{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T06:58:37Z","timestamp":1772953117625,"version":"3.50.1"},"reference-count":43,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2019,6,1]],"date-time":"2019-06-01T00:00:00Z","timestamp":1559347200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,6,1]],"date-time":"2019-06-01T00:00:00Z","timestamp":1559347200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 AR053628"],"award-info":[{"award-number":["R01 AR053628"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 AR066551"],"award-info":[{"award-number":["R01 AR066551"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Center for Investigation of Membrane Excitability Diseases"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Autoimmunity"],"published-print":{"date-parts":[[2019,6]]},"DOI":"10.1016\/j.jaut.2019.02.005","type":"journal-article","created":{"date-parts":[[2019,3,15]],"date-time":"2019-03-15T02:51:14Z","timestamp":1552618274000},"page":"62-74","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":31,"special_numbering":"C","title":["Plc\u03b32\/Tmem178 dependent pathway in myeloid cells modulates the pathogenesis of cytokine storm syndrome"],"prefix":"10.1016","volume":"100","author":[{"given":"Sahil","family":"Mahajan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Corinne E.","family":"Decker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhengfeng","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Deborah","family":"Veis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth D.","family":"Mellins","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberta","family":"Faccio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaut.2019.02.005_bib1","first-page":"329","article-title":"Making sense of the cytokine storm: a conceptual framework for understanding, diagnosing, and treating hemophagocytic syndromes","volume":"59","author":"Canna","year":"2012","journal-title":"Pediatr. Clin."},{"key":"10.1016\/j.jaut.2019.02.005_bib2","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1038\/cmi.2015.74","article-title":"The cytokine storm of severe influenza and development of immunomodulatory therapy","volume":"13","author":"Liu","year":"2016","journal-title":"Cell. Mol. Immunol."},{"key":"10.1016\/j.jaut.2019.02.005_bib3","doi-asserted-by":"crossref","first-page":"2918","DOI":"10.1182\/blood.V78.11.2918.2918","article-title":"Hypercytokinemia in familial hemophagocytic lymphohistiocytosis","volume":"78","author":"Henter","year":"1991","journal-title":"Blood"},{"key":"10.1016\/j.jaut.2019.02.005_bib4","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1111\/j.1600-065X.2008.00626.x","article-title":"Role of cytokines in rheumatoid arthritis: an education in pathophysiology and therapeutics","volume":"223","author":"Feldmann","year":"2008","journal-title":"Immunol. Rev."},{"key":"10.1016\/j.jaut.2019.02.005_bib5","doi-asserted-by":"crossref","first-page":"2709","DOI":"10.1182\/blood-2011-10-384388","article-title":"B-cell depletion and remissions of malignancy along with cytokine-associated toxicity in a clinical trial of anti-CD19 chimeric-antigen-receptor-transduced T cells","volume":"119","author":"Kochenderfer","year":"2012","journal-title":"Blood"},{"key":"10.1016\/j.jaut.2019.02.005_bib6","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1126\/scitranslmed.3008226","article-title":"Efficacy and toxicity management of 19-28z CAR T cell therapy in B cell acute lymphoblastic leukemia","volume":"6","author":"Davila","year":"2014","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.jaut.2019.02.005_bib7","doi-asserted-by":"crossref","first-page":"630","DOI":"10.1016\/S0022-3476(96)70140-3","article-title":"Macrophage activation syndrome in systemic juvenile rheumatoid arthritis","volume":"129","author":"Grom","year":"1996","journal-title":"J. Pediatr."},{"key":"10.1016\/j.jaut.2019.02.005_bib8","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1136\/adc.85.5.421","article-title":"Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders","volume":"85","author":"Sawhney","year":"2001","journal-title":"Arch. Dis. Child."},{"key":"10.1016\/j.jaut.2019.02.005_bib9","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/j.berh.2014.03.002","article-title":"Macrophage activation syndrome and cytokine-directed therapies","volume":"28","author":"Schulert","year":"2014","journal-title":"Best Pract. Res. Clin. Rheumatol."},{"key":"10.1016\/j.jaut.2019.02.005_bib10","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/nrrheum.2015.179","article-title":"Macrophage activation syndrome in the era of biologic therapy","volume":"12","author":"Grom","year":"2016","journal-title":"Nat. Rev. Rheumatol."},{"key":"10.1016\/j.jaut.2019.02.005_bib11","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1182\/blood-2003-10-3413","article-title":"An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder","volume":"104","author":"Jordan","year":"2004","journal-title":"Blood"},{"key":"10.1016\/j.jaut.2019.02.005_bib12","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1146\/annurev-med-041610-134208","article-title":"Familial and acquired hemophagocytic lymphohistiocytosis","volume":"63","author":"Janka","year":"2012","journal-title":"Annu. Rev. Med."},{"key":"10.1016\/j.jaut.2019.02.005_bib13","doi-asserted-by":"crossref","first-page":"1666","DOI":"10.1182\/blood-2015-12-684399","article-title":"Janus kinase inhibition lessens inflammation and ameliorates disease in murine models of hemophagocytic lymphohistiocytosis","volume":"127","author":"Das","year":"2016","journal-title":"Blood"},{"key":"10.1016\/j.jaut.2019.02.005_bib14","doi-asserted-by":"crossref","first-page":"5184","DOI":"10.1182\/blood-2013-04-495309","article-title":"Perforin deficiency impairs a critical immunoregulatory loop involving murine CD8(+) T cells and dendritic cells","volume":"121","author":"Terrell","year":"2013","journal-title":"Blood"},{"key":"10.1016\/j.jaut.2019.02.005_bib15","doi-asserted-by":"crossref","first-page":"3793","DOI":"10.1002\/art.22981","article-title":"Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome","volume":"56","author":"Fall","year":"2007","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.jaut.2019.02.005_bib16","doi-asserted-by":"crossref","first-page":"769","DOI":"10.3899\/jrheum.100655","article-title":"Association of IRF5 polymorphisms with susceptibility to macrophage activation syndrome in patients with juvenile idiopathic arthritis","volume":"38","author":"Yanagimachi","year":"2011","journal-title":"J. Rheumatol."},{"key":"10.1016\/j.jaut.2019.02.005_bib17","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1038\/ni.1990","article-title":"IRF5 promotes inflammatory macrophage polarization and TH1-TH17 responses","volume":"12","author":"Krausgruber","year":"2011","journal-title":"Nat. Immunol."},{"key":"10.1016\/j.jaut.2019.02.005_bib18","doi-asserted-by":"crossref","first-page":"1203","DOI":"10.1084\/jem.20102538","article-title":"Hemophagocytosis causes a consumptive anemia of inflammation","volume":"208","author":"Zoller","year":"2011","journal-title":"J. Exp. Med."},{"key":"10.1016\/j.jaut.2019.02.005_bib19","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1182\/blood-2004-08-2997","article-title":"Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-gamma-producing lymphocytes and IL-6- and TNF-alpha-producing macrophages","volume":"105","author":"Billiau","year":"2005","journal-title":"Blood"},{"key":"10.1016\/j.jaut.2019.02.005_bib20","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1038\/ng.3089","article-title":"An activating NLRC4 inflammasome mutation causes autoinflammation with recurrent macrophage activation syndrome","volume":"46","author":"Canna","year":"2014","journal-title":"Nat. Genet."},{"key":"10.1016\/j.jaut.2019.02.005_bib21","doi-asserted-by":"crossref","first-page":"10944","DOI":"10.1073\/pnas.1524487113","article-title":"TLR9-mediated inflammation drives a Ccr2-independent peripheral monocytosis through enhanced extramedullary monocytopoiesis","volume":"113","author":"Weaver","year":"2016","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jaut.2019.02.005_bib22","doi-asserted-by":"crossref","first-page":"2264","DOI":"10.1172\/JCI43157","article-title":"Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice","volume":"121","author":"Behrens","year":"2011","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.jaut.2019.02.005_bib23","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1038\/s41591-018-0036-4","article-title":"Monocyte-derived IL-1 and IL-6 are differentially required for cytokine-release syndrome and neurotoxicity due to CAR T cells","volume":"24","author":"Norelli","year":"2018","journal-title":"Nat. Med."},{"key":"10.1016\/j.jaut.2019.02.005_bib24","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1038\/s41591-018-0041-7","article-title":"CAR T cell-induced cytokine release syndrome is mediated by macrophages and abated by IL-1 blockade","volume":"24","author":"Giavridis","year":"2018","journal-title":"Nat. Med."},{"key":"10.1016\/j.jaut.2019.02.005_bib25","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1038\/ni.3710","article-title":"Opposing macrophage polarization programs show extensive epigenomic and transcriptional cross-talk","volume":"18","author":"Piccolo","year":"2017","journal-title":"Nat. Immunol."},{"key":"10.1016\/j.jaut.2019.02.005_bib26","doi-asserted-by":"crossref","first-page":"15654","DOI":"10.1073\/pnas.1511285112","article-title":"Tmem178 acts in a novel negative feedback loop targeting NFATc1 to regulate bone mass","volume":"112","author":"Decker","year":"2015","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jaut.2019.02.005_bib27","doi-asserted-by":"crossref","first-page":"1738","DOI":"10.4049\/jimmunol.165.4.1738","article-title":"Cutting edge: essential role of phospholipase C-gamma 2 in B cell development and function","volume":"165","author":"Hashimoto","year":"2000","journal-title":"J. Immunol."},{"key":"10.1016\/j.jaut.2019.02.005_bib28","doi-asserted-by":"crossref","first-page":"e8909","DOI":"10.1371\/journal.pone.0008909","article-title":"Phospholipase C gamma 2 is critical for development of a murine model of inflammatory arthritis by affecting actin dynamics in dendritic cells","volume":"5","author":"Cremasco","year":"2010","journal-title":"PLoS One"},{"key":"10.1016\/j.jaut.2019.02.005_bib29","doi-asserted-by":"crossref","first-page":"2712","DOI":"10.1002\/art.23757","article-title":"Vav\/Phospholipase Cgamma2-mediated control of a neutrophil-dependent murine model of rheumatoid arthritis","volume":"58","author":"Cremasco","year":"2008","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.jaut.2019.02.005_bib30","doi-asserted-by":"crossref","first-page":"1430","DOI":"10.1182\/blood-2017-06-789552","article-title":"Unopposed IL-18 signaling leads to severe TLR9-induced macrophage activation syndrome in mice","volume":"131","author":"Girard-Guyonvarc'h","year":"2018","journal-title":"Blood"},{"key":"10.1016\/j.jaut.2019.02.005_bib31","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1084\/jem.177.2.523","article-title":"Interferon gamma inhibits interleukin 10 production by monocytes","volume":"177","author":"Chomarat","year":"1993","journal-title":"J. Exp. Med."},{"key":"10.1016\/j.jaut.2019.02.005_bib32","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.immuni.2014.06.008","article-title":"Macrophage activation and polarization: nomenclature and experimental guidelines","volume":"41","author":"Murray","year":"2014","journal-title":"Immunity"},{"key":"10.1016\/j.jaut.2019.02.005_bib33","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1146\/annurev-physiol-022516-034339","article-title":"Macrophage polarization","volume":"79","author":"Murray","year":"2017","journal-title":"Annu. Rev. Physiol."},{"key":"10.1016\/j.jaut.2019.02.005_bib34","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1172\/JCI59643","article-title":"Macrophage plasticity and polarization: in vivo veritas","volume":"122","author":"Sica","year":"2012","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.jaut.2019.02.005_bib35","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1016\/j.immuni.2005.10.001","article-title":"Macrophage polarization comes of age","volume":"23","author":"Mantovani","year":"2005","journal-title":"Immunity"},{"key":"10.1016\/j.jaut.2019.02.005_bib36","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/j.clim.2011.12.008","article-title":"Alternative activation in systemic juvenile idiopathic arthritis monocytes","volume":"142","author":"Macaubas","year":"2012","journal-title":"Clin. Immunol."},{"key":"10.1016\/j.jaut.2019.02.005_bib37","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1002\/art.40071","article-title":"Review: cytokine storm syndrome: looking toward the precision medicine era","volume":"69","author":"Behrens","year":"2017","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.jaut.2019.02.005_bib38","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/j.clim.2015.06.005","article-title":"Interleukin-18 for predicting the development of macrophage activation syndrome in systemic juvenile idiopathic arthritis","volume":"160","author":"Shimizu","year":"2015","journal-title":"Clin. Immunol."},{"key":"10.1016\/j.jaut.2019.02.005_bib39","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1002\/art.22416","article-title":"The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis","volume":"56","author":"Bleesing","year":"2007","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.jaut.2019.02.005_bib40","doi-asserted-by":"crossref","first-page":"302","DOI":"10.3109\/s10165-009-0152-6","article-title":"The role of heme oxygenase-1 in systemic-onset juvenile idiopathic arthritis","volume":"19","author":"Takahashi","year":"2009","journal-title":"Mod. Rheumatol."},{"key":"10.1016\/j.jaut.2019.02.005_bib41","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/j.ajhg.2012.08.006","article-title":"A hypermorphic missense mutation in PLCG2, encoding phospholipase Cgamma2, causes a dominantly inherited autoinflammatory disease with immunodeficiency","volume":"91","author":"Zhou","year":"2012","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.jaut.2019.02.005_bib42","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1056\/NEJMoa1102140","article-title":"Cold urticaria, immunodeficiency, and autoimmunity related to PLCG2 deletions","volume":"366","author":"Ombrello","year":"2012","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.jaut.2019.02.005_bib43","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/j.immuni.2005.01.018","article-title":"Autoimmunity and inflammation due to a gain-of-function mutation in phospholipase C gamma 2 that specifically increases external Ca2+ entry","volume":"22","author":"Yu","year":"2005","journal-title":"Immunity"}],"container-title":["Journal of Autoimmunity"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896841118306978?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896841118306978?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T08:20:17Z","timestamp":1760257217000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0896841118306978"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,6]]},"references-count":43,"alternative-id":["S0896841118306978"],"URL":"https:\/\/doi.org\/10.1016\/j.jaut.2019.02.005","relation":{},"ISSN":["0896-8411"],"issn-type":[{"value":"0896-8411","type":"print"}],"subject":[],"published":{"date-parts":[[2019,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Plc\u03b32\/Tmem178 dependent pathway in myeloid cells modulates the pathogenesis of cytokine storm syndrome","name":"articletitle","label":"Article Title"},{"value":"Journal of Autoimmunity","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaut.2019.02.005","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}]}}