SJS/TEN 2019: From science to translation

Abstract

Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are potentially lifethreatening, immune-mediated adverse reactions characterized by widespread erythema, epidermal necrosis, and detachment of skin and mucosa. Efforts to grow and develop functional international collaborations and a multidisciplinary interactive network focusing on SJS/TEN as an uncommon but high burden disease will be necessary to improve efforts in prevention, early diagnosis and improved acute and long-term management. SJS/TEN 2019: From Science to Translation was a 1.5-day scientific program held April 26-27, 2019, in Vancouver, Canada. The meeting successfully engaged clinicians, researchers, and patients and conducted many productive discussions on research and patient care needs.

Stevens‐Johnson syndrome and toxic epidermal necrolysis associated with the use of macrolide antibiotics: a review of published cases

“…10 CD8 + cytotoxic T lymphocytes, that recognize HLA-drug epitopes, along with natural killer (NK) and NK T cells infiltrate skin lesions and secrete cytolytic proteins/chemokine mediators (e.g., granulysin), leading to the disseminated keratinocyte apoptosis in SJS/TEN. 10 Studies conducted in various patient populations indicate that the risk of SJS/TEN is highest when the drug has been recently initiated and subsequently declines within 8 weeks or more of administration. 49 Drugs which have been started in the 4 weeks before the onset without any previous use are most likely to cause SJS/TEN.…”

“…49 The clinical approach to the management of SJS/TEN should be multidisciplinary. 10 Prompt withdrawal of the culprit drug should be a priority, considering that its discontinuation is associated with a better prognosis, and every day of delay also worsens the outcomes. 10,49 The management should be undertaken in specialized centers capable of complex skin care and appropriate intensive care for more severe cases, like dermatology departments or burn units.…”

“…10 Prompt withdrawal of the culprit drug should be a priority, considering that its discontinuation is associated with a better prognosis, and every day of delay also worsens the outcomes. 10,49 The management should be undertaken in specialized centers capable of complex skin care and appropriate intensive care for more severe cases, like dermatology departments or burn units. 10 Since the pathophysiologic mechanism of SJS/TEN remains unknown, the treatment approach is primarily supportive and symptom targeted.…”

“…10,49 The management should be undertaken in specialized centers capable of complex skin care and appropriate intensive care for more severe cases, like dermatology departments or burn units. 10 Since the pathophysiologic mechanism of SJS/TEN remains unknown, the treatment approach is primarily supportive and symptom targeted. 8 Acute active management is controversial, and there is little consensus on medical interventions because of the lack of high-level evidence that any treatment (such as systemic corticosteroids and IVIG) is superior to supportive care alone.…”

“…9 Global clinical and financial burden of SJS/ TEN is considerable, resulting in prolonged hospital stays, mortality, and considerable long-term multisystem physical and mental health morbidity. 10 As previous review articles have mostly focused on adverse effects of macrolide antibiotics in general 1,11 and allergic reactions caused by macrolide antibiotics, 2 the aim of this review was to investigate and consolidate information regarding the characteristics of published cases of SJS/TEN suspected to be associated with the use of macrolide antibiotics in order to give The following data were extracted for each described case: demographics (age, gender), medical history, information about the macrolide antibiotic suspected to be associated with the occurrence of SJS/TEN (generic name of the drug, dosage, indication), concomitant medications, diagnosis of the reaction (SJS, TEN, SJS/TEN overlap), severity-of-illness score for toxic epidermal necrolysis (SCORTEN) if reported, diagnostic investigations (e.g., physical examination, skin biopsy, patch test), time to onset of the reaction (from the first dose of a macrolide antibiotic to the first symptoms of SJS/TEN, including prodromal symptoms), clinical manifestations, complications, sequelae, treatment, length of stay in hospital, outcome, algorithm for assessment of drug causality for epidermal necrolysis (ALDEN) if reported, and Naranjo score 12 for assessing the likelihood of causality of the association described if reported (the adverse drug reaction is assigned to a probability category based on the total Naranjo score as follows: "definite" ≥9, "probable" 5-8, "possible" 1-4, and "doubtful" ≤0).…”