Stevens-Johnson Syn (SJS)


Overview

SJS is a spectrum of rare, severe mucocutaneous drug reactions with an estimated incidence of 1 to 6 cases per million people per year.[1]

From a review 2022 [1]

    No. of studies overall    9
    No. of RCTs    3
No. of observational studies    6
Study years    1997-2018
No. of patients    308
Participants children    23
    Sex Male    131
    Female    155
    Countries     7 (China, Belgium, France, Greece, India, Spain, Taiwan)
        

   

    
    
 


Treatment 

  • Systemic corticosteroids
  • tumor necrosis factor-α inhibitors
  •  cyclosporine
  •  thalidomide
  •  N-acetylcysteine 
  • intravenous immunoglobulin
  • supportive care 

 Primary outcomes: 
  •  Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)–specific mortality 
  •  Adverse effects leading to discontinuation of SJS/TEN therapy
 Secondary outcomes: 
  • Time to complete re-epithelialization
  • , intensive care unit length of stay
  • , total hospital length of stay
  • , illness sequelae, 
  • other adverse effects attributed to systemic therapy 
 Summary of Findings This Cochrane systematic review analyzed the effects of systemic therapies for the treatment of SJS/TEN, including RCTs and prospective observational comparative studies. The available evidence was limited; 9 studies were included, with a total of 308 patients. The authors used the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology, a global standard for examining the level of evidence in systematic reviews. The evidence for potential benefit of etanercept vs corticosteroids came from a single unblinded RCT with 91 participants from Taiwan. The drugs were dosed as etanercept, 25 mg twice weekly (50 mg twice weekly if weight >65 kg), and intravenous prednisolone, 1 to 1.5 mg/kg/d until skin lesions healed. While the point estimate favored etanercept, there was no significant difference in mortality between the etanercept and corticosteroid treatment groups (8.3% vs 16.3%; P = .27; risk ratio, 0.51; 95% CI, 0.16-1.63). The GRADE certainty of the level of evidence for this comparison was “low.”5 Based on available evidence, it is uncertain if there is any difference between 3 other treatment comparisons in SJS/TEN-specific mortality. The evidence for corticosteroids vs supportive care was based on 2 studies with a total of 56 participants. The evidence for intravenous immunoglobulin (IVIG) vs supportive care was based on 1 study with 36 participants. The final comparison, cyclosporine vs IVIG, was based on a single study with 22 participants. The GRADE certainty of the level of evidence for all 3 comparisons was “very low,” meaning the Cochrane review authors had very little confidence in the effect estimate, and the true effect is likely to be substantially different. [1]

Clinical Question What are the effects of systemic therapies for Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)? Bottom Line There is limited high-quality evidence to support the use of systemic immunomodulatory therapies to decrease mortality rates in SJS/TEN.[1]

Prognosis

an overall mortality of 20% to 25%[1]
[1] Noe MH, Micheletti RG. Systemic Interventions for Treatment of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Summary of a Cochrane Review. JAMA Dermatol. 2022;158(12):1436–1437. doi:10.1001/jamadermatol.2022.4543