Treatment of Stevens-Johnson Syndrome with IVIG
IVIG should be considered as a treatment option for severe Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), particularly in steroid-unresponsive cases, with high-dose regimens (total 2-3 g/kg over 3-5 days) showing better outcomes than low-dose protocols. 1
Primary Treatment Approach
Immediate Management
- Discontinue the culprit drug immediately and admit to a burn unit or ICU with dermatology consultation 2
- Provide meticulous supportive care including fluid/electrolyte balance, wound care, and infection prevention 2
- Apply topical emollients (petrolatum-based), high-strength topical corticosteroids, and oral antihistamines 2
Systemic Immunomodulation
For Grade 3-4 SJS/TEN:
- First-line: IV methylprednisolone 0.5-1 mg/kg for Grade 3, or 1-2 mg/kg for Grade 4, tapering when toxicity resolves 2
- IVIG or cyclosporine may be added in severe or steroid-unresponsive cases 2
IVIG Dosing Protocols
High-Dose Regimen (Preferred)
- Total dose: 2-3 g/kg administered over 3-5 consecutive days 1
- Most successful protocols use 1 g/kg/day for 3 days 1
- Meta-regression analysis demonstrates a strong inverse correlation between IVIG dosage and mortality (slope: -0.59, P = 0.009), with doses ≥2 g/kg significantly decreasing mortality 3
Low-Dose Regimen (Less Favorable)
- Doses of 0.4 g/kg for 4 days showed mortality rates of 42%, indicating inferior outcomes 1
Evidence Quality and Limitations
Mixed Evidence Base
The evidence for IVIG remains controversial:
- No overall survival benefit was demonstrated in meta-analysis comparing IVIG to supportive care alone (OR 1.00,95% CI 0.58-1.75) 1
- However, high-dose IVIG (2-3 g/kg) has been associated with improved survival compared to low-dose regimens in adults 1
- A propensity-matched study showed IVIG combined with corticosteroids reduced time to arrest progression by 1.56 days (P = 0.000) and hospital stay by 3.37 days (P = 0.000) 4
Pediatric Considerations
- Pediatric patients treated with IVIG have significantly lower mortality than adults (0% vs. 21.6%) 1
- In a pediatric case series, fever duration was shortened from 14 days to 8 days with IVIG (P = 0.06), though statistical significance was marginal 5
Clinical Decision Algorithm
When to Use IVIG:
- Severe disease (Grade 4) with >10% BSA involvement and systemic symptoms 2
- Steroid-unresponsive cases after initial corticosteroid therapy 2
- Rapidly progressive disease despite supportive care 1
- Pediatric patients, given their superior outcomes with IVIG 1
When to Consider Alternatives:
- If IVIG unavailable or contraindicated, cyclosporine 3 mg/kg daily for 10 days (then tapered) is an alternative, showing effectiveness with no deaths despite SCORTEN-predicted mortality 6
Safety Monitoring During IVIG
Monitor for complications:
IVIG may be safer than systemic corticosteroids alone, which carry increased infection risk 1, 6
Important Caveats
Context-Specific Recommendations
- For immune checkpoint inhibitor-induced SJS/TEN, the usual prohibition of corticosteroids does not apply, as the mechanism is T-cell immune-directed toxicity requiring adequate immunosuppression 2
- UK guidelines note insufficient evidence to recommend IVIG routinely, suggesting it should only be administered under specialist supervision in the context of clinical research or case registry 2
Combination Therapy
- IVIG combined with corticosteroids showed 45% lower mortality compared to corticosteroids alone (though not statistically significant, P = 0.555) and significantly lower skin infection rates (P < 0.025) 4
- The total infection rate decreased by 67% with combination therapy (P = 0.047) 4
Multidisciplinary Consultations Required
Essential consultations for mucosal involvement:
- Ophthalmology (for ocular involvement) 2
- Otolaryngology (for oral/pharyngeal involvement) 2
- Urology/Gynecology (for genital involvement) 2
- Wound care services 2
High-quality supportive care remains the priority regardless of specific immunomodulatory interventions chosen 1, 6