Combination of intravenous immunoglobulin and systemic corticosteroid versus corticosteroid monotherapy in Stevens-Johnson Syndrome (SJS) patients: a systematic review

Video Abstract: https://youtu.be/qU2IS7W1tIw Background: Stevens-Johnson syndrome (SJS) is a severe, acute mucocutaneous disorder primarily triggered by medications, marked by significant morbidity and mortality. Despite improved supportive care, therapeutic management of SJS remains controversial due to inconsistent evidence regarding the optimal immunomodulatory approach. Systemic corticosteroids and intravenous immunoglobulin (IVIG) are commonly employed treatments, either individually or in combination, yet consensus on their comparative efficacy and safety is lacking. This systematic review aims to clarify the clinical effectiveness and safety of combining IVIG with systemic corticosteroids compared to corticosteroid monotherapy in patients with SJS. Methods: A systematic literature search was conducted in accordance with PRISMA guidelines, utilizing electronic databases. The databases searched included PubMed, Scopus, Google Scholar, and the Cochrane Library. Included studies are clinical trials on SJS, SJS-TEN, and TENS. All studies were evaluated using the Newcastle-Ottawa scale. Result: Of the 385 studies in the initial search, only eight cohort studies were eligible for this review, representing the analysis of 481 patients worldwide. Disease severity was primarily assessed using the SCORTEN (Severity of Illness Score for Toxic Epidermal Necrolysis) system in most studies, with mean and median SCORTEN scores ranging from 1.63 to 3. Combination therapy was associated with trends toward lower mortality, significantly faster disease resolution, and shorter hospitalisation time across multiple studies. Combination therapy arrests disease progression earlier, potentially reducing the total dose of corticosteroid needed. All studies reported adverse events and complications, including infections and IVIG-related adverse events (such as fever and chills). Conclusion: The combination of IVIg and corticosteroids may offer superior advantages in terms of increased survival benefits compared to corticosteroid monotherapy.