{"id":"https://openalex.org/W7222865576","doi":"https://doi.org/10.1155/crrh/9165820","title":"Hemophagocytic Lymphohistiocytosis Thought to Be due to Undifferentiated Connective Tissue Disease Treated With Ruxolitinib: A Case Report","display_name":"Hemophagocytic Lymphohistiocytosis Thought to Be due to Undifferentiated Connective Tissue Disease Treated With Ruxolitinib: A Case Report","publication_year":2026,"publication_date":"2026-01-01","ids":{"openalex":"https://openalex.org/W7222865576","doi":"https://doi.org/10.1155/crrh/9165820","pmid":"https://pubmed.ncbi.nlm.nih.gov/42894865","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/13677135"},"language":"en","primary_location":{"id":"doi:10.1155/crrh/9165820","doi":"https://doi.org/10.1155/crrh/9165820","is_oa":true,"landing_page_url":"https://doi.org/10.1155/crrh/9165820","pdf_url":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1155/crrh/9165820","source":{"id":"https://openalex.org/S2764880244","display_name":"Case Reports in Rheumatology","issn_l":"2090-6889","issn":["2090-6889","2090-6897"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj"],"host_organization":"https://openalex.org/P4310319869","host_organization_name":"Hindawi Publishing Corporation","host_organization_lineage":["https://openalex.org/P4310319869","https://openalex.org/P4310320595"],"host_organization_lineage_names":["Hindawi Publishing Corporation","Wiley"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Case Reports in Rheumatology","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1155/crrh/9165820","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5033723734","display_name":"Emily A. Vachon","orcid":"https://orcid.org/0009-0002-3638-482X","observed_orcids":["https://orcid.org/0009-0002-3638-482X"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Emily Vachon","raw_affiliation_strings":["Carle Illinois College of Medicine  Urbana Illinois USA"],"raw_orcid":"https://orcid.org/0009-0002-3638-482X","affiliations":[{"raw_affiliation_string":"Carle Illinois College of Medicine  Urbana Illinois USA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5159009496","display_name":"Heena Birbal Jain","orcid":"https://orcid.org/0009-0002-8323-3530","observed_orcids":["https://orcid.org/0009-0002-8323-3530"]},"institutions":[{"id":"https://openalex.org/I1308343090","display_name":"Carle Foundation Hospital","ror":"https://ror.org/02nfcgd30","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1308343090"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Heena Birbal Jain","raw_affiliation_strings":["Carle Foundation Hospital  Urbana Illinois USA  carle.org"],"raw_orcid":"https://orcid.org/0009-0002-8323-3530","affiliations":[{"raw_affiliation_string":"Carle Foundation Hospital  Urbana Illinois USA  carle.org","institution_ids":["https://openalex.org/I1308343090"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5074115387","display_name":"Suzanna Kitten","orcid":"https://orcid.org/0009-0007-9808-2807","observed_orcids":["https://orcid.org/0009-0007-9808-2807"]},"institutions":[{"id":"https://openalex.org/I1308343090","display_name":"Carle Foundation Hospital","ror":"https://ror.org/02nfcgd30","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1308343090"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Suzanna Kitten","raw_affiliation_strings":["Carle Foundation Hospital  Urbana Illinois USA  carle.org","Carle Illinois College of Medicine  Urbana Illinois USA"],"raw_orcid":"https://orcid.org/0009-0007-9808-2807","affiliations":[{"raw_affiliation_string":"Carle Foundation Hospital  Urbana Illinois USA  carle.org","institution_ids":["https://openalex.org/I1308343090"]},{"raw_affiliation_string":"Carle Illinois College of Medicine  Urbana Illinois USA","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5033723734"],"corresponding_institution_ids":[],"apc_list":{"value":790,"currency":"USD","value_usd":790},"apc_paid":{"value":790,"currency":"USD","value_usd":790},"fwci":0.0,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":{"value":0.76670442,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"2026","issue":"1","first_page":"9165820","last_page":"9165820"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","score":1.0,"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"}},"topics":[{"id":"https://openalex.org/T11616","display_name":"Autoimmune and Inflammatory Disorders Research","score":1.0,"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/T11892","display_name":"Kawasaki Disease and Coronary Complications","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2735","display_name":"Pediatrics, Perinatology and Child Health"},"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/T11078","display_name":"Inflammasome and immune disorders","score":0.0,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/hemophagocytic-lymphohistiocytosis","display_name":"hemophagocytic lymphohistiocytosis","score":0.9829999804496765},{"id":"https://openalex.org/keywords/secondary-hemophagocytic-lymphohistiocytosis","display_name":"secondary hemophagocytic lymphohistiocytosis","score":0.9480000138282776},{"id":"https://openalex.org/keywords/undifferentiated-connective-tissue-disease","display_name":"undifferentiated connective tissue disease","score":0.9829999804496765},{"id":"https://openalex.org/keywords/ruxolitinib","display_name":"ruxolitinib","score":0.9959999918937683},{"id":"https://openalex.org/keywords/lamotrigine","display_name":"lamotrigine","score":0.8169999718666077},{"id":"https://openalex.org/keywords/salvage-therapy","display_name":"salvage therapy","score":0.7350000143051147},{"id":"https://openalex.org/keywords/systemic-inflammatory-syndrome","display_name":"systemic inflammatory syndrome","score":0.7889999747276306}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8697999715805054},{"id":"https://openalex.org/C2779079688","wikidata":"https://www.wikidata.org/wiki/Q1642170","display_name":"Hemophagocytic lymphohistiocytosis","level":3,"score":0.6686999797821045},{"id":"https://openalex.org/C518705261","wikidata":"https://www.wikidata.org/wiki/Q25615","display_name":"Connective tissue","level":2,"score":0.6345999836921692},{"id":"https://openalex.org/C2776670291","wikidata":"https://www.wikidata.org/wiki/Q1779300","display_name":"Connective tissue disease","level":4,"score":0.5252000093460083},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.41429999470710754},{"id":"https://openalex.org/C16005928","wikidata":"https://www.wikidata.org/wiki/Q171171","display_name":"Dermatology","level":1,"score":0.4041999876499176},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.3774000108242035},{"id":"https://openalex.org/C2776112149","wikidata":"https://www.wikidata.org/wiki/Q7383611","display_name":"Ruxolitinib","level":4,"score":0.34450000524520874},{"id":"https://openalex.org/C2776015282","wikidata":"https://www.wikidata.org/wiki/Q644318","display_name":"Vasculitis","level":3,"score":0.31119999289512634},{"id":"https://openalex.org/C2778962941","wikidata":"https://www.wikidata.org/wiki/Q16543725","display_name":"Undifferentiated connective tissue disease","level":5,"score":0.2946000099182129},{"id":"https://openalex.org/C2779701055","wikidata":"https://www.wikidata.org/wiki/Q929833","display_name":"Rare disease","level":3,"score":0.28679999709129333},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.2847000062465668},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.2800000011920929},{"id":"https://openalex.org/C187212893","wikidata":"https://www.wikidata.org/wiki/Q123028","display_name":"Pediatrics","level":1,"score":0.2671000063419342},{"id":"https://openalex.org/C2778802184","wikidata":"https://www.wikidata.org/wiki/Q410346","display_name":"Lamotrigine","level":3,"score":0.26089999079704285},{"id":"https://openalex.org/C2775934546","wikidata":"https://www.wikidata.org/wiki/Q179991","display_name":"Biopsy","level":2,"score":0.2551000118255615},{"id":"https://openalex.org/C3018696855","wikidata":"https://www.wikidata.org/wiki/Q1414805","display_name":"Multisystem disease","level":3,"score":0.25380000472068787}],"mesh":[],"locations_count":4,"locations":[{"id":"doi:10.1155/crrh/9165820","doi":"https://doi.org/10.1155/crrh/9165820","is_oa":true,"landing_page_url":"https://doi.org/10.1155/crrh/9165820","pdf_url":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1155/crrh/9165820","source":{"id":"https://openalex.org/S2764880244","display_name":"Case Reports in Rheumatology","issn_l":"2090-6889","issn":["2090-6889","2090-6897"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj"],"host_organization":"https://openalex.org/P4310319869","host_organization_name":"Hindawi Publishing Corporation","host_organization_lineage":["https://openalex.org/P4310319869","https://openalex.org/P4310320595"],"host_organization_lineage_names":["Hindawi Publishing Corporation","Wiley"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Case Reports in Rheumatology","raw_type":"journal-article"},{"id":"pmh:oai:pubmedcentral.nih.gov:13677135","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13677135/","pdf_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13677135/pdf/CRRH-2026-9165820.pdf","source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"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":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Case Rep Rheumatol","raw_type":"Text"},{"id":"pmid:42894865","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/42894865","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":"Case reports in rheumatology","raw_type":"Journal Article"},{"id":"pmh:oai:doaj.org/article:59ab6dfd9552488bbbdeaf3bbf69dd93","doi":null,"is_oa":false,"landing_page_url":"https://doaj.org/article/59ab6dfd9552488bbbdeaf3bbf69dd93","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":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Case Reports in Rheumatology, Vol 2026 (2026)","raw_type":"article"}],"best_oa_location":{"id":"doi:10.1155/crrh/9165820","doi":"https://doi.org/10.1155/crrh/9165820","is_oa":true,"landing_page_url":"https://doi.org/10.1155/crrh/9165820","pdf_url":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1155/crrh/9165820","source":{"id":"https://openalex.org/S2764880244","display_name":"Case Reports in Rheumatology","issn_l":"2090-6889","issn":["2090-6889","2090-6897"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj"],"host_organization":"https://openalex.org/P4310319869","host_organization_name":"Hindawi Publishing Corporation","host_organization_lineage":["https://openalex.org/P4310319869","https://openalex.org/P4310320595"],"host_organization_lineage_names":["Hindawi Publishing Corporation","Wiley"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Case Reports in Rheumatology","raw_type":"journal-article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":1.0}],"study_designs":[{"display_name":"Case Report","id":"https://openalex.org/study-designs/case-report"}],"awards":[],"funders":[],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W7222865576.pdf","grobid_xml":"https://content.openalex.org/works/W7222865576.grobid-xml"},"referenced_works_count":10,"referenced_works":["https://openalex.org/W2013522127","https://openalex.org/W2162251899","https://openalex.org/W2413707759","https://openalex.org/W2593544935","https://openalex.org/W2936734228","https://openalex.org/W3045499022","https://openalex.org/W3131959715","https://openalex.org/W4391542192","https://openalex.org/W4396800799","https://openalex.org/W7161307930"],"related_works":["https://openalex.org/W2747246083","https://openalex.org/W4380323595","https://openalex.org/W4406366476","https://openalex.org/W4319063109","https://openalex.org/W2978671365","https://openalex.org/W4401085681","https://openalex.org/W4300082159","https://openalex.org/W4232835791","https://openalex.org/W4322773786","https://openalex.org/W2915602374"],"abstract_inverted_index":{"Hemophagocytic":[0],"lymphohistiocytosis":[1],"(HLH)":[2],"is":[3,55,178],"a":[4,29,60,84,110,181,193],"life-threatening,":[5],"systemic":[6],"inflammatory":[7],"syndrome.":[8],"Cases":[9],"without":[10],"an":[11,122],"identifiable":[12],"acquired":[13],"trigger":[14],"are":[15],"referred":[16],"to":[17,41,48,51,58,199],"as":[18],"secondary":[19,80],"HLH.":[20,81],"In":[21],"this":[22],"report,":[23],"we":[24],"describe":[25],"the":[26,68,77,187],"case":[27,185],"of":[28,36,62,79,128,190],"24":[30],"year-old":[31],"woman":[32],"whose":[33],"underlying":[34],"cause":[35],"HLH":[37,172,196],"was":[38,45,97],"particularly":[39],"difficult":[40],"determine.":[42],"Her":[43],"condition":[44,148],"initially":[46],"suspected":[47],"be":[49,59],"due":[50,198],"lamotrigine":[52],"use":[53,189],"but":[54,176],"now":[56],"thought":[57],"result":[61],"undifferentiated":[63,200],"connective":[64,201],"tissue":[65,202],"disease.":[66,203],"During":[67],"patient's":[69],"prolonged":[70],"and":[71,101,113,141],"complex":[72],"hospital":[73],"stay,":[74],"she":[75,150,157],"received":[76],"diagnosis":[78],"Discontinuing":[82],"lamotrigine,":[83],"drug":[85],"that":[86],"rarely":[87],"causes":[88],"HLH,":[89],"did":[90,131],"not":[91,132],"improve":[92],"her":[93],"condition.":[94],"The":[95,143],"patient":[96,144,194],"tested":[98],"for":[99],"infectious":[100],"malignant":[102],"conditions":[103],"with":[104,117,153,155,195],"negative":[105,118],"results.":[106],"Further":[107],"workup":[108],"revealed":[109],"positive":[111],"ANA":[112],"bilateral":[114],"MCP":[115],"tenosynovitis,":[116],"specific":[119],"antibodies,":[120],"suggesting":[121],"undefined":[123],"rheumatologic":[124],"process.":[125],"A":[126],"number":[127],"conventional":[129],"therapies":[130],"show":[133],"clear":[134],"benefit,":[135],"including":[136],"etoposide,":[137],"high-dose":[138],"steroids,":[139],"rituximab,":[140],"plasmapheresis.":[142],"remained":[145],"in":[146,170,173,192],"critical":[147],"until":[149],"began":[151],"treatment":[152],"ruxolitinib,":[154],"which":[156],"remains":[158],"stable":[159],"more":[160],"than":[161],"5":[162],"years":[163],"later.":[164],"Ruxolitinib":[165],"has":[166],"shown":[167],"some":[168],"success":[169],"treating":[171],"preliminary":[174],"studies,":[175],"it":[177],"still":[179],"considered":[180],"salvage":[182],"therapy.":[183],"This":[184],"illustrates":[186],"successful":[188],"ruxolitinib":[191],"likely":[197]},"counts_by_year":[],"updated_date":"2026-10-29T09:56:32.521200","created_date":"2026-10-19T00:00:00"}
