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J Allergy Clin Immunol Pract. 2018 Jan - Feb;6(1):38-69. doi: 10.1016/j.jaip.2017.11.023.

SJS/TEN 2017: Building Multidisciplinary Networks to Drive Science and Translation.

Author information

1
Vanderbilt University Medical Center, Nashville, Tenn.
2
Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
3
University of Southampton, Southampton, United Kingdom.
4
Wilford Hall Ambulatory Surgical Center, Lackland Air Force Base, San Antonio, Texas.
5
Loyola University Medical Center, Chicago, Ill.
6
University of British Columbia, Vancouver, British Columbia, Canada; B.C. Children's Hospital, British Columbia, Vancouver, British Columbia, Canada.
7
Massachusetts Eye and Ear, Harvard Medical School, Boston, Mass.
8
National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Md.
9
University of Tennessee Health Sciences, Memphis, Tenn.
10
Emek Medical Center, Technion-Institute of Technology, Afula, Israel; Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada.
11
University of Alabama at Birmingham, Birmingham, Ala.
12
Children's Mercy Hospital Kansas City, Kansas City, Mo.
13
Wake Forest Baptist Medical Center, Winston-Salem, NC; Wake Forest University School of Medicine, Winston-Salem, NC.
14
National Yang-Ming University, Taipei, Taiwan.
15
Memorial Sloan Kettering Cancer Center, New York, NY.
16
University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa.
17
Vanderbilt University Medical Center, Nashville, Tenn; Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, Western Australia, Australia.
18
National Human Genome Research Institute, National Institutes of Health, Bethesda, Md; F. Edward Hébert School of Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md.
19
University of Pennsylvania, Philadelphia, Pa.
20
Massachusetts General Hospital, Boston, Mass.
21
Medical Center and Medical Faculty-University of Freiburg, Freiburg, Germany.
22
University of Florida, Gainesville, Fla.
23
Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, Western Australia, Australia.
24
University of Liverpool, Liverpool, United Kingdom.
25
University of Toronto, Toronto, Ontario, Canada; Hospital for Sick Children, Toronto, Ontario, Canada.
26
University of California, San Francisco, Calif.
27
University Paris-Est-Créteil, Créteil, France.
28
National Human Genome Research Institute, National Institutes of Health, Bethesda, Md.
29
Showa University School of Medicine, Tokyo, Japan.
30
Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
31
Duke-NUS Medical School, Singapore, Singapore; Health Sciences Authority, Singapore, Singapore.
32
Austin Health, Heidelberg, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia.
33
US Food and Drug Administration, Silver Spring, Md.
34
National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
35
University of Ottawa, Ottawa, Ontario, Canada.
36
Chang Gung Memorial Hospital, Taipei, Taiwan.
37
Vanderbilt University Medical Center, Nashville, Tenn; Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, Western Australia, Australia. Electronic address: elizabeth.j.phillips@vanderbilt.edu.

Abstract

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a life-threatening, immunologically mediated, and usually drug-induced disease with a high burden to individuals, their families, and society with an annual incidence of 1 to 5 per 1,000,000. To effect significant reduction in short- and long-term morbidity and mortality, and advance clinical care and research, coordination of multiple medical, surgical, behavioral, and basic scientific disciplines is required. On March 2, 2017, an investigator-driven meeting was held immediately before the American Academy of Dermatology Annual meeting for the central purpose of assembling, for the first time in the United States, clinicians and scientists from multiple disciplines involved in SJS/TEN clinical care and basic science research. As a product of this meeting, this article summarizes the current state of knowledge and expert opinion related to SJS/TEN covering a broad spectrum of topics including epidemiology and pharmacogenomic networks; clinical management and complications; special populations such as pediatrics, the elderly, and pregnant women; regulatory issues and the electronic health record; new agents that cause SJS/TEN; pharmacogenomics and immunopathogenesis; and the patient perspective. Goals include the maintenance of a durable and productive multidisciplinary network that will significantly further scientific progress and translation into prevention, early diagnosis, and management of SJS/TEN.

KEYWORDS:

Electronic health record; Granulysin; HLA; Networks; Pharmacogenomics; Pharmacovigilance; Stevens-Johnson; T cells; Toxic epidermal necrolysis

PMID:
29310768
PMCID:
PMC5857362
DOI:
10.1016/j.jaip.2017.11.023
[Indexed for MEDLINE]
Free PMC Article

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