Edward M. Behrens, MD
Locations: Main Building
About Edward M. Behrens, MD
Dr. Edward Behrens received his undergraduate training at Johns Hopkins University, where he majored in biology. He attended medical school at the University of Pennsylvania, where he received the prestigious Howard Hughes Medical Institute's Medical Student Research Fellowship to train in the laboratory of Dr. Philip Cohen. After medical school, Ed completed a Pediatrics Residency and Pediatric Rheumatology Fellowship at the Children’s Hospital of Philadelphia. He completed two post-doctoral fellowships in the laboratories of Stefania Gallucci and Gary Koretzky, after which he joined the faculty of the Division of Rheumatology at CHOP in 2009.
He became Chief of the Division of Pediatric Rheumatology in 2014 and currently holds the Joseph Lee Hollander Chair in Pediatric Rheumatology. Ed’s research interest is the pathogenesis and treatment of cytokine storm syndromes, including the hemophagocytic syndromes Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS). These are uniquely pediatric immunologic conditions that result in severe systemic inflammation and death if unrecognized and untreated. Despite current therapies, mortality remains high for these conditions, hovering around 50% of patients. Ed has developed a novel murine model of MAS, which has enabled careful immunologic dissection of disease mediators. More recently, Ed has begun to work on the role of non-immune cell mediators in MAS and other diseases. His work has been recognized with many awards from the American College of Rheumatology, the Histiocyte Society, and the Arthritis Foundation. He is the founding member of the International MAS Study Group and has given lectures internationally on MAS/HLH. He is an elected member of the American Society for Clinical Investigation.
Dr. Behrens’s interest in global health stems from the universal truth that immune system problems affect children everywhere. He has recently developed an interest in how cytokine storms lead to morbidity and mortality during Dengue virus infections, a global health problem affecting 400 million people, predominantly in tropical regions. In conjunction with Dr. Andrew Steenhoff and collaborators in the Dominican Republic, he has an active research program to study the immunology of Dengue infection in children to develop novel therapies that will hopefully save the lives of those with the most severe outcomes of this infection.
Titles
Chief, Division of Rheumatology
Joseph Lee Hollander Chair in Pediatric Rheumatology
Assistant Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania
Certifications
Pediatric Rheumatology – American Board of Pediatrics
Pediatrics – American Board of Pediatrics
Awards and Honors
2022, Philadelphia Magazine's Top Doctors in Pediatric Rheumatology
2014, Joseph L. Hollander Endowed Chair in Pediatric Rheumatology, University of Pennsylvania
2013, American Society for Clinical Investigators – Young Physician-Scientist Award
2012, Nezelof Award in Basic Science – Histiocyte Society
2012, Stuart McKracken Award for Research – Arthritis Foundation
2011, Nezelof Award in Basic Science – Histiocyte Society
2011, Arthritis Foundation Profession Education Award
2010, University of Pennsylvania Department of Medicine Austrian Award
2009, Howard Hughes Medical Institute Physician Scientist Early Career Award
2007, American College of Rheumatology Distinguished Fellow Award
2007, Clinical Immunology Society School of Autoimmune Diseases Travel Grant
2006, MedImmune Inflammation and Autoimmunity Abstract Competition Finalist
2006, Earl J. Brewer Research Travel Grant Award in Pediatric Rheumatology
2006, American College of Rheumatology REF Pediatric Rheumatology Research Award
2005-2008, National Institutes of Health Loan Repayment Program Recipient
2005, Philadelphia Rheumatism Society Fellows Research Forum Award
2005, Clinical Immunology Society Travel Grant Award – FOCIS meeting
2004, AMGEN Rheumatology Fellowship Award
2003, Seymour Warshaw Distinguished First Year Resident Award – The Children’s Hospital of Philadelphia
2002, Judy S. Finkelstein Memorial Symposium Award
2001, Howard Hughes Medical Institute Medical Student Continued Support Award
2000, Howard Hughes Medical Institute Medical Student Research Training Fellowship
2000, Four Schools Physician Scientist Fellowship
1998, National Institutes of Health Short Term Research Grant
Leadership and Memberships
Memberships in Professional Organizations
International
2010-present, Histiocyte Society
2007-present, International Macrophage Activation Syndrome Study Group
- 2007-present, Founding Member
National
2024-present, National Institute of Arthritis and Musculoskeletal and Skin Diseases
- 2024-present, Board of Scientific Councilors)
2016-present, American Society for Clinical Investigation
2015-present, Society for Pediatric Research
2013-present, Rheumatology Research Foundation
- 2023-present, Chair, Scientific Advisory Council
2008-present, American Association of Immunologists
2007-present, American College of Rheumatology
Editorial and Academic Positions
Editorial Positions
2020-present, Associate Editor, Arthritis and Rheumatology
2018-present, Ad Hoc Reviewer, Science
2011-present, Ad Hoc Reviewer, Journal of Clinical Investigation
2011-present, Peer Reviewer, Up-To-Date Allergy and Immunology Section
2010-present, Review Editor, Frontiers in Primary Immunodeficiencies
2010-present, Ad Hoc Editor, European Journal of Pediatrics
2010-present, Ad Hoc Editor, Blood
2010-present, Ad Hoc Editor, Arthritis and Rheumatism
2010-present, Ad Hoc Reviewer, Clinical and Vaccine Immunology
2010-present, Ad Hoc Reviewer, Journal of Clinical Rheumatology
2010-present, Ad Hoc Reviewer, Pediatrics
2010-present, Ad Hoc Reviewer, Journal of Rheumatology
2010-present, Ad Hoc Reviewer, International Archives of Allergy and Immunology
2008-present, Ad Hoc Reviewer, Clinical Immunology
2008-present, Ad Hoc Reviewer, Nature Clinical Practice Rheumatology
2007-present, Ad Hoc Reviewer, Rheumatology
2006-present, Ad Hoc Reviewer, Pediatric Rheumatology Online Journal
Institutional Committees
2022-present, Quantitative Education Strategies for Trainees (QuEST), University of Pennsylvania
2022-present, Research & Innovation Pillar Council
2018-present, Co-Principle Investigator, Rheumatology T32, Hospital of the University of Pennsylvania
2018-present, Institutional Biosafety Committee (IBC)
2013-present, Director, University of Pennsylvania Institute for Immunology - Autoimmunity Program (committee of the IFI at PSOM), Hospital of the University of Pennsylvania
2011-present, Clinical Data Reporting and Quality Subcommittee
2011-present, Co-Director, Center for Protective Immunity and Immunopathology Affinity Group (Committee of the Research Affinity Group Council)
2009-present, Perelman School of Medicine MD/PhD Admission Committee, University of Pennsylvania
Education & training
Medical Degree
MD - University of Pennsylvania School of Medicine, Philadelphia, PA
Internship
Pediatrics - The Children's Hospital of Philadelphia, Philadelphia, PA
Residency
Pediatrics - The Children's Hospital of Philadelphia, Philadelphia, PA
Fellowship
Pediatric Rheumatology - The Children's Hospital of Philadelphia, Philadelphia, PA
Additional Training
Medical Student Research Training Fellowship - Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, PA
Publications
Publications
2024
Kilich G, Hassey K, Behrens EM, Falk M, Vanderver A, Rader DJ, Cahill PJ, Raper A, Zhang Z, Westerfer D, Jadhav T, Conlin L, Izumi K, Rajagopalan R, Sullivan KE; UDN Consortium: Kagami Ogata syndrome: a small deletion refines critical region for imprinting. NPJ Genom Med 9(1), Jan 2024.
Huang Y, Sompii-Montgomery L, Patti J, Pickering A, Yasin S, Do T, Baker E, Gao D, Abdul-Aziz R, Behrens EM, Canna S, Clark M, Co DO, Collins KP, Eberhard B, Friedman M, Graham TB, Hahn T, Hersh AO, Hobday P, Holland MJ, Huggins J, Lu PY, Mannion ML, Manos CK, Neely J, Onel K, Orandi AB, Ramirez A, Reinhardt A, Riskalla M, Santiago L, Stoll ML, Ting T, Grom AA, Towe C, Schulert GS: Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease. Arthritis Care Res 76(3): 328-339, Mar 2024.
Huang Y, Sompii-Montgomery L, Patti J, Pickering A, YasinA, Do T, Baker E, Gao D, Abdul-Aziz R, Behrens EM, Canna S, Clark M, Co DO, Collins KP, Eberhard B, Friedman M, Graham TB, Hahn T, Hersh AO, Hobday P, Holland MJ, Huggins J, Lu PY, Mannion ML, Manos CK, Neely J, Onel K, Orandi AB, Ramirez A, Reinhardt A, Riskalla M, Santiago L, Stoll ML, Ting T, Grom AA, Towe C, Schulert GS: Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease. Arthritis Care and Research 76(3): 328-339, March 2024.
Gupta K, Xu JP, Diamond T, de Jong IEM, Glass A, Llewellyn J, Theise ND, Waisbourd-Zinman O, Winkler JD, Behrens EM, Mesaros C, Wells RG: Low-dose biliatresone treatment of pregnant mice causes subclinical biliary disease in their offspring: Evidence for a spectrum of neonatal injury. PLoS One 19(4), Apr 2024.
Chandrakasan S, Jordan MB, Baker A, Behrens EM, Bhatla D, Chien M, Eckstein OS, Henry MM, Hermiston ML, Hinson AP, Leiding JW, Oladapo A, Patel SA, Pednekar P, Ray AK, Dávila Saldaña B, Sarangi SN, Walkovich KJ, Yee JD, Zoref-Lorenz A, Allen CE: Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab. Blood Adv 8(9): 2248-2258, May 2024.
Adang LA, D'Aiello R, Takanohashi A, Woidill S, Gavazzi F, Behrens EM, Sullivan KE, Goldbach-Mansky R, de Jesus AA; AGS Clinical Trial Readiness Workgroup; Vanderver A: FN-signaling gene expression as a diagnostic biomarker for monogenic interferonopathies. JCI Insight 9(14), Jun 2024.
Chapin CA, Diamond T, Perez A, Kreiger PA, Loomes KM, Behrens EM, Alonso EM: Identification of pediatric activated T-cell hepatitis using clinical immune studies. Clin Res Hepatol Gastroenterol 48(7), Aug 2024.
2023
Rood JE, Rezk A, Pogoriler J, Finn LS, Burnham JM, Josephson MB, Bar-Or A, Behrens EM, Canna SW: Improvement of Refractory Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease with Single-Agent Blockade of IL-1β and IL-18. J Clin Immunol 43(1): 101-108, Jan 2023 Notes: Epub 2022 Aug 25, doi:10.1007/s10875-022-01353-y.
Chapin CA, Burn TM, Diamond T, Loomes KM, Alonso EM, Behrens EM: Effector Memory CD8 T-cells as a Novel Peripheral Blood Biomarker for Activated T-cell Pediatric Acute Liver Failure. PLoS One 18(6): e0286394, June 2023.
Diamond T, Lau M, Morrissette J, Chu N, Behrens 6EM: CXCL9 Inhibition Does Not Ameliorate Disease in Murine Models of Both Primary and Secondary Hemophagocytic Lymphohistiocytosis Sci Rep 13(1): 12298, July 2023 Notes: doi: 10.1038/s41598-023-39601-9.
Chapin CA, Diamond T, Harris RM, Vaccaro O, Loomes KM, Alonso EM, Behrens EM: Adenovirus is Not Detected in Liver Tissue from a Historical Multicenter Cohort of Children with Acute Liver Failure. J Pediatr Gastroenterol Nutr 77(3): 393-395, Sep 2023 Notes: doi: 10.1097/MPG.0000000000003851.
Schuelke MR, Bassiri H, Behrens EM, Canna S, Croy C, DiNofia A, Gollomp K, Grupp S, Lambert M, Lambrix A, Maude SL, Myers R, Newman H, Petrosa W, Seif A, Sullivan KE, Teachey DT, Diorio C: Emapalumab for the treatment of refractory cytokine release syndrome in pediatric patients. Blood Adv 7(18): 5603-5607, September 2023 Notes: DOI: 10.1182/bloodadvances.2023010712.
Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, Piskin D, Ravelli A, Sinha R, Aletaha D, Allen C, Bassiri H, Behrens EM, Carcillo J, Carl L, Chatham W, Cohen JI, Cron RQ, Drewniak E, Grom AA, Henderson L, Horne A, Jordan MB, Nichols KE, Schulert G, Vastert S, Demirkaya E, Goldbach-Mansky R, de Benedetti F, Marsh RA, Canna SW; HLH/MAS task force: The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) Ann Rheum Dis 82(10): 1271-1285, October 2023 Notes: doi: 10.1136/ard-2023-224123. Epub 2023 Jul 24.
Chang JC, Varghese SA, Behrens EM, Gmuca S, Kennedy JS, Liebling EJ, Lerman MA, Mehta JJ, Rutstein BH, Sherry DD, Stingl CJ, Weaver LK, Weiss PF, Burnham JM: Improving Outcomes of Pediatric Lupus Care Delivery with Provider Goal-Setting Activities and Multidisciplinary Care Models Arthritis Care Res (Hoboken) 75(11): 2267-2276, Nov 2023.
Dar AA, Kim DD, Gordon SM, Klinzing K, Rosen S, Guha I, Porter N, Ortega Y, Forsyth KS, Roof J, Fazelinia H, Spruce LA, Eisenlohr LC, Behrens EM, Oliver PM: c-Myc uses Cul4b to preserve genome integrity and promote antiviral CD8+ T cell immunity. Nat Commun 14(1), Nov 2023.
2022
Li EY, Xu J, Nelson ND, Teachey D, Tan K, Romberg N, Behrens EN, Pillai V, Kikuchi-Fujimoto: Disease is Mediated by an Aberrant Type I Interferon Response. Mod Path 35(4): 462-469, Apr 2022 Notes: Epub 2021 Dec 24. doi: 10.1038/s41379-021-00992-7.
Khanna C, Le Coz C, Vaccaro C, Pillarisetti P, Knox AVC, Sy A, Behrens EM, Buchbinder D, Romberg N: Lymphocytes Utilize Somatic Mutations, Epigenetic Silencing, and the Proteasome to Escape Truncated WASP Expression. J Clin Immunol 42(4): 753-759, May 2022 Notes: Epub 2022 Feb 12. doi:10.1007/s10875-022-01224-6.
Diamond T, Burn TN, Nishiguchi MA, Minichino D, Chase J, Chu N, Kreiger PA, Behrens EM: Familial Hemophagocytic Lymphohistiocytosis Hepatitis is Mediated by IFN-y in a Predominantly Hepatic-Intrinsic Manner. PLoS One 17(6): e0269553, June 2022 Notes: doi: 10.1371/journal.pone.0269553. eCollection 2022.
Sharmistha Rudra, Eliana Shaul, Maire Conrad, Trusha Patel, Astrela Moore, Noor Dawany, Megan C Canavan, Kathleen E Sullivan, Edward Behrens, Judith R Kelsen: Ruxolitinib: Targeted Approach for Treatment of Autoinflammatory Very Early Onset Inflammatory Bowel Disease Clin Gastroenterol Hepatol 42(4): 1408-1410, June 2022 Notes: doi: 10.1016/j.cgh.2021.07.040. Epub 2021 July 27.
Adang LA, Gavazzi F, D'Aiello R, Isaacs D, Bronner N, Arici ZS, Flores Z, Jan A, Scher C, Sherbini O, Behrens EM, Goldbach-Mansky R, Olson TS, Lambert MP, Sullivan KE, Teachey DT, Witmer C, Vanderver A, Shults J.: Hematologic Abnormalities in Aicardi Goutières Syndrome. Mol Genet Metab 136(4): 324-329, Aug 2022 Notes: Epub 2022 Jun 16. doi: 10.1016/j.ymgme2022.06.003.
Burn TN, Gordon SM, Culberson EJ, Diamond T, Kreiger PA, Hayer KE, Bhattacharyya A, Jones, JM, Bassing CH Behrens: The RAG1 Ubiquitin Ligase Domain Stimulates Recombination of TCRβ and TCRa Genes and Influences Development of aβT Cell Lineages. J Immunol 209(5): 938-949, Sep 2022 Notes: doi:10.4049/jimmunol.2001441. Epub 2022 Aug 10.
Diorio C, Shraim R, Myers R, Behrens EM, Canna S, Bassiri H, Aplenc R, Burudpakdee C, Chen F, DiNofia AM, Gill S, Gonzalez V, Lambert MP, Barz Leahy A, Levine BL, Lindell RB, Maude SL, Melenhorst JJ, Newman H, Perazzelli J, Seif AE, Lacey SF, June CH, Barrett DM, Grupp SA, Teachey DT.: Comprehensive Serum Proteome Profiling of Cytokine Release Syndrome and Immune Effector Cell Associated Neurotoxicity Syndrome in B-Cell ALL Patients Receiving CART19. Clin Cancer Res 28(17): 3804-3813, Sep 2022 Notes: doi:10.1158/1078-0432.CCR-22-0822. Lynch HM, Beslow LA, Treat JR, Corcoran A, Lin JH, Gunawardena N, Maris JM, Behrens EM: Paraneoplastic Myasthenia Gravis and Pemphigus Associated With Follicular Dendritic Cell Sarcoma Leading to Cardiorespiratory Collapse in a 7-year-old. Pediatr Blood Cancer 69(11), Nov 2022 Notes: doi:10.1002/pbc.29723. Epub 2022 Apr 29.
Minichino D, Lv K, Chu N, Tong W, Behrens EM: BRAF-V600E Utilizes Posttranscriptional Mechanisms to Amplify LPS-induced TNFα Production in Dendritic Cells in a Mouse Model of Langerhans Cell Histiocytosis. J Leukoc Biol 112(5): 1089-1104, Nov 2022 Notes: doi: 10.1002/JLB.3A0122-075RR. Online ahead of print.
2021
Kelson JR, Dawany N, Conrad MA, Karakasheva TA, Maurer K, Wei JM, Uman S, Dent MH, Behera R, Bryant LM, Ma X, Moreira L, Chatterji P, Shraim R, Merz A, Simon LA, Muir AB, Giraudo C, Behrens EM, Whelan KA, Dovoto M, Russo PA, Andres SF, Sullivan KE, Hamilton KE: Colonoids from Patients with Pediatric Inflammatory Bowel Disease Exhibit Decreased Growth Associated with Inflammation Severity and Durable Upregulation of Antigen Presentation Genes. Inflamm Bowel Disease 27(2): 256-267, Jan 2021 Notes: doi: 10.1093/ibd/izaa145.
Vella LA, Giles JR, Baxter AE, Oldridge DA, Diorio C, Kuri-Cervantes L, Alanio C, Pampena MB, Wu JE, Chen Z, Huang YJ, Anderson EM, Gouma S, McNerney KO, Chase J, Burudpakdee C, Lee JH, Apostolidis SA, Huang AC, Divij M, Kuthuru O, Goodwin EC, Madison E. Weirick, Marcus J. Bolton, Claudia P. Arevalo, Ramos A, CJ Jasen, Conrey PE, Sayed S, Giannini HM, D'Andrea K, The UPenn COVID Processing Unit, Meyer NJ, Behrens EM, Bassiri H, Hensley SE, Henrickson SE, Teachey DT, Betts MR, Wherry EJ: Deep Immune Profiling of MIS-C Demonstrates Marked but Transient Immune Activation Compared to Adult and Pediatric COVID-19. Sci Immunol 6(57): eabf7570, Mar 2021 Notes: doi: 10.1126/Sciimmunol.abf7570.
Anderson EM, Diorio C, Goodwin EC, McNerney KO, Weirick ME, Gouma S, Bolton MJ, Arevalo CP, Chase J, Hicks P, Manzoni TB, Baxter AE, Andrea KP, Burudpakdee C, Lee JH, Vella LA, Henrickson SE, Harris RM, Wherry EJ, Bates P, Bassiri H,* Behrens EM,* Teachey DT*: Severe Acute Respiratory Syndrome-Coronavirus (SARS-CoV-2) Antibody Responses in Children with Multisystem Inflammatory Syndrome in Children (MIS-C) and Mild and Severe Coronavirus Disease 2019 (COVID-19). J Ped Infect Dis Soc 10(5): 669-673, May 2021 Notes: doi: 10.1093/jpids/piaa161, *denotes equal authorship.
Choa R, Tohyama J, Wada S, Meng H, Hu J, Okumura M, May RM, Robertson TF, Pai RL, Nace A, Hopkins C, Jacobsen EA, Haldar M, FitzGerald GA, Behrens EM, Minn AJ, Seale P, Cotsarelis G, Kim B, Seykora JT, Li M, Arany Z, Kambayashi T: Thymic Stromal Lymphopoietin Induces Adipose Loss Through Sebum Hypersecretion. Science 373(6554): eabd2893, July 2021 Notes: doi:10.1106/science.abd2893.
Triebwasser MP, Barrett DM, Bassiri H, Bunin N, ElGarten C, Freedman J, Geera AS, Monos D, Lambert MP, Olson T, Seif AE, Paessler M, Petrosa W, Reilly AF, Romberg N, Sullivan KE, Quinn GZ, Behrens EM, Teachey DT.: Combined use of Emapalumab and Ruxolitinib in a Patient with Refractory Hemophagocytic Lymphohistiocytosis was Safe and Effective. Pediatr Blood Cancer 68(7): e29026, July 2021 Notes: doi:10.1002/pbc.29026. Epub 2021 Mar 22. Chang JC, Matsubara D, Morgan RW, Diorio C, Nadaraj S, Teachey DT, Bassiri H, Behrens EM, Banerjee A: Skewed Cytokine Responses Rather Than the Magnitude of the Cytokine Storm May Drive Cardiac Dysfunction in Multisystem Inflammatory Syndrome in Children J Am Heart Assoc 10(16): e21428, Aug 2021 Notes: doi:10.1161/JAHA.121.021428.
Chapin A, Taylor SA, Malladi P, Neighbors K, Melin-Aldana H, Kreiger PA, Bowsher N, Schipma MJ, Loomes KM, Behrens EM, Alonso EM.: Transcriptional Analysis of Liver Tissue Identifies Distinct Phenotypes of Indeterminate. Hepatol Commun 5(8): 1373-1384, Aug 2021 Notes: doi: 10.1002/hep4.1726. eCollectin 2021.
Si SJ, Tasian SK, Bassiri H, Fisher BT, Atalla J, Patel R, Romberg N, Lambert MP, Paessler M, Behrens EM, Teachey DT, Sullivan KE.: Diagnostic Challenges in Pediatric Hemophagocytic Lymphohistiocytosis. J. Clin. Immunol 41(6): 1213-1218, Aug 2021 Notes: doi:10.1007/s10875-021-01025-3. Epub 2021 Mar 4.
Otto WR, Behrens EM, Teachey DT, Lamson DM, Barrett DM, Bassiri H, Lambert MP, Mount S, Petrosa WL, Romberg N, Sullivan KE, Topjian AA, Fisher BT, Kajon AE.: Human Adenovirus 7-Associated Hemophagocytic Lymphohistiocytosis-Like Illness: Clinical and Virological Characteristics in a Cluster of Five Pediatric Cases. Clin Infect Dis 73(7): e1532-e1538, Oct 2021 Notes: doi: 10.1093/cid/ciaa1277.
Ouahed J, Kelsen JR, Spessott WA, Kooshesh K, Sanmillan ML, Dawany N, Sullivan KE, Hamilton KE, Slowik V, Nejentsev S, Neves JF, Flores H, Chung WK, Wilson A, Anyane-Yeboa K, Wou K, Jain P, Field M, Tollefson S, Dent MH, Li D, Naito T, McGovern DPB, Kwong AC, Taliaferro F, Ordovas-Montanes J, Horwitz BH, Kotlarz D, Klein C, Evans J, Dorsey J, Warner N, Elkadri A, Muise AM, Goldsmith J, Thompson B, Engelhardt KR, Cant AJ, Hambleton S, Barclay A, Toth-Petroczy A, Vuzman D, Carmichael N, Bodea C, Cassa CA, Devoto M, Maas RL, Behrens EM, Giraudo CG, Snapper SB: Variants in STXBP3 are Associated with Very Early Onset Inflammatory Bowel Disease, Bilateral Sensorineural Hearing Loss and Immune Dysregulation. J Crohns Colitis 15(11): 1908-1919, Nov 2021 Notes: doi: 10.1093/ecco-jcc/jjab077.
Diorio , Shraim R, Vella LA, Giles JR, Baxter AE, Oldridge DA, Canna SW, Henrickson SE, McNerney KO, Balamuth F, Burudpakdee C, Lee J, Leng T, Farrel A, Lambert MP, Sullivan KE, Wherry EJ, Teachey DT, Bassiri H, Behrens EM: Proteomic profiling of MIS-C patients Indicates Heterogeneity Relating to Interferon Gamma Dysregulation and Vascular Endothelial Dysfunction. Nature Commun 12(1): 7222, Dec 2021 Notes: doi:10.1038/s41667-021-27544-6.
2020
Burn TN, Weaver L, Rood JE, Chu N, Bodansky A, Kreiger PA, Behrens EM: Genetic deficiency of interferon-y reveals interferon-y-independent manifestations of murine hemophagocytic lymphohistiocytosis. Arthritis Rheumatol 72(2): 335-347, Feb 2020.
Burn TN, Lee KD, Dawany N, Robertson TF, Fisher MR, Bassing CH, Behrens EM : A spontaneous RAG1 Nonsense Mutation Unveils Naturally Occurring N-terminal Truncated RAG1 Isoforms Immunohorizons 4(3): 119-128, March 2020 Notes: doi: 10.4049/immunohorizons.2000001.
Biswas C, Chu N, Burn TN, Kreiger PA, Behrens EM.: Monomethylfumarate Ameliorates Murine Macrophage Activation Syndrome in Both Heme Oxygenase 1-dependent and -independent Manners. Arthritis Rheumatol 73(5): 885-895, May 2020.
Kelsen JR, Dawany N, Conrad MA, Karakasheva TA, Maurer K, Wei JM, Uman S, Dent MH, Behera R, Bryant LM, Ma X, Moreira L, Chatterji P, Shraim R, Merz A, Mizuno R, Simon LA, Muir AB, Giraudo C, Behrens EM, Whelan KA, Devoto M, Russo PA, Andres SF, Sullivan KE, Hamilton KE : Colonoids from patients with pediatric inflammatory bowel disease exhibit decreased growth associated with inflammation severity and durable upregulation of antigen presentation genes Inflamm Bowel Dis 27(2): 256-267, June 2020 Notes: doi: 10.1093/ibd/izaa145.
Chiotos K, Bassiri H, Behrens EM, Blatz AM, Chang J, Diorio C, Fitzgerald JC, Topjian A, John ARO: Multisystem inflammatory syndrome in children during the coronavirus 2019 Pandemic: A case series J Pediatric Infect Dis Soc 9(3): 393-398, July 2020 Notes: doi: 10.1093/jpids/piaa069
Gordon SM, Nishiguchi MA, Chase JM, Mani S, Mainigi MA, Behrens EM : IFNs Drive Development of Novel IL-15-Responsive Macrophages J Immunol 205(4): 1113-1124, August 2020 Notes: doi: 10.4049/jimmunol.2000184. Epub 2020 Jul 20.
Corwin DJ, Sartori LF, Chiotos K, Odom John AR, Cohn K, Bassiri H, Behrens EM, Teachey DT, Henrickson SE, Diorio CJ, Zorc JJ, Balamuth F.: Distinguishing Multisystem Inflammatory Syndrome in Children From Kawasaki Disease and Benign Inflammatory Illnesses in the SARS-CoV-2 Pandemic. Pediatr Emerg Care 36(11): 554-558, Nov 2020 Notes: doi: 10.1097/PEC.00000000000022.48.
Diorio C, Anderson EM, McNerney KO, Goodwin EC, Chase JC, Bolton MJ, Arevalo CP, Weirick ME, Gouma S, Vella LA, Henrickson SE, Chiotos K, Fitzgerald JC, Kilbaugh TJ, John ARO, Blatz AM, Lambert MP, Sullivan KE, Tartaglione MR, Zambrano D, Martin M, Lee JH, Young P, Friedman D, Sesok-Pizzini DA, Hensley SE, Behrens EM, Bassiri H, Teachey DT.: Convalescent Plasma for Pediatric Patients with SARS-CoV-2-Associated Acute Respiratory Distress Syndrome. Pediatr Blood Cancer 67(11): e28693, Nov 2020 Notes: doi: 10.1002/pbc.28693, Epub 2020 Sep 4.
Diorio C, Henrickson SE, Vella LA, McNerney KO, Chase JM, Burudpakdee C, Lee JH, Jasen C, Balamuth F, Barrett DM, Banwell B, Bernt KM, Blatz AM, Chiotos K, Fisher BT, Fitzgerald JC, Gerber JS, Gollomp K, Gray C, Grupp SA, Harris RM, Kilbaugh TJ, Odom John AR, Lambert MP, Liebling EJ, Paessler M, Petrosa W, Phillips CA, Reilly AF, Romberg N, Seif AE, Sesok-Pizzini D, Sullivan K, Vardaro J, Behrens EM*, Teachey DT*, Bassiri H*: Multisystem Inflammatory Syndrome in Children and COVID-19 are Distinct Presentations of SARS-CoV-2. J Clin Invest 130(11): 5967-5975, Nov 2020 Notes: doi: 10.1172/JCI140970. Online ahead of print.* denotes equal authorship
Henderson LA, Canna SW, Friedman KG, Gorelik M, Lapidus SK, Bassiri H, Behrens EM, Ferris A, Kernan KF, Schulert GS, Seo P, Son MBF, Tremoulet AH, Yeung RSM, Mudano AS, Turner AS, Karp DR, Mehta JJ.: American College of Rheumatology Clinical Guidance for Pediatric Patients with Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with SARS-CoV-2 and Hyperinflammation in COVID-19. Version 2. Arthritis Rheumatol 72(11): 1791-1805, November 2020 Notes: doi: 10.1002/art.41454.
Hung LY, Tanaka Y, Herbine K, Pastore C, Singh B, Ferguson A, Vora N, Douglas B, Zullo K, Behrens EM, Li Hui Tan T, Kohanski MA, Bryce P, Lin C, Kambayashi T, Reed DR, Brown BL, Cohen NA, Herbert DR.: Cellular context of IL-33 expression dictates impact on anti-helminth immunity. Sci Immunol 5(53): eabc6259, Nov 2020 Notes: doi;10.1126/sciimmunol.abc6259.
Anderson EM, Diorio C, Goodwin EC, McNerney KO, Weirick ME, Gouma S, Bolton MJ, Arevalo CP, Chase J, Hicks P, Manzoni TB, Baxter AE, Andrea KP, Burudpakdee C, Lee JH, Vella LA, Henrickson SE, Harris RM, Wherry EJ, Bates P, Bassiri H, Behrens EM, Teachey DT, Hensley SE.: SARS-CoV-2 Antibody Responses in Children with MIS-C and Mild and Severe COVID-19. J Pediatric Infect Dis Soc 10(5): 669-673, December 2020.
Chapin CA, Melin-Aldana H, Kreiger PA, Burn T, Neighbors K, Taylor SA, Ostilla L, Wechsler JB, Horslen SP, Leonis MA, Loomes KM, Behrens EM, Squires RH, Alonso EM.: Activated CD8 T-cell Hepatitis in Children with Indeterminate Acute Liver Failure: Results from a Multicenter Cohort. Pediatric Acute Liver Failure Study Group.J Pediatr Gastroenterol Nutr. 71(6): 713-719, Dec 2020 Notes: doi: 10.1097/MPG.0000000000002893.
Diorio C, McNerney KO, Lambert M, Paessler M, Anderson EM, Henrickson SE, Chase J, Liebling E, Burudpakdee C, Lee J, Balamuth F, Blatz AM, Chiotos K, Fitzgerald J, Giglia T, Gollomp K, Odom John AR, Jasen C, Leng T, Petrosa W, Vella LA, Wtmer C, Sullivan KE, Laskin B, Hensley SE, Bassiri H*, Behrens EM*, Teachey DT*: Evidence of Thrombotic Microangiopathy in Children with SARS-CoV-2 Across the Spectrum of Clinical Presentations Blood Adv 4(23): 6051-6063, December 2020 Notes: doi: 10.1182/bloodadvances.2020003471, *denotes equals authorship".
2014
Weaver LK, Behrens EM. Hyperinflammation, rather than hemophagocytosis, is the common link between macrophage activation syndrome and hemophagocytic lymphohistiocytosis. Curr Opin Rheumatol. 2014 Sep 26(5):562-9.
Zhang M, Behrens EM, Atkinson TP, Shakoory B, Grom AA, Cron RQ. Genetic defects in cytolysis in macrophage activation syndrome. Curr Rheumatol Rep. 2014;16(9):439.
Karimi MA, Lee E, Bachman MH, Salicioni AM, Behrens EM, Kambayashi T, Baldwin CL. Measuring cytotoxicity by bioluminescence imaging outperforms the standard chromium-51 release assay. PLOS One. 2014 Feb 19;9(2):e89357.
Canna SW, Costa-Reis P, Bernal WE, Chu N, Sullivan KE, Paessler ME, Behrens EM. Alternative activation of laser-captured murine hemophagocytes. Arthritis Rheumatol. 2014 Jun;66(6):1666-71.
2013
Joshi RP, Schmidt AM, Das J, Pytel D, Riese MJ, Lester M, Diehl JA, Behrens EM, Kambayashi T, Koretzky GA. The ζ isoform of diacylglycerol kinase plays a predominant role in regulatory T cell development and TCR-mediated ras signaling. Sci Signal. 2013 Nov 26;6(303).
Costa-Reis P, Weaver LK, Behrens EM, Sullivan KE. Chronic non-bacterial osteomyelitis in a patient with common variable immunodeficiency. Joint Bone Spine. 2014 Mar;81(2):193-4. Epub 2013 Sep 20..
Canna SW, Wrobel J, Chu N, Kreiger P, Paessler M, Behrens EM. Interferon-γ mediates anemia but is dispensable for Fulminant Toll-Like Receptor 9-induced Macrophage Activation Syndrome and Hemophagocytosis. Arthritis & Rheumatism. 2013 Jul;65(7):1764-75.
Rao S, Liu X, Freedman BD, Behrens EM. Spleen tyrosine kinase (Syk) dependent calcium signals mediate efficient CpG-induced exocytosis of tumor necrosis factor alpha (TNFα) in innate immune cells. J Biol Chem. 2013 May 3;288(18):12448-58,
Weaver LK, Behrens EM. How to use... antinuclear antibodies in paediatric rheumatic diseases. Arch Dis Child Educ Pract Ed. 2013 Apr;98(2):64-70.
2012
Sriram U, Varghese L, Bennett HL, Jog NR, Shivers DK, Ning Y, Behrens EM, Caricchio R, Gallucci S. Myeloid Dendritic Cells from B6.NZM Sle1/Sle2/Sle3 Lupus-prone Mice express an Interferon Signature that Precedes Disease Onset. J Immunol. 2012 Jul 1;189(1):80-91. Epub 2012 Jun 1.
Lieberman SM, Kim JS, Corbo-Rodgers E, Kambayashi T, Maltzman JS, Behrens EM, Turka LA. Site-specific accumulation of recently activated CD4+ Foxp3+ regulatory T cells following adoptive transfer. Eur J Immunol. 2012 Jun;42(6):1429-35.
Canna SW, Behrens EM. Making sense of the cytokine storm: a conceptual framework for understanding, diagnosing and treating hemophagocytic syndromes. Pediatr Clin North Am. 2012 Apr;59(2):329-44.
Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun. 2012 Jun;13(4):289-98. Epub 2012 Mar 15.
Canna SW, Behrens EM. Not all hemophagocytes are created equally: appreciating the heterogeneity of the hemophagocytic syndromes. Curr Opin Rheumatol. 2012 Jan;24(1):113-8.
2011
Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice. Behrens EM, Canna SW, Slade K, Rao S, Kreiger PA, Paessler M, Kambayashi T, Koretzky GA. J Clin Invest. 2011 Jun;121(6):2264-77.
Not all hemophagocytes are created equally: appreciating the heterogeneity of the hemophagocytic syndromes. Behrens EM, Canna SW, Slade K, Rao S, Kreiger PA, Paessler M, Kambayashi T, Koretzky GA. J Clin Invest. 2011 Jun;121(6):2264-77.
Lectures by Invitation
2024
Behrens E. "Diseases of Interferons: From the Bench to the Clinic", Lurie Children's Hospital of Chicago, Chicago, IL, USA. Apr 2024.
Behrens E. "Cytocine Storm", Pediatric Grand Rounds, Mount Sinai Hospital, New York, NY. Mar 2024.
2023
Behrens E. "When and How to Gather: Building Social Connections and Intellectual Comradery", American College of Rheumatology Convergence 2023, San Diego, CA. Nov 2023.
Behrens E. "Treatment Patterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still's Disease Treated with Emapalumab: The REAL-HLH Study" American College of Rheumatology Convergence 2023, San Diego, CA. Nov 2023.
Behrens E. "sJIA/MAS" Children's National Hospital, Washington, DC. Nov 2023.
Behrens E. "Digging in the DIRT to Avoid Going Down a Rabbit Hole: A multi-disciplinary approach to complex immune dysregulation patients", VEO-IBD Symposium, Virtual. Nov 2023.
Behrens E. "Treatment Patterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still's Disease Treated with Emapalumab: The REAL-HLH Study", America College of Rheumatology Convergence, San Diego, CA. Nov 2023.
Behrens E. "HLH: Meet the Expert", Histiocyte Society, Athens, Greece. Oct 2023.
Behrens E. "Cytokine Storm/Systemic JIA", American College of Rheumatology Convergence, ViP Pediatrics 2023, Virtual. Jul 2023.
Behrens E. "Rational Treatment of HLH. Washington University, Division of Rheumatology, Kroc Visiting Professorship, Saint Louis, MO. Apr 2023.
Behrens E. "Rational Treatment of HLH" Second Edition of the Virtual Meeting on Cytokine Release Syndrome, Rainbow Children's Hospital, Bangalore, India. Mar 2023.
Behrens E. "Immune Dysregulation in Rheumatology" Rheumatology Grand Rounds and Visiting Professor, University of Alabama, Division of Clinical Immunology and Rheumatology, Birmingham, AL. Feb 2023.
Behrens E. "HLH/Macrophage Activating Syndrome in Adults" Rheumatology Grand Rounds, Temple University, Division Rheumatology, Philadelphia, PA. Feb 2023.
Editorials, Reviews, Chapters
2023
Behrens EM: UnDRESSing Systemic Juvenile Idiopathic Arthritis Lung Disease. Arthritis Care Res (Hoboken) 75(10): 2033-2035, April 2023.
Perfetto J, Behrens EM, Lerman MA, Paessler ME, Liebling EJ: Avoid a Rash Diagnosis: Reconsidering Cytophagic Histiocytic Panniculitis as a Distinct Clinical-pathologic Entity. JAAD Case Report 11(36): 40-44, April 2023 Notes: DOI: 10.1016/j.jdcr.2023.03.019.
Diamond T, Bennett AD, Behrens EM: The Liver in Hemophagocytic Lymphohistiocytosis: Not an Innocent Bystander. J Pediatr Gastroenterol Nutr 77(2): 153-159, Aug 2023 Notes: doi: 10.1097/MPG.0000000000003807. Epub 2023 Apr 25.
Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, Piskin D, Ravelli A, Sinha R, Aletaha D, Allen C, Bassiri H, Behrens EM, Carcillo J, Carl L, Chatham W, Cohen JI, Cron RQ, Drewniak E, Grom AA, Henderson LA, Horne A, Jordan MB, Nichols KE, Schulert G, Vastert S, Demirkaya E, Goldbach-Mansky R, de Benedetti F, Marsh RA, Canna SW; HLH/MAS task force: The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS). Ann Rheum Dis 82(10): 1271-1285, October 2023 Notes: doi: 10.1136/ard-2023-224123. Epub 2023 Jul 24.
Canna CW, Behrens EM: Etiology and Pathogenesis of Juvenile 12th Edition of Kelley's 12th Edition of Kelley's 2023.
2022
Henderson LA, Canna SW, Friedman KG, Gorelik M, Lapidus SK, Bassiri H, Behrens EM, Kernan KF, Schulert GS, Seo P, Son MBF, Tremoulet AH, VanderPluym C, Yeung RSM, Mudano AS, Turner AS, Karp DR, Mehta JJ: American College of Rheumatology Clinical Guidance for Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 and Hyperinflammation in Pediatric COVID-19: Version 3. Arthritis Rheumatol 74(4): e1-e20, April 2022 Notes: Epub 2022 Feb 3. doi: 10/1002/art.42062.
2021
Henderson LA, Canna SW, Friedman KG, Gorelik M, Lapidus SK, Bassiri H, Behrens EM, Ferris A, Kernan KF, Schulert GS, Seo P, Son MBF, Tremoulet AH, Yeung RMS, Mudano AS, Turner AS, Karp DR, Mehta JJ.: American College of Rheumatology Clinical Guidance for Multisystem Inflammatory Syndrome in Children Associated with SARS-CoV-2 and Hyperinflammation in Pediatric COVID-19: Version 2. Arthritis Rheumatol 73(4): e13-e29, Apr 2021 Notes: Epub 2021 Feb 15. doi:10.1002/art.41616.
Henderson LA, Canna SW, Friedman KG, Gorelik M, Lapidus SK, Bassiri H, Behrens EM, Ferris A, Kernan KF, Schulert GS, Seo P, Son MBF, Tremoulet AH, Yeung RSM, Mudano AS, Turner AS, Karp DR, Mehta JJ: American College of Rheumatology Clinical Guidance for Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 and Hyperinflammation in Pediatric COVID-19: Version 2. Arthritis Rheumatol 73(4): e13-e29, April 2021 Notes: Epub 2021 Feb 15.
Henderson LA, Friedman KG, Son MBF, Kernan KF, Canna SW, Gorelik M, Lapidus SK, Ferris A, Schulert GS, Seo P, Tremoulet AH, Yeung RSM, Karp DR, Bassiri H, Behrens EM, Mehta JJ: Reply. Arthritis Rheumatol 73(7): 1342-1343, July 2021 Notes: doi: 10.1002/art.41689. Epub 2021.
2020
Liebling EJ, Sze RW, Behrens EM: Vitamin C Deficiency Mimicking Inflammatory Bone Disease of the Hand. Pediatr Rheumatol Online 18(1): 45, June 2020 Notes: doi: 10.1186/s12969-020-00439-4.
Behrens EM: Etiology and Pathogenesis of Juvenile Idiopathic Arthritis. Firestein & Kelley's Textbook of Rheumatology, 2-Volume Set, 11th Edition. Gary Koretzky (eds.). Elsevier, Page: 1905-1910, July 2020.
Chiotos K, Bassiri H, Behrens EM, Blatz AM, Chang J, Diorio C, Fitzgerald JC, Topjian A, John ARO: Multisystem Inflammatory Syndrome in Children During the Coronavirus 2019 Pandemic: A Case Series. H Pediatric Infect Dis Soc 9(3): 393-398, July 2020 Notes: doi:10.1093/jpids/piaa069.
Henderson LA, Canna SW, Schulert GS, Volpi S, Lee PY, Kernan KF, Caricchio R, Mahmud S, Hazen MM, Halyabar O, Hoyt KJ, Han J, Grom AA, Gattorno M, Ravelli A, De Benedetti F, Behrens EM, Cron RQ, Nigrovic PA : On the alert for cytokine storm: immunopathology in COVID-19 Arthritis Rheumatol 72(7): 1059-1063, July 2020 Notes: doi: 10.1002/art.41285. Epub 2020 May 10.
Henderson LA, Canna SW, Friedman KG, Gorelik M, Lapidus SK, Bassiri H, Behrens EM, Ferris A, Kernan KF, Schulert GS, Seo P, F Son MB, Tremoulet AH, Yeung RSM, Mudano AS, Turner AS, Karp DR, Mehta JJ: American College of Rheumatology Clinical Guidance for Pediatric Patients with Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with SARS-CoV-2 and Hyperinflammation in COVID-19. Version 1 Arthritis Rheumatol 72(11): 1791-1805, Nov 2020.
Behrens EM, Martini A, Gattorno M: Innate Immunity. Textbook of Pediatric Rheumatology. Ross Petty Ronald Laxer Carol Lindsley Lucy Wedderburn Robert Fuhlbrigge Elizabeth Mellins (eds.). Elsevier, December 2020.
Dulek DE, Fuhlbrigge RC, Tribble AC, Connelly JA, Loi MM, El Chebib H, Chandrakasan S, Otto WR, Diorio C, Keim G, Walkovich K, Jaggi P, Girotto JE, Yarbrough A, Behrens EM, Cron RQ, Bassiri H.: Multidisciplinary Guidance Regarding the Use of Immunomodulatory Therapies for Acute COVID-19 in Pediatric Patients. J Pediatric Infect Dis Soc 9(6): 716-737, Dec 2020 Notes: piaa098. doi: 10.1093/jpids/piaa098.
2019
Cron R, Behrens EM: Cytokines in Cytokine Storm Syndrome. Springer Nature Switzerland Ag 2019 Page: 197-207, 2019 Notes: https://doi.org/10.1007/978-3-030-22094-5_25.
Grom AA, Behrens BM: Macrophage Activation Syndrome in Rheumatic Disease. Textbook of Autoinflammation. Philip J. Hashkes, Ronald M. Laxer, Anna Simon (eds.). Springer, 2019.
2017
Alonso EM, Horslen SP, Behrens EM, Doo E: Pediatric Acute Liver Failure of Undetermined Cause: a Research Workshop. Hepatology 65(3): 1026-1037, March 2017 Notes: Epub 2017 Jan 6. doi:10.1002/hep.28944.
Canna SW, Girard C, Malle L, de Jesus A, Romberg N, Kelsen J, Surrey LF, Russo P, Sleight A, Schiffrin E, Gabay C, Goldbach-Mansky R, Behrens EM: Reply. J Allergy Clin Immunol 140(1): 316-317, July 2017 Notes: doi: 10.1016/j.jac.2017.03.016, Epub 2017 April 29.
2015
Basiaga ML, Weiss PF, Behrens EM: BIRC4 Mutation: An Important Rare Cause of uUveitis. J Clin Rheumatol 21(8): 444-447, December 2015 Notes: doi: 10.1097/RHU.0000000000000327.
2014
Behrens EM and Einsenberg RA: Pediatric Allergy. Biologic Drugs. Donald Leung (eds.). 2014 Notes: Chapter.
2010
Miettunen PM, Narendran A, Jayanthan A, Behrens EM, Cron RQ: Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients. Rheumatology 50(2): 417-419, February 2011 Notes: Epub 2010 Aug 7, doi:10.1093/rheumatology/keq218.
2005
Behrens EM: Literature Review: FcgRIIB regulation as a new therapeutic target in lupus. Pediatr Rheumatol Online J 3(1): 61-2, January/February 2005.