{"id":"https://openalex.org/W7224874366","doi":"https://doi.org/10.1016/j.heliyon.2026.e45635","title":"Efficacy of JAK-inhibition in therapy-refractory full-house membranous glomerulopathy with type I interferon-pathway activation in the setting of anti-synthetase syndrome: a case report","display_name":"Efficacy of JAK-inhibition in therapy-refractory full-house membranous glomerulopathy with type I interferon-pathway activation in the setting of anti-synthetase syndrome: a case report","publication_year":2026,"publication_date":"2026-10-30","ids":{"openalex":"https://openalex.org/W7224874366","doi":"https://doi.org/10.1016/j.heliyon.2026.e45635"},"language":"en","primary_location":{"id":"doi:10.1016/j.heliyon.2026.e45635","doi":"https://doi.org/10.1016/j.heliyon.2026.e45635","is_oa":true,"landing_page_url":"https://doi.org/10.1016/j.heliyon.2026.e45635","pdf_url":null,"source":{"id":"https://openalex.org/S2898612692","display_name":"Heliyon","issn_l":"2405-8440","issn":["2405-8440"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","ki-jl-1","norway-1","poland-40","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Heliyon","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","doaj"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://doi.org/10.1016/j.heliyon.2026.e45635","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5160217464","display_name":"D. Verhoeven","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I4210151833","display_name":"Amsterdam University Medical Centers","ror":"https://ror.org/05grdyy37","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I4405253132","display_name":"Amsterdam institute for Immunology and Infectious Diseases","ror":"https://ror.org/00bcn1057","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I4405253132"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"D. Verhoeven","raw_affiliation_strings":["Department of Experimental Immunology, Amsterdam Institute for Immunology & Infectious Diseases, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Immunology, Amsterdam Institute for Immunology & Infectious Diseases, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I4210151833","https://openalex.org/I4405253132","https://openalex.org/I887064364"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5160164826","display_name":"J.J.T.H. Roelofs","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I4210151833","display_name":"Amsterdam University Medical Centers","ror":"https://ror.org/05grdyy37","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"J.J.T.H. Roelofs","raw_affiliation_strings":["Department Pathology, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department Pathology, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I4210151833","https://openalex.org/I887064364"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5022671153","display_name":"Marc  L. Hilhorst","orcid":"https://orcid.org/0000-0003-3712-7171","observed_orcids":["https://orcid.org/0000-0003-3712-7171"]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I4405253132","display_name":"Amsterdam institute for Immunology and Infectious Diseases","ror":"https://ror.org/00bcn1057","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I4405253132"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"M.L. Hilhorst","raw_affiliation_strings":["Department of Medicine and Department of Nephrology, Amsterdam Institute for Immunology & Infectious Diseases, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine and Department of Nephrology, Amsterdam Institute for Immunology & Infectious Diseases, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I4405253132","https://openalex.org/I887064364"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5011278499","display_name":"Arjan J. Kwakernaak","orcid":"https://orcid.org/0000-0002-5155-6581","observed_orcids":["https://orcid.org/0000-0002-5155-6581"]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I4405253132","display_name":"Amsterdam institute for Immunology and Infectious Diseases","ror":"https://ror.org/00bcn1057","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I4405253132"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":true,"raw_author_name":"A.J. Kwakernaak","raw_affiliation_strings":["Department of Medicine and Department of Nephrology, Amsterdam Institute for Immunology & Infectious Diseases, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","Department of Medicine, Section Clinical Immunology & Allergy, Amsterdam Institute for Immunology & Infectious Diseases, Center for Immunodeficiency and Immune Dysregulation Amsterdam, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":"https://orcid.org/0000-0002-5155-6581","affiliations":[{"raw_affiliation_string":"Department of Medicine and Department of Nephrology, Amsterdam Institute for Immunology & Infectious Diseases, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I4405253132","https://openalex.org/I887064364"]},{"raw_affiliation_string":"Department of Medicine, Section Clinical Immunology & Allergy, Amsterdam Institute for Immunology & Infectious Diseases, Center for Immunodeficiency and Immune Dysregulation Amsterdam, Amsterdam University Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":4,"corresponding_author_ids":["https://openalex.org/A5011278499"],"corresponding_institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I4405253132","https://openalex.org/I887064364"],"apc_list":{"value":2270,"currency":"USD","value_usd":2270},"apc_paid":{"value":2270,"currency":"USD","value_usd":2270},"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.85460404,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"12","issue":"16","first_page":"e45635","last_page":"e45635"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11029","display_name":"Renal Diseases and Glomerulopathies","score":0.9136000275611877,"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"}},"topics":[{"id":"https://openalex.org/T11029","display_name":"Renal Diseases and Glomerulopathies","score":0.9136000275611877,"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/T11724","display_name":"Inflammatory Myopathies and Dermatomyositis","score":0.0544000007212162,"subfield":{"id":"https://openalex.org/subfields/2713","display_name":"Epidemiology"},"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/T10697","display_name":"Vasculitis and related conditions","score":0.015300000086426735,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/membranous-nephropathy","display_name":"membranous nephropathy","score":0.9769999980926514},{"id":"https://openalex.org/keywords/antisynthetase-syndrome","display_name":"antisynthetase syndrome","score":0.9300000071525574},{"id":"https://openalex.org/keywords/jak-inhibition","display_name":"JAK inhibition","score":0.9559999704360962},{"id":"https://openalex.org/keywords/tofacitinib","display_name":"tofacitinib","score":1.0},{"id":"https://openalex.org/keywords/nephrotic-syndrome","display_name":"nephrotic syndrome","score":0.8889999985694885},{"id":"https://openalex.org/keywords/inflammatory-myositis","display_name":"inflammatory myositis","score":0.7870000004768372},{"id":"https://openalex.org/keywords/autoimmune-kidney-disease","display_name":"autoimmune kidney disease","score":0.5270000100135803},{"id":"https://openalex.org/keywords/jak-stat-signaling","display_name":"JAK/STAT signaling","score":0.7689999938011169}],"concepts":[{"id":"https://openalex.org/C2778930706","wikidata":"https://www.wikidata.org/wiki/Q504790","display_name":"Nephrotic syndrome","level":2,"score":0.8309000134468079},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8014000058174133},{"id":"https://openalex.org/C2780199430","wikidata":"https://www.wikidata.org/wiki/Q3347169","display_name":"Glomerulopathy","level":4,"score":0.6948999762535095},{"id":"https://openalex.org/C128057223","wikidata":"https://www.wikidata.org/wiki/Q420566","display_name":"Calcineurin","level":3,"score":0.51419997215271},{"id":"https://openalex.org/C2780942790","wikidata":"https://www.wikidata.org/wiki/Q372016","display_name":"Pathogenesis","level":2,"score":0.5069000124931335},{"id":"https://openalex.org/C2780091579","wikidata":"https://www.wikidata.org/wiki/Q9377","display_name":"Kidney","level":2,"score":0.5024999976158142},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.4999000132083893},{"id":"https://openalex.org/C2780368995","wikidata":"https://www.wikidata.org/wiki/Q605006","display_name":"Glomerulonephritis","level":3,"score":0.49219998717308044},{"id":"https://openalex.org/C159641895","wikidata":"https://www.wikidata.org/wiki/Q108377937","display_name":"Renal function","level":2,"score":0.451200008392334},{"id":"https://openalex.org/C2778653478","wikidata":"https://www.wikidata.org/wiki/Q1054718","display_name":"Kidney disease","level":2,"score":0.43149998784065247},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.4300000071525574},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.424699991941452},{"id":"https://openalex.org/C54847362","wikidata":"https://www.wikidata.org/wiki/Q177635","display_name":"Nephrology","level":2,"score":0.3662000000476837},{"id":"https://openalex.org/C2778236600","wikidata":"https://www.wikidata.org/wiki/Q592324","display_name":"Immunofluorescence","level":3,"score":0.35580000281333923},{"id":"https://openalex.org/C2775946357","wikidata":"https://www.wikidata.org/wiki/Q1713037","display_name":"Membranous nephropathy","level":4,"score":0.33410000801086426},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3149999976158142},{"id":"https://openalex.org/C2777146197","wikidata":"https://www.wikidata.org/wiki/Q138912","display_name":"Podocyte","level":4,"score":0.3147999942302704},{"id":"https://openalex.org/C2777747828","wikidata":"https://www.wikidata.org/wiki/Q1323002","display_name":"Minimal change disease","level":5,"score":0.2919999957084656},{"id":"https://openalex.org/C2779561371","wikidata":"https://www.wikidata.org/wiki/Q570197","display_name":"Proteinuria","level":3,"score":0.2865999937057495},{"id":"https://openalex.org/C2778415529","wikidata":"https://www.wikidata.org/wiki/Q1435223","display_name":"Focal segmental glomerulosclerosis","level":4,"score":0.2757999897003174},{"id":"https://openalex.org/C2779147130","wikidata":"https://www.wikidata.org/wiki/Q2074424","display_name":"Nephritic syndrome","level":4,"score":0.2705000042915344}],"mesh":[],"locations_count":2,"locations":[{"id":"doi:10.1016/j.heliyon.2026.e45635","doi":"https://doi.org/10.1016/j.heliyon.2026.e45635","is_oa":true,"landing_page_url":"https://doi.org/10.1016/j.heliyon.2026.e45635","pdf_url":null,"source":{"id":"https://openalex.org/S2898612692","display_name":"Heliyon","issn_l":"2405-8440","issn":["2405-8440"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","ki-jl-1","norway-1","poland-40","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Heliyon","raw_type":"journal-article"},{"id":"pmh:oai:doaj.org/article:04d4cef079c74650a770fa741cbf111b","doi":null,"is_oa":false,"landing_page_url":"https://doaj.org/article/04d4cef079c74650a770fa741cbf111b","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":"Heliyon, Vol 12, Iss 16, Pp e45635- (2026)","raw_type":"article"}],"best_oa_location":{"id":"doi:10.1016/j.heliyon.2026.e45635","doi":"https://doi.org/10.1016/j.heliyon.2026.e45635","is_oa":true,"landing_page_url":"https://doi.org/10.1016/j.heliyon.2026.e45635","pdf_url":null,"source":{"id":"https://openalex.org/S2898612692","display_name":"Heliyon","issn_l":"2405-8440","issn":["2405-8440"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","ki-jl-1","norway-1","poland-40","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Heliyon","raw_type":"journal-article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":0.98580002784729}],"study_designs":[{"display_name":"Case Report","id":"https://openalex.org/study-designs/case-report"}],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":12,"referenced_works":["https://openalex.org/W1548480696","https://openalex.org/W2003390541","https://openalex.org/W2081031295","https://openalex.org/W2244306209","https://openalex.org/W2606492431","https://openalex.org/W2792531399","https://openalex.org/W3165822352","https://openalex.org/W3169626318","https://openalex.org/W3212528209","https://openalex.org/W4365518056","https://openalex.org/W4393199241","https://openalex.org/W4403600643"],"related_works":["https://openalex.org/W3210572125","https://openalex.org/W2520826912","https://openalex.org/W4399303207","https://openalex.org/W7163352436","https://openalex.org/W4408740715","https://openalex.org/W1981757422","https://openalex.org/W2588151570","https://openalex.org/W2578968431","https://openalex.org/W4396989966","https://openalex.org/W2153067428"],"abstract_inverted_index":{"We":[0,60],"report":[1],"a":[2,16,145],"patient":[3,31,48],"with":[4,7,15,85,99],"nephrotic":[5,113],"syndrome":[6,114,123],"preserved":[8],"kidney":[9,72],"function":[10],"due":[11,56],"to":[12,34,57],"membranous":[13],"glomerulopathy":[14],"full-house":[17],"immunofluorescence":[18],"pattern,":[19],"in":[20,70,78,92,108,121,130,149],"the":[21,30,47,71,79,82,86,90,93],"setting":[22],"of":[23,29,37,63,81,89,95,111,128],"anti-synthetase":[24,122],"syndrome.":[25],"The":[26],"glomerular":[27,131,152],"disease":[28,98,117,132],"was":[32],"resistant":[33],"multiple":[35],"lines":[36],"treatment":[38,147],"including":[39],"corticosteroids,":[40],"anti-CD20":[41],"therapy":[42],"and":[43,46,54,126],"calcineurin":[44],"inhibition,":[45],"suffered":[49],"from":[50],"serious":[51],"treatment-related":[52],"morbidity":[53],"complications":[55],"ongoing":[58],"nephrosis.":[59],"found":[61],"evidence":[62],"an":[64],"increased":[65],"type":[66],"I":[67],"interferon":[68],"activation":[69],"tissue":[73],"specimen":[74],"as":[75,77],"well":[76],"circulation":[80],"patient,":[83],"consistent":[84],"pivotal":[87],"role":[88],"JAK-STAT-pathway":[91],"pathogenesis":[94],"systemic":[96],"auto-immune":[97],"inflammatory":[100,150],"myositis.":[101],"Using":[102],"tofacitinib,":[103],"we":[104],"initiated":[105],"JAK-inhibition":[106,129,142],"resulting":[107],"complete":[109],"response":[110],"his":[112],"(and":[115],"extra-renal":[116],"activity).":[118],"Kidney":[119],"involvement":[120],"is":[124],"rare":[125],"efficacy":[127],"has":[133],"not":[134],"been":[135],"reported":[136],"before.":[137],"Our":[138],"case":[139],"suggests":[140],"that":[141],"may":[143],"be":[144],"promising":[146],"modality":[148],"myositis-associated":[151],"disease.":[153]},"counts_by_year":[],"updated_date":"2026-11-03T10:50:19.383937","created_date":"2026-10-31T00:00:00"}