-
Something wrong with this record ?
Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Insights From a Pan-European Multicenter Study
K. Kridin, MC. Brüggen, SL. Chua, A. Bygum, S. Walsh, MC. Nägeli, V. Kucinskiene, L. French, F. Tétart, B. Didona, B. Milpied, A. Ranki, C. Salavastru, E. Brezinová, S. Divani-Patel, T. Lorentzen, JL. Nagel, S. Valiukeviciene, V. Karpaviciute,...
Language English Country United States
Document type Journal Article, Multicenter Study
- MeSH
- Cyclosporine therapeutic use MeSH
- Adult MeSH
- Immunoglobulins, Intravenous therapeutic use MeSH
- Cohort Studies MeSH
- Middle Aged MeSH
- Humans MeSH
- Retrospective Studies MeSH
- Stevens-Johnson Syndrome * diagnosis epidemiology etiology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
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.
Azrieli Faculty of Medicine Bar Ilan University Safed Israel
Carol Davila University of Medicine and Pharmacy Bucharest Romania
Department of Dermatology Allergology and Venereology University of Helsinki Helsinki Finland
Department of Dermatology Inserm U519 Rouen University Hospital Rouen France
Department of Dermatology King's College Hospital London United Kingdom
Department of Dermatology Saint André Hospital Bordeaux France
Department of Dermatology University Hospital Munich University of Ludwig Maximilian Munich Germany
Department of Dermatology University Hospital of Zurich Zurich Switzerland
Department of Dermatology University Medical Center Schleswig Holstein Lübeck Germany
Department of Pediatric Dermatology Colentina Clinical Hospital Bucharest Romania
Department of Skin and Venereal Diseases Lithuanian University of Health Sciences Kaunas Lithuania
Dermatology Department AP HP Henri Mondor Hospital Créteil France
Faculty of Medicine University of Zurich Zurich Switzerland
Helsinki University Hospital Inflammation Center Helsinki Finland
Intensive care unit AP HP Henri Mondor Hospital Créteil France
Lübeck Institute of Experimental Dermatology University of Lübeck Lübeck Germany
Medical Campus Davos Davos Switzerland
Rare Disease Unit 1 Dermatology Division Istituto Dermopatico dell'Immacolata IRCCS Rome Italy
ToxiTEN group European Reference Network for Rare Skin Diseases Paris France
References provided by Crossref.org
- 000
- 00000naa a2200000 a 4500
- 001
- bmc22012158
- 003
- CZ-PrNML
- 005
- 20220506131038.0
- 007
- ta
- 008
- 220425s2021 xxu f 000 0|eng||
- 009
- AR
- 024 7_
- $a 10.1001/jamadermatol.2021.3154 $2 doi
- 035 __
- $a (PubMed)34431984
- 040 __
- $a ABA008 $b cze $d ABA008 $e AACR2
- 041 0_
- $a eng
- 044 __
- $a xxu
- 100 1_
- $a Kridin, Khalaf $u Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany $u Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel
- 245 10
- $a Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Insights From a Pan-European Multicenter Study / $c K. Kridin, MC. Brüggen, SL. Chua, A. Bygum, S. Walsh, MC. Nägeli, V. Kucinskiene, L. French, F. Tétart, B. Didona, B. Milpied, A. Ranki, C. Salavastru, E. Brezinová, S. Divani-Patel, T. Lorentzen, JL. Nagel, S. Valiukeviciene, V. Karpaviciute, GS. Tiplica, E. Oppel, A. Oschmann, N. de Prost, A. Vorobyev, S. Ingen-Housz-Oro
- 520 9_
- $a 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.
- 650 _2
- $a dospělí $7 D000328
- 650 _2
- $a kohortové studie $7 D015331
- 650 _2
- $a cyklosporin $x terapeutické užití $7 D016572
- 650 _2
- $a ženské pohlaví $7 D005260
- 650 _2
- $a lidé $7 D006801
- 650 _2
- $a intravenózní imunoglobuliny $x terapeutické užití $7 D016756
- 650 _2
- $a lidé středního věku $7 D008875
- 650 _2
- $a retrospektivní studie $7 D012189
- 650 12
- $a Stevensův-Johnsonův syndrom $x diagnóza $x epidemiologie $x etiologie $7 D013262
- 655 _2
- $a časopisecké články $7 D016428
- 655 _2
- $a multicentrická studie $7 D016448
- 700 1_
- $a Brüggen, Marie-Charlotte $u Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland $u Faculty of Medicine, University of Zurich, Zurich, Switzerland $u Medical Campus Davos, Davos, Switzerland $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France
- 700 1_
- $a Chua, Ser-Ling $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Department of Dermatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom
- 700 1_
- $a Bygum, Anette $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Department of Dermatology and Allergy Center, Odense University Hospital, Clinical Institute, University of Southern Denmark, Odense, Denmark
- 700 1_
- $a Walsh, Sarah $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Department of Dermatology, King's College Hospital, London, United Kingdom
- 700 1_
- $a Nägeli, Mirjam C $u Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland $u Faculty of Medicine, University of Zurich, Zurich, Switzerland
- 700 1_
- $a Kucinskiene, Vesta $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Department of Skin and Venereal Diseases, Lithuanian University of Health Sciences (LUHS), Hospital of LUHS Kauno Klinikos, European Reference Network for Rare and Complex Diseases of the Skin members, Kaunas, Lithuania $u Department of Skin and Venereal Diseases, Lithuanian University of Health Sciences, Kaunas, Lithuania
- 700 1_
- $a French, Lars $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Department of Dermatology, University Hospital, Munich University of Ludwig Maximilian, Munich, Germany $u Dr Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida
- 700 1_
- $a Tétart, Florence $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Toxic Bullous Dermatoses and Severe Drug Reactions reference center, TOXIBUL FIMARAD network, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France $u Department of Dermatology, Inserm U519, Rouen University Hospital, Rouen, France
- 700 1_
- $a Didona, Biagio $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Rare Disease Unit, I Dermatology Division, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy
- 700 1_
- $a Milpied, Brigitte $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Toxic Bullous Dermatoses and Severe Drug Reactions reference center, TOXIBUL FIMARAD network, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France $u Department of Dermatology, Saint André Hospital, Bordeaux, France
- 700 1_
- $a Ranki, Annamari $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Department of Dermatology, Allergology and Venereology, University of Helsinki, Helsinki, Finland $u Helsinki University Hospital, Inflammation Center, Helsinki, Finland
- 700 1_
- $a Salavastru, Carmen $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Carol Davila University of Medicine and Pharmacy, Bucharest, Romania $u Department of Pediatric Dermatology, Colentina Clinical Hospital, Bucharest, Romania
- 700 1_
- $a Brezinová, Eva $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u First Department of Dermatovenereology, Masaryk University Faculty of Medicine, St Ann's Faculty Hospital in Brno, Brno, Czech Republic
- 700 1_
- $a Divani-Patel, Sapna $u Department of Dermatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom
- 700 1_
- $a Lorentzen, Tine $u Department of Dermatology and Allergy Center, Odense University Hospital, Clinical Institute, University of Southern Denmark, Odense, Denmark
- 700 1_
- $a Nagel, Julie Loft $u Department of Dermatology and Allergy Center, Odense University Hospital, Clinical Institute, University of Southern Denmark, Odense, Denmark
- 700 1_
- $a Valiukeviciene, Skaidra $u Department of Skin and Venereal Diseases, Lithuanian University of Health Sciences (LUHS), Hospital of LUHS Kauno Klinikos, European Reference Network for Rare and Complex Diseases of the Skin members, Kaunas, Lithuania $u Department of Skin and Venereal Diseases, Lithuanian University of Health Sciences, Kaunas, Lithuania
- 700 1_
- $a Karpaviciute, Viktorija $u Department of Skin and Venereal Diseases, Lithuanian University of Health Sciences, Kaunas, Lithuania
- 700 1_
- $a Tiplica, George-Sorin $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Carol Davila University of Medicine and Pharmacy, Bucharest, Romania $u Department of Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
- 700 1_
- $a Oppel, Eva $u Department of Dermatology, University Hospital, Munich University of Ludwig Maximilian, Munich, Germany
- 700 1_
- $a Oschmann, Anna $u Department of Dermatology, University Hospital, Munich University of Ludwig Maximilian, Munich, Germany
- 700 1_
- $a de Prost, Nicolas $u Toxic Bullous Dermatoses and Severe Drug Reactions reference center, TOXIBUL FIMARAD network, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France $u Intensive care unit, AP-HP, Henri Mondor Hospital, Créteil, France
- 700 1_
- $a Vorobyev, Artem $u Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Department of Dermatology, University Medical Center Schleswig-Holstein, Lübeck, Germany
- 700 1_
- $a Ingen-Housz-Oro, Saskia $u ToxiTEN group, European Reference Network for Rare Skin Diseases, Paris, France $u Toxic Bullous Dermatoses and Severe Drug Reactions reference center, TOXIBUL FIMARAD network, Assistance Publique-Hôpitaux de Paris, Henri Mondor Hospital, Créteil, France $u Dermatology Department, AP-HP, Henri Mondor Hospital, Créteil, France $u University Paris-Est Créteil EpiDermE, Créteil, France
- 773 0_
- $w MED00180925 $t JAMA dermatology $x 2168-6084 $g Roč. 157, č. 10 (2021), s. 1182-1190
- 856 41
- $u https://pubmed.ncbi.nlm.nih.gov/34431984 $y Pubmed
- 910 __
- $a ABA008 $b sig $c sign $y p $z 0
- 990 __
- $a 20220425 $b ABA008
- 991 __
- $a 20220506131030 $b ABA008
- 999 __
- $a ok $b bmc $g 1789659 $s 1163359
- BAS __
- $a 3
- BAS __
- $a PreBMC
- BMC __
- $a 2021 $b 157 $c 10 $d 1182-1190 $e 20211001 $i 2168-6084 $m JAMA dermatology $n JAMA Dermatol $x MED00180925
- LZP __
- $a Pubmed-20220425
