{"id":"https://openalex.org/W2931789851","doi":"https://doi.org/10.1093/rheumatology/kez058.033","title":"093. ATYPICAL PRESENTATION OF ANCA-ASSOCIATED VASCULITIS: A CASE SERIES","display_name":"093. ATYPICAL PRESENTATION OF ANCA-ASSOCIATED VASCULITIS: A CASE SERIES","publication_year":2019,"publication_date":"2019-03-01","ids":{"openalex":"https://openalex.org/W2931789851","doi":"https://doi.org/10.1093/rheumatology/kez058.033","mag":"2931789851"},"language":"en","primary_location":{"id":"doi:10.1093/rheumatology/kez058.033","doi":"https://doi.org/10.1093/rheumatology/kez058.033","is_oa":true,"landing_page_url":"https://doi.org/10.1093/rheumatology/kez058.033","pdf_url":"https://academic.oup.com/rheumatology/article-pdf/58/Supplement_2/kez058.033/28281140/kez058.033.pdf","source":{"id":"https://openalex.org/S183989336","display_name":"Lara D. Veeken","issn_l":"1462-0324","issn":["1462-0324","1462-0332"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311648","host_organization_name":"Oxford University Press","host_organization_lineage":["https://openalex.org/P4310311648","https://openalex.org/P4310311647"],"host_organization_lineage_names":["Oxford University Press","University of Oxford"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":true,"oa_status":"bronze","oa_url":"https://academic.oup.com/rheumatology/article-pdf/58/Supplement_2/kez058.033/28281140/kez058.033.pdf","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5070519692","display_name":"Sam Kant","orcid":"https://orcid.org/0000-0001-5863-3562","observed_orcids":["https://orcid.org/0000-0001-5863-3562"]},"institutions":[{"id":"https://openalex.org/I1315496137","display_name":"University of Maryland Medical Center","ror":"https://ror.org/00sde4n60","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1315496137","https://openalex.org/I14285509"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sam Kant","raw_affiliation_strings":["University of Maryland Medical Center Baltimore, MD"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"University of Maryland Medical Center Baltimore, MD","institution_ids":["https://openalex.org/I1315496137"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5048771742","display_name":"Eric J. Gapud","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210150714","display_name":"Johns Hopkins Hospital","ror":"https://ror.org/05cb1k848","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I2799853436","https://openalex.org/I4210150714"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Eric Gapud","raw_affiliation_strings":["Johns Hopkins Hospital Baltimore, MD"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Johns Hopkins Hospital Baltimore, MD","institution_ids":["https://openalex.org/I4210150714"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5073621138","display_name":"Philip Seo","orcid":"https://orcid.org/0000-0003-3435-6737","observed_orcids":["https://orcid.org/0000-0003-3435-6737"]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Philip Seo","raw_affiliation_strings":["Johns Hopkins University Baltimore, MD"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Johns Hopkins University Baltimore, MD","institution_ids":["https://openalex.org/I145311948"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5004382663","display_name":"Rebecca Manno","orcid":"https://orcid.org/0000-0001-8113-8893","observed_orcids":["https://orcid.org/0000-0001-8113-8893"]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Rebecca Manno","raw_affiliation_strings":["Johns Hopkins University Baltimore, MD"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Johns Hopkins University Baltimore, MD","institution_ids":["https://openalex.org/I145311948"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5047864877","display_name":"Brendan Antiochos","orcid":"https://orcid.org/0000-0001-6166-9750","observed_orcids":["https://orcid.org/0000-0001-6166-9750"]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Brendan Antiochos","raw_affiliation_strings":["Johns Hopkins University Baltimore, MD"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Johns Hopkins University Baltimore, MD","institution_ids":["https://openalex.org/I145311948"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Michael Phillips","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Michael Phillips","raw_affiliation_strings":["Johns Hopkins University Baltimore, MD"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Johns Hopkins University Baltimore, MD","institution_ids":["https://openalex.org/I145311948"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5058786394","display_name":"Duvuru Geetha","orcid":"https://orcid.org/0000-0001-8353-5542","observed_orcids":["https://orcid.org/0000-0001-8353-5542"]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]},{"id":"https://openalex.org/I2802697821","display_name":"Johns Hopkins Bayview Medical Center","ror":"https://ror.org/04pwc8466","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I2799853436","https://openalex.org/I2802697821"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Duvuru Geetha","raw_affiliation_strings":["Johns Hopkins Bayview Medical Center Baltimore, MD"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Johns Hopkins Bayview Medical Center Baltimore, MD","institution_ids":["https://openalex.org/I145311948","https://openalex.org/I2802697821"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":4,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":{"value":3486,"currency":"GBP","value_usd":4597},"apc_paid":null,"fwci":0.0,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":{"value":0.03857656,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"58","issue":"Supplement_2","first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10697","display_name":"Vasculitis and related conditions","score":0.9994999766349792,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10697","display_name":"Vasculitis and related conditions","score":0.9994999766349792,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"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/T11029","display_name":"Renal Diseases and Glomerulopathies","score":0.0003000000142492354,"subfield":{"id":"https://openalex.org/subfields/2727","display_name":"Nephrology"},"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/T10308","display_name":"Systemic Lupus Erythematosus Research","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/anca-associated-vasculitis","display_name":"ANCA-associated vasculitis","score":1.0},{"id":"https://openalex.org/keywords/atypical-presentation","display_name":"atypical presentation","score":0.9929999709129333},{"id":"https://openalex.org/keywords/pr3-anca","display_name":"PR3-ANCA","score":0.7099999785423279},{"id":"https://openalex.org/keywords/acute-kidney-injury","display_name":"acute kidney injury","score":0.8989999890327454},{"id":"https://openalex.org/keywords/renal-dysfunction","display_name":"renal dysfunction","score":0.6600000262260437},{"id":"https://openalex.org/keywords/pancreatitis","display_name":"pancreatitis","score":0.6510000228881836},{"id":"https://openalex.org/keywords/usual-interstitial-pneumonia","display_name":"usual interstitial pneumonia","score":0.7699999809265137},{"id":"https://openalex.org/keywords/cystic-lung-disease","display_name":"cystic lung disease","score":0.9110000133514404},{"id":"https://openalex.org/keywords/splenic-infarction","display_name":"splenic infarction","score":0.9769999980926514},{"id":"https://openalex.org/keywords/delayed-diagnosis","display_name":"delayed diagnosis","score":0.7559999823570251},{"id":"https://openalex.org/keywords/rituximab","display_name":"rituximab","score":0.7680000066757202},{"id":"https://openalex.org/keywords/cyclophosphamide","display_name":"cyclophosphamide","score":0.9369999766349792},{"id":"https://openalex.org/keywords/corticosteroid-therapy","display_name":"corticosteroid therapy","score":0.4880000054836273},{"id":"https://openalex.org/keywords/dialysis-dependence","display_name":"dialysis dependence","score":0.6729999780654907}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9581654071807861},{"id":"https://openalex.org/C2777601897","wikidata":"https://www.wikidata.org/wiki/Q3409113","display_name":"Presentation (obstetrics)","level":2,"score":0.7780827283859253},{"id":"https://openalex.org/C2910998276","wikidata":"https://www.wikidata.org/wiki/Q7148839","display_name":"ANCA-Associated Vasculitis","level":4,"score":0.6976873278617859},{"id":"https://openalex.org/C2776015282","wikidata":"https://www.wikidata.org/wiki/Q644318","display_name":"Vasculitis","level":3,"score":0.6338019371032715},{"id":"https://openalex.org/C16005928","wikidata":"https://www.wikidata.org/wiki/Q171171","display_name":"Dermatology","level":1,"score":0.5364220142364502},{"id":"https://openalex.org/C143724316","wikidata":"https://www.wikidata.org/wiki/Q312468","display_name":"Series (stratigraphy)","level":2,"score":0.4760337173938751},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.2374105155467987},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.13715115189552307},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.056761324405670166},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.0},{"id":"https://openalex.org/C151730666","wikidata":"https://www.wikidata.org/wiki/Q7205","display_name":"Paleontology","level":1,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1093/rheumatology/kez058.033","doi":"https://doi.org/10.1093/rheumatology/kez058.033","is_oa":true,"landing_page_url":"https://doi.org/10.1093/rheumatology/kez058.033","pdf_url":"https://academic.oup.com/rheumatology/article-pdf/58/Supplement_2/kez058.033/28281140/kez058.033.pdf","source":{"id":"https://openalex.org/S183989336","display_name":"Lara D. Veeken","issn_l":"1462-0324","issn":["1462-0324","1462-0332"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311648","host_organization_name":"Oxford University Press","host_organization_lineage":["https://openalex.org/P4310311648","https://openalex.org/P4310311647"],"host_organization_lineage_names":["Oxford University Press","University of Oxford"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology","raw_type":"journal-article"}],"best_oa_location":{"id":"doi:10.1093/rheumatology/kez058.033","doi":"https://doi.org/10.1093/rheumatology/kez058.033","is_oa":true,"landing_page_url":"https://doi.org/10.1093/rheumatology/kez058.033","pdf_url":"https://academic.oup.com/rheumatology/article-pdf/58/Supplement_2/kez058.033/28281140/kez058.033.pdf","source":{"id":"https://openalex.org/S183989336","display_name":"Lara D. Veeken","issn_l":"1462-0324","issn":["1462-0324","1462-0332"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311648","host_organization_name":"Oxford University Press","host_organization_lineage":["https://openalex.org/P4310311648","https://openalex.org/P4310311647"],"host_organization_lineage_names":["Oxford University Press","University of Oxford"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Rheumatology","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W2931789851.pdf","grobid_xml":"https://content.openalex.org/works/W2931789851.grobid-xml"},"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W2943214398","https://openalex.org/W4379650368","https://openalex.org/W4379802221","https://openalex.org/W2389630554","https://openalex.org/W3029000232","https://openalex.org/W2741896151","https://openalex.org/W2929653453","https://openalex.org/W4404382017","https://openalex.org/W3028858905","https://openalex.org/W2132473580"],"abstract_inverted_index":{"Background:":[0],"ANCA":[1,40,179,186],"associated":[2,271],"vasculitis":[3],"(AAV)":[4],"can":[5,269],"present":[6,161],"in":[7,25,38,108,162,174,211,267],"an":[8,191],"atypical":[9,46,75,142],"manner":[10],"and":[11,22,50,60,91,153,157,181,196,203,250,281],"obscure":[12],"the":[13,67,120,139,199,212,218],"clinical":[14,20],"picture.":[15],"We":[16,30],"sought":[17],"to":[18,42,73,279],"characterize":[19],"characteristics":[21],"renal":[23,53,58,182,229],"outcomes":[24],"these":[26,175,273],"uncommon":[27],"presentations.":[28,76,274],"Methods:":[29],"conducted":[31],"a":[32,246,264],"retrospective":[33],"study":[34],"of":[35,48,82,112,119,132,170,185,194,208,239,243,257],"136":[36,68],"patients":[37,44,78,99,121,164,176,189,220,225],"our":[39],"database":[41],"identify":[43],"with":[45,84,130,147,233,245,272],"presentation":[47,143],"AAV":[49,171,240],"subsequently":[51,125],"developed":[52,126],"dysfunction.":[54],"Patient":[55],"demographics,":[56],"serologies,":[57],"indices":[59],"treatment":[61],"regimens":[62],"were":[63,71,79,100],"assessed.":[64],"Results:":[65],"Of":[66],"patients,":[69],"8":[70],"identified":[72],"have":[74],"These":[77],"usually":[80],"extremes":[81,242],"age":[83,244],"three":[85],"being":[86,93,160],"less":[87],"than":[88,95],"30":[89],"years":[90],"four":[92],"more":[94],"70":[96],"years.":[97],"Six":[98,188],"positive":[101,178],"for":[102],"PR3":[103,247],"antibodies.":[104],"The":[105,168],"mean":[106],"delay":[107,266],"diagnosis":[109,169,216,268],"from":[110],"time":[111],"symptom":[113],"development":[114,256],"was":[115,138,172],"9":[116],"months.":[117],"Majority":[118],"(n":[122,144],"=":[123,145],"7)":[124],"acute":[127],"kidney":[128],"injury,":[129],"two":[131,232],"them":[133],"requiring":[134],"temporary":[135],"hemodialysis.":[136],"Pancreatitis":[137],"most":[140],"frequent":[141],"3),":[146],"pulmonary":[148],"pathologies":[149],"(cystic":[150],"lung":[151],"disease":[152,222],"usual":[154],"interstitial":[155],"pneumonia)":[156],"splenic":[158],"infarcts":[159],"2":[163],"each":[165],"(Table":[166],"1).":[167],"established":[173],"by":[177,254],"serology":[180],"biopsy":[183],"evidence":[184],"GN.":[187],"had":[190],"induction":[192],"regimen":[193],"steroids":[195,202],"rituximab,":[197],"whilst":[198],"remainder":[200],"received":[201],"cyclophosphamide.":[204],"One":[205],"patient":[206],"died":[207],"respiratory":[209],"failure":[210],"first":[213],"month":[214],"following":[215],"while":[217],"remaining":[219],"achieved":[221],"remission.":[223],"Four":[224],"did":[226],"not":[227],"recover":[228],"function":[230],"(including":[231],"dialysis":[234],"dependence).":[235],"Conclusion:":[236],"Atypical":[237],"presentations":[238],"afflict":[241],"predominant":[248],"antibody":[249],"would":[251],"be":[252,270],"complicated":[253],"subsequent":[255],"AKI.":[258],"This":[259],"case":[260],"series":[261],"demonstrates":[262],"that":[263],"significant":[265],"Disclosures:":[275],"Duvuru":[276],"Geetha-":[277],"consultant":[278],"Chemocentry":[280],"Kyowa":[282],"Hakko":[283],"Kirin;":[284],"Other":[285],"authors-none":[286]},"counts_by_year":[],"updated_date":"2026-10-29T09:56:32.521200","created_date":"2019-04-11T00:00:00"}