Treating toxic epidermal necrolysis with systemic immunomodulating therapies: A systematic review and network meta-analysis

Tsai, Tsung-Ting; Huang, I‐Hsin; Chao, Yuan-Chen; Li, Hua; Hsieh, Tyng‐Shiuan; Wang, Hsiao-Han; Huang, Yuting; Chen, Chun-Yuan; Cheng, Ying‐Chih; Kuo, Po-Hsiu; Huang, Yu‐Chen; Tu, Yu–Kang

doi:10.1016/j.jaad.2020.08.122

Cited by 73 publications

(70 citation statements)

References 26 publications

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“…In another study, Tsai et al [ 90 ] analyzed treatment outcomes of a number of therapies and performed a meta-analysis of 67 studies involving 2079 patients. The authors only examined the mortality outcomes of patients with SJS/TEN overlap and TEN, choosing to exclude SJS outcomes because mortality is typically lower.…”

Section: Clinical Updatesmentioning

confidence: 99%

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

et al. 2021

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Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. In this scoping review, Excerpta Medica dataBASE and PubMed were searched for publications that addressed recent advances in the diagnosis and management of the disease. Multiple proteins (galectin 7 and RIP3) were identified that are promising potential biomarkers for SJS/TEN, although both are still in early phases of research. Regarding treatment, cyclosporine is the most effective therapy for the treatment of SJS, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN. Due to the rare nature of the disease, there is a lack of prospective, randomized controlled trials and conducting these in the future would provide valuable insights into the management of this disease.

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Section: Clinical Updatesmentioning

confidence: 99%

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

et al. 2021

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“…A study by Wang et al [23] found etanercept was superior than systemic corticosteroids in the treatment of SJS/TEN. A recent systematic review and meta-analysis of 67 studies and 2079 patients showed that combination therapy with corticosteroids and IVIg could reduce the risk of mortality in patients with SJS/TEN; while cyclosporine and etanercept are promising therapies but more studies are needed to provide clearer evidence [24]. In our study, IVIg was used with or without corticosteroids in approximately one fourth of the patients successfully.…”

Section: Treatment Protocolmentioning

confidence: 58%

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Retrospective Analysis of 23 Patients

Yalıcı-Armağan

Demircan²,

Akdoğan

et al. 2021

Acta Medica

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Objective: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are dermatologic emergencies. There is a lack of consensus regarding appropriate management of SJS/TEN. The aim of this study was to evaluate demographic and clinical features, management and outcomes of SJS/TEN patients. Materials and Methods: The data of patients who were ≥18 years old and hospitalized with the diagnosis of SJS, SJS-TEN overlap and TEN at Hacettepe University, between 1992 and 2018 were analyzed retrospectively. Patient demographics, medications, time between the first causative drug intake and the onset of symptoms, mucous membrane involvement, treatment modalities including supportive measures, intravenous immunoglobulin, cyclosporine and systemic corticosteroids, duration of hospitalization, and mortality outcomes were recorded from patient charts. Results: A total of 23 patients (11 men; 12 women) with a mean age of 46.4 ± 19.5 years were included in the study. Twelve patients (52.2%) had SJS, 8 patients (34.8%) had SJS/TEN overlap and 3 patients (13%) had TEN. Mean duration of hospitalization was 12.6 ± 6.5 days. Twenty-two patients (95.7%) were attributed to a specific medication, whereas triggering factor was not detected in 1 patient (4.3%). More than one drug was responsible for 9 patients, including antimicrobials (n=11, 47.8%), anticonvulsants (n=8, 34.8%) and/or non-steroid anti-inflammatory drugs (n=5, 21.7%). The median time between the intake of medication and the onset of symptoms was 20 days (IQR 5.5-30). Sixteen patients used systemic corticosteroids (69.6%) and 4 patients used intravenous immunoglobulin (17.4%) whereas 3 patients used systemic corticosteroids in combination with intravenous immunoglobulin (n=2, 8.7%) or cyclosporin (n=1, 4.3%). The median time between the onset of symptoms and the onset of the rash to the treatment was 5 days (IQR 3-7). Mortality was not observed in our cohort. Conclusion: SJS/TEN was most commonly developed because of drugs, mainly antimicrobials, anticonvulsants and/or non-steroid anti-inflammatory drugs. The absence of mortality in our cohort was considered to be associated with younger age, low rate of TEN, prompt initiation of treatment (mainly corticosteroids) following the rapid discontinuation of the suspected drug.

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“…Recently, a significantly lower mortality rate in the steroid treatment group than the mortality rate predicted by the SCORTEN has been reported ( 14 ). Furthermore, there are multiple previous studies that showed the possible usefulness of combination therapy with corticosteroids and IVIG as mentioned above ( 15 , 26 – 28 , 33 , 38 ), even though steroid use alone did not show sufficient effects in those studies. Therefore, the therapeutic effect of steroids remains controversial.…”

Section: High-dose Systemic Corticosteroid Therapy For Sjs/tenmentioning

confidence: 98%

“…The latest network meta-analysis of review articles in the Journal of the American Academy of Dermatology evaluated 66 studies, involving 2,079 SJS/TEN overlap and TEN patients from 1999 to 2019. Although it was reported that none of the included systemic immunomodulating therapies reduced mortality rates in patients with SJS/TEN overlap and TEN, based on standardized mortality ratio, the combination of corticosteroids and IVIG significantly reduced the standardized mortality ratio and hinted at the synergistic effects of targeting different pathways ( 33 ).…”

Section: Ivig Therapy For Sjs/tenmentioning

confidence: 99%

Recent Dermatological Treatments for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Japan

2021

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Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions characterized by necrosis of the skin and mucus membranes, and are mainly caused by medication and infections. Although the exact pathomechanism of SJS/TEN remains unclear, keratinocyte death is thought to be triggered by immune reactions to these antigens. While there is no established therapy for SJS/TEN, corticosteroids and intravenous immunoglobulin (IVIG) have been utilized as immunomodulator. We previously conducted a study to evaluate the efficacy of IVIG therapy in Japanese patients with SJS/TEN. IVIG was administered at a dosage of 400 mg/kg/day for 5 consecutive days as an additional therapy with systemic steroids. Prompt amelioration was observed in seven of the eight patients. All patients survived without sequelae. Recently, we retrospectively analyzed 132 cases of SJS/TEN treated in our two hospitals. The mortality rates in the patients treated with methylprednisolone pulse were 0% (0/31) for SJS and 7.0% (3/43) for TEN, and 0% (0/10) in the TEN patients treated with methylprednisolone pulse in combination with IVIG. These results suggest that early treatment with high-dose steroids, including methylprednisolone pulse therapy, and IVIG together with corticosteroids are possible therapeutic options to improve the prognosis of SJS/TEN.

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Treating toxic epidermal necrolysis with systemic immunomodulating therapies: A systematic review and network meta-analysis

Cited by 73 publications

References 26 publications

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Retrospective Analysis of 23 Patients

Recent Dermatological Treatments for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Japan

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