,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10371","display_name":"Immune Response and Inflammation","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/innate-immune-dysregulation","display_name":"innate immune dysregulation","score":0.7789999842643738},{"id":"https://openalex.org/keywords/monogenic-autoinflammatory-diseases","display_name":"monogenic autoinflammatory diseases","score":0.8700000047683716},{"id":"https://openalex.org/keywords/familial-mediterranean-fever","display_name":"familial Mediterranean fever","score":0.9179999828338623},{"id":"https://openalex.org/keywords/adult-onset-still-s-disease","display_name":"adult-onset Still's disease","score":0.9919999837875366},{"id":"https://openalex.org/keywords/fever-of-unknown-origin","display_name":"fever of unknown origin","score":0.9480000138282776},{"id":"https://openalex.org/keywords/polyserositis","display_name":"polyserositis","score":0.8870000243186951},{"id":"https://openalex.org/keywords/amyloidosis","display_name":"amyloidosis","score":0.8479999899864197},{"id":"https://openalex.org/keywords/delayed-diagnosis","display_name":"delayed diagnosis","score":0.7480000257492065},{"id":"https://openalex.org/keywords/colchicine","display_name":"colchicine","score":0.8569999933242798},{"id":"https://openalex.org/keywords/il-1-inhibitors","display_name":"IL-1 inhibitors","score":0.9959999918937683},{"id":"https://openalex.org/keywords/tumor-necrosis-factor-inhibitors","display_name":"tumor necrosis factor inhibitors","score":0.968999981880188},{"id":"https://openalex.org/keywords/immunosuppressive-therapy","display_name":"immunosuppressive therapy","score":0.8500000238418579}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9294463396072388},{"id":"https://openalex.org/C2779537928","wikidata":"https://www.wikidata.org/wiki/Q1144618","display_name":"Familial Mediterranean fever","level":3,"score":0.8515211939811707},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.5551822781562805},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.5472573041915894},{"id":"https://openalex.org/C2776252253","wikidata":"https://www.wikidata.org/wiki/Q7663815","display_name":"Systemic inflammation","level":3,"score":0.49473831057548523},{"id":"https://openalex.org/C2780194698","wikidata":"https://www.wikidata.org/wiki/Q6005429","display_name":"Immune dysregulation","level":3,"score":0.4190119206905365},{"id":"https://openalex.org/C2777767895","wikidata":"https://www.wikidata.org/wiki/Q4691924","display_name":"Age of onset","level":3,"score":0.41687291860580444},{"id":"https://openalex.org/C2778674544","wikidata":"https://www.wikidata.org/wiki/Q1414805","display_name":"Systemic disease","level":3,"score":0.41491320729255676},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.3947048485279083},{"id":"https://openalex.org/C2780648854","wikidata":"https://www.wikidata.org/wiki/Q2745824","display_name":"Immunopathology","level":2,"score":0.2503144145011902},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.23061367869377136},{"id":"https://openalex.org/C2776914184","wikidata":"https://www.wikidata.org/wiki/Q101991","display_name":"Inflammation","level":2,"score":0.22159159183502197}],"mesh":[{"descriptor_ui":"D000079424","descriptor_name":"Tumor Necrosis Factor Inhibitors","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000894","descriptor_name":"Anti-Inflammatory Agents, Non-Steroidal","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D001327","descriptor_name":"Autoimmune Diseases","qualifier_ui":"Q000145","qualifier_name":"classification","is_major_topic":true},{"descriptor_ui":"D003078","descriptor_name":"Colchicine","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005335","descriptor_name":"Fever of Unknown Origin","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007113","descriptor_name":"Immunity, Innate","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007166","descriptor_name":"Immunosuppressive Agents","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D007375","descriptor_name":"Interleukin-1","qualifier_ui":"Q000037","qualifier_name":"antagonists & inhibitors","is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D013256","descriptor_name":"Steroids","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D017668","descriptor_name":"Age of Onset","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D056660","descriptor_name":"Hereditary Autoinflammatory Diseases","qualifier_ui":"Q000145","qualifier_name":"classification","is_major_topic":true},{"descriptor_ui":"D056660","descriptor_name":"Hereditary Autoinflammatory Diseases","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D056660","descriptor_name":"Hereditary Autoinflammatory Diseases","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":true},{"descriptor_ui":"D056660","descriptor_name":"Hereditary Autoinflammatory Diseases","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D057210","descriptor_name":"Delayed Diagnosis","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":3,"locations":[{"id":"doi:10.4081/reumatismo.2019.1192","doi":"https://doi.org/10.4081/reumatismo.2019.1192","is_oa":true,"landing_page_url":"https://doi.org/10.4081/reumatismo.2019.1192","pdf_url":"https://reumatismo.org/index.php/reuma/article/download/1192/855","source":{"id":"https://openalex.org/S2738672850","display_name":"Reumatismo","issn_l":"0048-7449","issn":["0048-7449","2240-2683"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","ddh","doaj","medline","poland-40","russia-white-list-3"],"host_organization":"https://openalex.org/P4310319913","host_organization_name":"PAGEPress (Italy)","host_organization_lineage":["https://openalex.org/P4310319913"],"host_organization_lineage_names":["PAGEPress (Italy)"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Reumatismo","raw_type":"journal-article"},{"id":"pmid:31995956","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/31995956","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Reumatismo","raw_type":"Journal Article"},{"id":"pmh:oai:doaj.org/article:e2b94411db7d483f94c1e253a7ff6d5f","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/e2b94411db7d483f94c1e253a7ff6d5f","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Reumatismo, Vol 71, Iss 4 (2020)","raw_type":"article"}],"best_oa_location":{"id":"doi:10.4081/reumatismo.2019.1192","doi":"https://doi.org/10.4081/reumatismo.2019.1192","is_oa":true,"landing_page_url":"https://doi.org/10.4081/reumatismo.2019.1192","pdf_url":"https://reumatismo.org/index.php/reuma/article/download/1192/855","source":{"id":"https://openalex.org/S2738672850","display_name":"Reumatismo","issn_l":"0048-7449","issn":["0048-7449","2240-2683"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","ddh","doaj","medline","poland-40","russia-white-list-3"],"host_organization":"https://openalex.org/P4310319913","host_organization_name":"PAGEPress (Italy)","host_organization_lineage":["https://openalex.org/P4310319913"],"host_organization_lineage_names":["PAGEPress (Italy)"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Reumatismo","raw_type":"journal-article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","score":1.0,"id":"https://metadata.un.org/sdg/3"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W3003157228.pdf","grobid_xml":"https://content.openalex.org/works/W3003157228.grobid-xml"},"referenced_works_count":78,"referenced_works":["https://openalex.org/W560283840","https://openalex.org/W1756186076","https://openalex.org/W1872746982","https://openalex.org/W1936100365","https://openalex.org/W1968555750","https://openalex.org/W1987218634","https://openalex.org/W1988788868","https://openalex.org/W1994705699","https://openalex.org/W2009141378","https://openalex.org/W2012883414","https://openalex.org/W2015602908","https://openalex.org/W2023601531","https://openalex.org/W2027227375","https://openalex.org/W2029873080","https://openalex.org/W2033618411","https://openalex.org/W2038864991","https://openalex.org/W2043935898","https://openalex.org/W2051588919","https://openalex.org/W2059194364","https://openalex.org/W2074394673","https://openalex.org/W2074547696","https://openalex.org/W2077145568","https://openalex.org/W2078464144","https://openalex.org/W2095667188","https://openalex.org/W2102498884","https://openalex.org/W2102865263","https://openalex.org/W2103497547","https://openalex.org/W2103599902","https://openalex.org/W2121150699","https://openalex.org/W2124927689","https://openalex.org/W2144483124","https://openalex.org/W2147831784","https://openalex.org/W2152325695","https://openalex.org/W2160604747","https://openalex.org/W2172060710","https://openalex.org/W2184002743","https://openalex.org/W2228700947","https://openalex.org/W2288193256","https://openalex.org/W2315422770","https://openalex.org/W2341032101","https://openalex.org/W2469890242","https://openalex.org/W2470366316","https://openalex.org/W2480597338","https://openalex.org/W2487030781","https://openalex.org/W2510457124","https://openalex.org/W2513561265","https://openalex.org/W2516720691","https://openalex.org/W2529023545","https://openalex.org/W2530407119","https://openalex.org/W2531506426","https://openalex.org/W2531755736","https://openalex.org/W2550568699","https://openalex.org/W2555100605","https://openalex.org/W2555351070","https://openalex.org/W2556238270","https://openalex.org/W2558307473","https://openalex.org/W2572897530","https://openalex.org/W2585237743","https://openalex.org/W2592260987","https://openalex.org/W2601048381","https://openalex.org/W2645220177","https://openalex.org/W2735705864","https://openalex.org/W2738124581","https://openalex.org/W2740861638","https://openalex.org/W2741605507","https://openalex.org/W2742590969","https://openalex.org/W2756936698","https://openalex.org/W2763945511","https://openalex.org/W2796861675","https://openalex.org/W2797247601","https://openalex.org/W4230462158","https://openalex.org/W4241915332","https://openalex.org/W4242091643","https://openalex.org/W4244070729","https://openalex.org/W4255220861","https://openalex.org/W4296481940","https://openalex.org/W6669136114","https://openalex.org/W6813486059"],"related_works":["https://openalex.org/W3130900687","https://openalex.org/W2402516657","https://openalex.org/W3172967612","https://openalex.org/W4399040953","https://openalex.org/W2998794013","https://openalex.org/W2998511993","https://openalex.org/W2943877762","https://openalex.org/W4416445511","https://openalex.org/W4399702642","https://openalex.org/W2912659876"],"abstract_inverted_index":{"Autoinflammatory":[0],"disorders":[1],"(AIDs)":[2],"are":[3,33,61,104,146],"a":[4,12,174],"subgroup":[5],"of":[6,15,39,123,163,177],"immune-mediated":[7],"syndromes":[8],"that":[9],"result":[10],"from":[11],"primary":[13],"dysfunction":[14],"the":[16,47],"innate":[17],"immune":[18],"system.":[19],"AIDs":[20,32,72,85,88,103,114,125,157],"can":[21],"be":[22,64],"either":[23],"monogenic":[24,71,99,113],"or":[25,82,143,181],"polygenic":[26,56,96,105],"diseases.":[27],"Unlike":[28],"organspecific":[29],"AIDs,":[30,52],"systemic":[31,51,84,124,205],"characterized":[34],"by":[35],"fever":[36,119,162],"and/or":[37],"elevation":[38],"acute-phase":[40],"reactants.":[41],"This":[42],"review":[43],"aims":[44],"to":[45,63,132,153],"describe":[46],"most":[48],"common":[49,156,201],"adult-onset":[50,98,102,178],"focusing":[53],"mostly":[54],"on":[55],"and":[57,97,135,195],"mixed-pattern":[58],"diseases":[59],"which":[60],"expected":[62],"more":[65],"prevalent":[66],"in":[67,78,91,126,158],"adult":[68,159],"patients":[69,160],"than":[70],"overall.":[73],"The":[74,121],"literature":[75],"was":[76],"searched":[77],"Medline":[79],"database.":[80],"Organ-specific":[81],"childhood-onset":[83],"were":[86,89],"excluded.":[87],"divided":[90],"three":[92],"distinct":[93],"groups:":[94],"mixed-pattern,":[95],"AIDs.":[100,206],"Most":[101],"but":[106],"late-onset":[107],"disease":[108,180],"is":[109,128,138,183],"not":[110],"rare":[111],"among":[112],"such":[115,170],"as":[116,171],"familial":[117],"Mediterranean":[118],"(FMF).":[120],"diagnosis":[122],"adults":[127],"often":[129],"delayed":[130],"due":[131],"several":[133],"factors":[134],"sometimes":[136],"it":[137,149],"only":[139],"established":[140],"when":[141],"amyloidosis":[142],"other":[144],"complications":[145],"present.":[147],"Therefore,":[148],"probably":[150,167],"makes":[151],"sense":[152],"primarily":[154],"exclude":[155],"with":[161],"unknown":[164],"origin":[165],"(and":[166],"different":[168],"presentations":[169],"polyserositis)":[172],"since":[173],"high":[175],"prevalence":[176],"Still's":[179],"FMF":[182],"usually":[184],"expected.":[185],"Colchicine,":[186],"nonsteroidal":[187],"anti-inflammatory":[188],"drugs,":[189],"steroids,":[190],"immunosuppressive":[191],"agents,":[192],"interleukin-1":[193],"inhibitors":[194],"tumor":[196],"necrosis":[197],"factor":[198],"antagonists":[199],"constitute":[200],"therapeutic":[202],"options":[203],"for":[204]},"counts_by_year":[{"year":2026,"cited_by_count":2},{"year":2025,"cited_by_count":1},{"year":2024,"cited_by_count":1},{"year":2023,"cited_by_count":3},{"year":2022,"cited_by_count":3},{"year":2021,"cited_by_count":1}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2020-02-07T00:00:00"}
