Assessment of Treatment Approaches and Outcomes in
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Insights From a Pan-European Multicenter Study

J 2021

Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights From a Pan-European Multicenter Study

KRIDIN, K., M. C. BRUGGEN, S. L. CHUA, A. BYGUM, S. WALSH et. al.

Basic information

Original name

Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights From a Pan-European Multicenter Study

Authors

KRIDIN, K., M. C. BRUGGEN, S. L. CHUA, A. BYGUM, S. WALSH, M. C. NAGELI, V. KUCINSKIENE, L. FRENCH, F. TETART, B. DIDONA, B. MILPIED, A. RANKI, C. SALAVASTRU, Eva BŘEZINOVÁ (203 Czech Republic, belonging to the institution), S. DIVANI-PATEL, T. LORENTZEN, J. L. NAGEL, S. VALIUKEVICIENE, V. KARPAVICIUTE, G. S. TIPLICA, E. OPPEL, A. OSCHMANN, N. DE PROST, A. VOROBYEV and S. INGEN-HOUSZ-ORO (guarantor)

Edition

JAMA dermatology, Chicago, IL, American Medical Association, 2021, 2168-6068

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30216 Dermatology and venereal diseases

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 11.816

RIV identification code

RIV/00216224:14110/21:00122521

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1001/jamadermatol.2021.3154

UT WoS

000689745000006

Keywords in English

Stevens-Johnson Syndrome; Toxic Epidermal Necrolysis; Treatment Approaches; Outcomes

Abstract

V originále

IMPORTANCE Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug reactions associated with a high rate of mortality and morbidity. There is no consensus on the treatment strategy. OBJECTIVE To explore treatment approaches across Europe and outcomes associated with the SJS/TEN disease course, as well as risk factors and culprit drugs. DESIGN, SETTING, AND PARTICIPANTS A retrospective pan-European multicenter cohort study including 13 referral centers belonging to the ToxiTEN ERN-skin subgroup was conducted. A total of 212 adults with SJS/TEN were included between January 1, 2015, and December 31, 2019, and data were collected from a follow-up period of 6 weeks. MAIN OUTCOMES AND MEASURES Risk factors for severe acute-phase complications (acute kidney failure, septicemia, and need for mechanical ventilation) and mortality 6 weeks following admission were evaluated using a multivariable-adjusted logistic regression model. One tool used in evaluation of severity was the Score of Toxic Epidermal Necrolysis (SCORTEN), which ranges from 0 to 7, with 7 the highest level of severity. RESULTS Of 212 patients (134 of 211 [63.7%] women; mean [SD] age, 51.0 [19.3] years), the mean (SD) body surface area detachment was 27% (32.8%). In 176 (83.0%) patients, a culprit drug was identified. Antibiotics (21.2%), followed by anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%), were the most common suspected agents. Treatment approaches ranged from best supportive care only (38.2%) to systemic glucocorticoids (35.4%), intravenous immunoglobulins (23.6%), cyclosporine (10.4%), and antitumor necrosis factor agents (3.3%). Most patients (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Maximal body surface area detachment (>= 30%) was found to be independently associated with severe acute-phase complications (fully adjusted odds ratio [OR], 2.49; 95% CI, 1.21-5.12; P = .01) and SCORTEN greater than or equal to 2 was significantly associated with mortality (fully adjusted OR, 10.30; 95% CI, 3.82-27.78; P < .001). Cyclosporine was associated with a higher frequency of greater than or equal to 20% increase in body surface area detachment in the acute phase (adjusted OR, 3.44; 95% CI, 1.12-10.52; P = .03) and an increased risk of infections (adjusted OR, 7.16; 95% CI, 1.52-33.74; P = .01). Systemic glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40; 95% CI, 0.18-0.88; P = .02). No significant difference in 6-week mortality was found between treatment groups. CONCLUSIONS AND RELEVANCE This cohort study noted differences in treatment strategies for SJS/TEN in Europe; the findings suggest the need for prospective therapeutic studies to be conducted and registries to be developed.

Citovat

KRIDIN, K., M. C. BRUGGEN, S. L. CHUA, A. BYGUM, S. WALSH, M. C. NAGELI, V. KUCINSKIENE, L. FRENCH, F. TETART, B. DIDONA, B. MILPIED, A. RANKI, C. SALAVASTRU, Eva BŘEZINOVÁ, S. DIVANI-PATEL, T. LORENTZEN, J. L. NAGEL, S. VALIUKEVICIENE, V. KARPAVICIUTE, G. S. TIPLICA, E. OPPEL, A. OSCHMANN, N. DE PROST, A. VOROBYEV and S. INGEN-HOUSZ-ORO. Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights From a Pan-European Multicenter Study. JAMA dermatology. Chicago, IL: American Medical Association, 2021, vol. 157, No 10, p. 1182-1190. ISSN 2168-6068. Available from: https://dx.doi.org/10.1001/jamadermatol.2021.3154.

@article{1796020,
   author = {Kridin, K. and Bruggen, M. C. and Chua, S. L. and Bygum, A. and Walsh, S. and Nageli, M. C. and Kucinskiene, V. and French, L. and Tetart, F. and Didona, B. and Milpied, B. and Ranki, A. and Salavastru, C. and Březinová, Eva and DivaniandPatel, S. and Lorentzen, T. and Nagel, J. L. and Valiukeviciene, S. and Karpaviciute, V. and Tiplica, G. S. and Oppel, E. and Oschmann, A. and de Prost, N. and Vorobyev, A. and IngenandHouszandOro, S.},
   article_location = {Chicago, IL},
   article_number = {10},
   doi = {http://dx.doi.org/10.1001/jamadermatol.2021.3154},
   keywords = {Stevens-Johnson Syndrome; Toxic Epidermal Necrolysis; Treatment Approaches; Outcomes},
   language = {eng},
   issn = {2168-6068},
   journal = {JAMA dermatology},
   title = {Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights From a Pan-European Multicenter Study},
   url = {https://jamanetwork.com/journals/jamadermatology/fullarticle/2783034},
   volume = {157},
   year = {2021}
}

TY  - JOUR
ID  - 1796020
AU  - Kridin, K. - Bruggen, M. C. - Chua, S. L. - Bygum, A. - Walsh, S. - Nageli, M. C. - Kucinskiene, V. - French, L. - Tetart, F. - Didona, B. - Milpied, B. - Ranki, A. - Salavastru, C. - Březinová, Eva - Divani-Patel, S. - Lorentzen, T. - Nagel, J. L. - Valiukeviciene, S. - Karpaviciute, V. - Tiplica, G. S. - Oppel, E. - Oschmann, A. - de Prost, N. - Vorobyev, A. - Ingen-Housz-Oro, S.
PY  - 2021
TI  - Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights From a Pan-European Multicenter Study
JF  - JAMA dermatology
VL  - 157
IS  - 10
SP  - 1182-1190
EP  - 1182-1190
PB  - American Medical Association
SN  - 21686068
KW  - Stevens-Johnson Syndrome
KW  - Toxic Epidermal Necrolysis
KW  - Treatment Approaches
KW  - Outcomes
UR  - https://jamanetwork.com/journals/jamadermatology/fullarticle/2783034
N2  - IMPORTANCE Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug reactions associated with a high rate of mortality and morbidity. There is no consensus on the treatment strategy. OBJECTIVE To explore treatment approaches across Europe and outcomes associated with the SJS/TEN disease course, as well as risk factors and culprit drugs. DESIGN, SETTING, AND PARTICIPANTS A retrospective pan-European multicenter cohort study including 13 referral centers belonging to the ToxiTEN ERN-skin subgroup was conducted. A total of 212 adults with SJS/TEN were included between January 1, 2015, and December 31, 2019, and data were collected from a follow-up period of 6 weeks. MAIN OUTCOMES AND MEASURES Risk factors for severe acute-phase complications (acute kidney failure, septicemia, and need for mechanical ventilation) and mortality 6 weeks following admission were evaluated using a multivariable-adjusted logistic regression model. One tool used in evaluation of severity was the Score of Toxic Epidermal Necrolysis (SCORTEN), which ranges from 0 to 7, with 7 the highest level of severity. RESULTS Of 212 patients (134 of 211 [63.7%] women; mean [SD] age, 51.0 [19.3] years), the mean (SD) body surface area detachment was 27% (32.8%). In 176 (83.0%) patients, a culprit drug was identified. Antibiotics (21.2%), followed by anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%), were the most common suspected agents. Treatment approaches ranged from best supportive care only (38.2%) to systemic glucocorticoids (35.4%), intravenous immunoglobulins (23.6%), cyclosporine (10.4%), and antitumor necrosis factor agents (3.3%). Most patients (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Maximal body surface area detachment (>= 30%) was found to be independently associated with severe acute-phase complications (fully adjusted odds ratio [OR], 2.49; 95% CI, 1.21-5.12; P = .01) and SCORTEN greater than or equal to 2 was significantly associated with mortality (fully adjusted OR, 10.30; 95% CI, 3.82-27.78; P < .001). Cyclosporine was associated with a higher frequency of greater than or equal to 20% increase in body surface area detachment in the acute phase (adjusted OR, 3.44; 95% CI, 1.12-10.52; P = .03) and an increased risk of infections (adjusted OR, 7.16; 95% CI, 1.52-33.74; P = .01). Systemic glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40; 95% CI, 0.18-0.88; P = .02). No significant difference in 6-week mortality was found between treatment groups. CONCLUSIONS AND RELEVANCE This cohort study noted differences in treatment strategies for SJS/TEN in Europe; the findings suggest the need for prospective therapeutic studies to be conducted and registries to be developed.
ER  -

KRIDIN, K., M. C. BRUGGEN, S. L. CHUA, A. BYGUM, S. WALSH, M. C. NAGELI, V. KUCINSKIENE, L. FRENCH, F. TETART, B. DIDONA, B. MILPIED, A. RANKI, C. SALAVASTRU, Eva BŘEZINOVÁ, S. DIVANI-PATEL, T. LORENTZEN, J. L. NAGEL, S. VALIUKEVICIENE, V. KARPAVICIUTE, G. S. TIPLICA, E. OPPEL, A. OSCHMANN, N. DE PROST, A. VOROBYEV and S. INGEN-HOUSZ-ORO. Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights From a Pan-European Multicenter Study. \textit{JAMA dermatology}. Chicago, IL: American Medical Association, 2021, vol.~157, No~10, p.~1182-1190. ISSN~2168-6068. Available from: https://dx.doi.org/10.1001/jamadermatol.2021.3154.

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