{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T21:53:47Z","timestamp":1790286827894,"version":"4.1.0"},"reference-count":45,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,11,5]],"date-time":"2024-11-05T00:00:00Z","timestamp":1730764800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100012112","name":"Swedish Orphan Biovitrum","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012112","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Rheumatologic disease\u2013associated hemophagocytic lymphohistiocytosis (HLH), a rare, life\u2010threatening, systemic hyperinflammatory syndrome, occurs as a complication of underlying rheumatologic disease. Real\u2010world evidence is lacking on emapalumab, a fully human monoclonal antibody that neutralizes the proinflammatory cytokine interferon\u2010\u03b3, approved for treating patients with primary HLH.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>REAL\u2010HLH, a retrospective medical chart review study conducted across 33 US hospitals, assessed real\u2010world treatment patterns and outcomes in patients with HLH treated with one or more dose of emapalumab between November 20, 2018, and October 31, 2021. Data are presented for the subset of patients with rheumatologic disease\u2013associated HLH.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifteen of 105 patients (14.3%) had rheumatologic disease\u2013associated HLH. Of these, nine (60.0%) had systemic juvenile idiopathic arthritis, and one (6.7%) had adult\u2010onset Still disease. Median (range) age at HLH diagnosis was 5 (0.9\u201339) years. Most patients (9 of 15; 60.0%) initiated emapalumab in an intensive care unit. Emapalumab was most frequently initiated for treating refractory or recurrent (10 of 15; 66.7%) disease. Most patients received HLH\u2010related therapies before (10 of 15; 66.7%) and concurrently with (15 of 15; 100.0%) emapalumab. Emapalumab\u2010containing regimens stabilized or achieved physician\u2010determined normalization of most laboratory parameters, including absolute neutrophil count and absolute lymphocyte count (13 of 14; 92.9%), chemokine ligand 9 (9 of 11; 81.8%), and platelets and alanine transaminase (11 of 14; 78.6%), and reduced glucocorticoid dose by 80%. Overall survival and 12\u2010month survival probability from emapalumab initiation were 86.7%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Emapalumab\u2010containing regimens stabilized or normalized most key laboratory parameters, reduced glucocorticoid dose, and were associated with low disease\u2010related mortality, thereby demonstrating potential benefits in patients with rheumatologic disease\u2013associated HLH.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42985","type":"journal-article","created":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T01:44:03Z","timestamp":1725846243000},"page":"226-238","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Emapalumab Treatment in Patients With Rheumatologic Disease\u2013Associated Hemophagocytic Lymphohistiocytosis in the United States: A Retrospective Medical Chart Review Study"],"prefix":"10.1002","volume":"77","author":[{"given":"Shanmuganathan","family":"Chandrakasan","sequence":"first","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University  Atlanta Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carl E.","family":"Allen","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine  Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepika","family":"Bhatla","sequence":"additional","affiliation":[{"name":"Saint Louis University  Saint Louis Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Carter","sequence":"additional","affiliation":[{"name":"Oregon Health and Science University  Portland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"May","family":"Chien","sequence":"additional","affiliation":[{"name":"Lucile Packard Children's Hospital at Stanford University  Palo Alto California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Cooper","sequence":"additional","affiliation":[{"name":"Bellflower Medical Center  Bellflower California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Draper","sequence":"additional","affiliation":[{"name":"Saint Louis University  Saint Louis Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olive S.","family":"Eckstein","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine  Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rabi","family":"Hanna","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Children's Hospital  Cleveland Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Allyson","family":"Hays","sequence":"additional","affiliation":[{"name":"Children's Mercy Hospital  Kansas City Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle L.","family":"Hermiston","sequence":"additional","affiliation":[{"name":"University of California  San Francisco"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley P.","family":"Hinson","sequence":"additional","affiliation":[{"name":"Atrium Health Levine Children's Hospital  Charlotte North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia M.","family":"Hobday","sequence":"additional","affiliation":[{"name":"University of Minnesota Medical School  Minneapolis"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael S.","family":"Isakoff","sequence":"additional","affiliation":[{"name":"Connecticut Children's Medical Center  Hartford"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael B.","family":"Jordan","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine  Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer W.","family":"Leiding","sequence":"additional","affiliation":[{"name":"Johns Hopkins University, Baltimore, Maryland, and Johns Hopkins All Children's Hospital  St. Petersburg Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renee","family":"Modica","sequence":"additional","affiliation":[{"name":"University of Florida Health Shands Children's Hospital  Gainesville"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taizo A.","family":"Nakano","sequence":"additional","affiliation":[{"name":"Children's Hospital Colorado  Aurora"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abiola","family":"Oladapo","sequence":"additional","affiliation":[{"name":"Sobi, Inc.  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