IVIG for Stevens-Johnson Syndrome
IVIG is not recommended as standard therapy for Stevens-Johnson syndrome (SJS) based on the highest quality evidence showing no mortality benefit compared to supportive care alone. 1
Evidence Quality and Recommendations
The 2016 UK guidelines for management of SJS/TEN provide the most definitive guidance on this question. A meta-analysis of controlled studies found no survival benefit with IVIG, with a pooled odds ratio for mortality of 1.00 (95% CI 0.58-1.75; P = 0.99) when comparing IVIG to supportive care. 1 This represents level 3-4 evidence, which is insufficient to support routine use. 2
Key Findings from Guidelines
The overall mortality rate in 221 patients with TEN and SJS-TEN overlap treated with IVIG was 19.9%, showing no statistical improvement over supportive care. 1
High-quality supportive care remains the priority in SJS/TEN management regardless of specific interventions. 2
There is no active therapeutic regimen with unequivocal benefit for SJS/TEN. 2
Dosing Considerations If IVIG Is Used
Despite lack of proven efficacy, if IVIG is considered in severe or refractory cases, dosing matters significantly:
High-dose IVIG (total 2-3 g/kg over 3-5 days) shows better outcomes than low-dose regimens in adults. 2 Meta-regression demonstrated a strong inverse correlation between IVIG dosage and mortality (slope: -0.59,95% CI -0.14 to -1.03; P = 0.009). 3
Low-dose protocols (0.4 g/kg for 4 days) showed unfavorable outcomes with 42% mortality. 2
The standard protocol when used is 1 g/kg/day for 3 consecutive days (total 3 g/kg). 2
Population-Specific Considerations
Pediatric patients treated with IVIG had significantly lower mortality than adults (0% vs. 21.6%, P = 0.01). 1 However, this finding requires cautious interpretation as it comes from observational data with potential selection bias.
Important Caveat for Mycoplasma-Associated SJS
In Mycoplasma pneumoniae-associated SJS specifically, IVIG alone was associated with longer hospital stays and more febrile days compared to supportive care. 4 When systemic corticosteroids were used alone or with IVIG, outcomes trended better than IVIG monotherapy. 4 This suggests IVIG should not be used as monotherapy in this subset.
Ocular Outcomes
When given in the acute phase of SJS/TEN, there is no robust evidence for benefit of IVIG to improve ocular outcomes. 1 A retrospective study comparing 8 patients receiving IVIG to 8 historical controls found no difference in severity of visually significant ocular complications. 1
Safety Profile
IVIG may be safer than systemic corticosteroids, which carry concerns about increased infection risk. 2 However, adverse effects include headache, aseptic meningitis, thromboembolic events, renal dysfunction, and anaphylaxis (particularly in IgA-deficient patients). 1, 2
Clinical Algorithm
Given the evidence:
- Prioritize high-quality supportive care as the foundation of treatment 2
- Do not use IVIG as routine first-line therapy 1
- Consider high-dose IVIG (2-3 g/kg total) only in severe, life-threatening cases where benefits may outweigh costs and lack of proven efficacy 2, 3
- In Mycoplasma-associated SJS, avoid IVIG monotherapy; if immunomodulation is needed, consider corticosteroids instead 4
- In pediatric cases, IVIG may have a more favorable risk-benefit profile than in adults 1
The bottom line: supportive care remains the evidence-based standard, and IVIG should be reserved for exceptional circumstances with informed discussion about limited evidence of benefit. 1, 2