What is the role of systemic corticosteroids (steroids) in the treatment of Steven Johnson Syndrome (SJS)?

Role of Systemic Corticosteroids in Stevens-Johnson Syndrome

Systemic corticosteroids may be beneficial in Stevens-Johnson Syndrome (SJS) when administered early in the disease course, but their use remains controversial due to potential increased risk of infection. 1

Evidence Assessment

Corticosteroid Efficacy

• Retrospective analysis from EuroSCAR data showed lower mortality in German patients (but not French patients) treated with corticosteroids compared to supportive care alone 1
• Two studies of high-dose pulsed IV corticosteroids demonstrated decreased mortality compared to SCORTEN predictions:
  • Kardaun's study: IV dexamethasone (100 mg or 1.5 mg/kg) for 3 days 1
  • Hirahara's study: IV methylprednisolone (1000 mg) for 3 consecutive days, followed by tapering 1
• A meta-analysis including 96 studies and 3248 patients suggested a survival benefit with glucocorticosteroids, though this was significant in only one of three statistical analyses 1

Timing and Dosing

• Early administration appears crucial for effectiveness 1, 2
• Recommended dosing regimens:
  • Methylprednisolone: 40-80 mg daily for moderate cases; pulse therapy with IV methylprednisolone (1000 mg) for 3 consecutive days in severe cases 3
  • Prednisone: 1-2 mg/kg/day with rapid taper for moderate disease 3

Treatment Algorithm Based on Disease Severity

1. Mild SJS (limited skin involvement):

   • Topical corticosteroids to affected areas
   • Supportive care
   • Close monitoring for progression

2. Moderate SJS (10-30% skin involvement):

   • Oral prednisolone 1-2 mg/kg/day with rapid taper
   • Topical corticosteroids to mucosal surfaces
   • Apply potent topical corticosteroid ointment once daily to involved, non-eroded urogenital surfaces 3

3. Severe SJS/TEN (>30% skin involvement):

   • IV methylprednisolone pulse therapy (1000 mg daily for 3 days)
   • Consider specialized burn unit or intensive care setting
   • Consider adjunctive therapies (ciclosporin or etanercept) in combination with corticosteroids 4

Risks and Monitoring

• Primary concern: Increased risk of infection, especially sepsis 1
• Monitoring requirements:
  • Daily examination of affected mucosal surfaces
  • Regular screening for signs of secondary infection
  • Blood pressure monitoring
  • Glycemic control
  • Serum potassium levels 3

Emerging Evidence

Recent research suggests that combination therapy with etanercept and corticosteroids may be more effective than corticosteroid monotherapy:

• Lower actual mortality (0% vs 6.63%)
• Reduced skin healing time (12.0 days vs 13.0 days)
• Lower incidence of adverse events, particularly gastrointestinal hemorrhage in TEN patients 4

Clinical Pitfalls to Avoid

1. Delayed treatment initiation: Early administration of corticosteroids appears crucial for effectiveness 2
2. Inadequate dosing: Insufficient corticosteroid doses may not effectively suppress the inflammatory cascade
3. Abrupt discontinuation: Can lead to withdrawal syndrome (weakness, nausea, arthralgia) 3
4. Neglecting supportive care: Corticosteroids should complement, not replace, comprehensive supportive management
5. Failing to monitor for complications: Regular screening for infection and metabolic derangements is essential

While the evidence for systemic corticosteroids in SJS remains somewhat equivocal, early administration of high-dose corticosteroids appears to offer benefit in reducing mortality and morbidity when initiated promptly, particularly in more severe cases. The decision to use systemic corticosteroids should be made with careful consideration of the potential benefits versus risks of infection.