Table 1.
IVIG and systemic corticosteroids therapy for SJS/TEN in the world.
| References | Study design | Sample size and classification, study period | Treatment | Author conclusion |
|---|---|---|---|---|
| IVIG therapy for SJS/TEN | ||||
| Viard et al. (10) | Prospective, open, uncontrolled, multicenter | 1 SJS, 4 SJS/TEN overlap, 5 TEN, N/A | IVIG (0.2~0.75 g/kg per day for 4 consecutive days) | Effective, response within 24–48 h |
| Bachot et al. (22) | Prospective non-comparative SCORTEN based comparison | 9 SJS, 5 SJS/TEN overlap, 20 TEN, 1999~2000 | IVIG (total dose of 2 g/kg within 2 days) | Ineffective |
| Tran and Sidhu (23) | Retrospective chart review | 18 SJS, 6 SJS/TEN overlap, 18 TEN, 2000~2009, 2010~2017 | (1) Skin and supportive, (2) Oral corticosteroids (3) IVIG alone (4) Oral corticosteroids and IVIG IVIG doses (1–3 g/kg/day) |
Effective, improvement in mortality in IVIG groups |
| Lee et al. (24) | Retrospective, a single referral center | 28 SJS/TEN overlap, 36 TEN1. 2003-12, 2010 | IVIG dosage (1) <3 g/kg (2) larger than 3 g/kg | Ineffective in mortality between dosage |
| Antoon et al. (25) | Retrospective Cohort Study, multicenter data from the Pediatric Health Information System |
774 SJS, 124 TEN(IVIG only: 56 TEN)(IVIG and steroid: 15 TEN)(Steroid only: 12 TEN)2008~2015 | 167 steroids only, 229 IVIG only, 153 both IVIG and steroids |
Ineffective, bias in distribution of severity |
| Yang et al. (26) | Retrospective, SCORTEN based comparison | (1) 10 SJS, 35 TEN, 1993~2001 (2) 8 SJS, 12 TEN, 2001~2007 | (1) 1–1.5 mg/kg/day methylprednisolone (2) 2 g/kg of IVIG (0.4 g/kg/day for 5 days) with a combination of corticosteroids | Effective in mortality, disease progression and time of hospitalization in combined group |
| Chan and Cook (27) | Retrospective, a single referral center | 10 SJS, 6 SJS/TEN overlap, 26 TEN, 2006~2016, | (1) Skin and supportive 3 SJS, 1 SJS/TEN, 2 TEN (2) Corticosteroids alone 2 SJS, 0 SJS/TEN, 5 TEN (3) IVIG alone 1 SJS, 4 SJS/TEN, 11 TEN (4) corticosteroids and IVIG 4 SJS, 1 SJS/TEN, 8 TEN |
Effective in mortality in combined group |
| Micheletti et al. (15) | Retrospective, multicenter of 18 academic medical centers | 110 SJS, 158 SJS/TEN overlap, 79 TEN, 2000~2015 | (1) Skin and supportive, (2) Corticosteroids, mean (148 mg prednisone for 9.8 days) (3) IVIG alone, mean (1 g/kg/day for 3 days) (4) Oral corticosteroids and IVIG (5) Cyclosporine or tumor necrosis factor inhibitor |
Ineffective. However, concluded co-administration of corticosteroids and IVIG deserving further prospective trials |
| Yang et al. (28) | Retrospective, SCORTEN based comparison, a single referral center | 141 SJS, 19 SJS/TEN overlap, 53 TEN, 2008~2018 | Systemic corticosteroids and IVIG (mainly 0.4 g/kg/day for 5 days) | Effective in mortality without significance |
| Pham et al. (20) | Retrospective, a single institution | 13 SJS/TEN, N/A | (1) Etanercept alone (2) IVIG for 3 days and Etanercept (3) Without Etanercept (Etanercept 50 mg) |
Effective under Etanercept treatment without statistical significance |
| An open-label, multicenter, single-arm study of IVIG therapy in Japan | ||||
| Aihara et al. (29) | Prospective, open-label, multicenter, single-arm study | 5 SJS, 3 TEN, N/A | Systemic corticosteroids and IVIG (400 mg/kg/day for 5 consecutive days, total 2 g/kg) | Effective without mortality in all patients |
| Systemic corticosteroid therapy for SJS/TEN | ||||
| Yamane et al. (5) | Retrospective, two university hospitals | 52 SJS, 35 TEN, 2000~2013 | Steroid pulse therapy in combination with plasmapheresis and/or IVIG | Effective, lower than SCORTEN based mortality |
| Liu et al. (14) | Retrospective, SCORTEN based comparison, a single referral center | 18 SJS, 23 SJS/TEN overlap, 29 TEN, 2008~2015 | (1) Low-dose group (≤ 2 mg/kg/d) (2) High-dose group >2 mg/kg/d (5 mg prednisone or 4 mg methylprednisolone or 5 mg hydroprednisone or 0.75 mg dexamethasone). | Supporting the use of systemic corticosteroids for SJS/TEN. |
| Araki et al. (16) | Prospective, observational case series | 4 SJS, 1 TEN, N/A | Steroid pulse therapy; 500 or 1000 mg/day for 3 to 4 days. Additional steroid (prednisolone 40~60 mg/day) | Effective, early steroid pulse therapy improving ocular symptoms |
| Hirahara et al. (30) | Retrospective | 3 SJS, 2 SJS/TEN overlap, 3 TEN, 2008~2015 | Methylprednisolone pulse therapy (1,000 mg/d for 3 consecutive days), Oral prednisolone at 0.8–1 mg/kg/d | Effective, reduction in the mean levels of IFN-γ, TNF-α, and IL-6 |
| Watanabe et al. (31) | Retrospective | 75 SJS, 53 TEN, 2000~2019 | Methylprednisolone pulse therapy (500–1,000 mg/day of methylprednisolone for 3 days), prednisolone equivalent 1 mg/kg/day, part of patients combined with IVIG and/or plasmapheresis | Effective, the mortality rate are lower than the global mortality rates |
| Sunaga et al. (32) | Retrospective, a nationwide survey in Japan,160 institutions | 315 SJS, 174 TEN, 2016~2018 | (1) 37.8 % high-dose steroid alone (2) 29.2% pulse therapy followed by tapering (3) 11.7% high-dose steroid plus IVIG (4) 13.7% steroid pulse therapy plus IVIG (5) High-dose steroid (0.80–1.21 mg/kg) (6) IVIG (0.36–0.43 g/kg for 5 days) | Effective in mortality in high-dose steroid followed by pulse group |
IVIG, intravenous immunoglobulin; N /A, no data; SJS, Stevens–Johnson syndrome; TEN, toxic epidermal necrolysis; SJS /TEN, SJS-TEN overlap. The classification was made according to the original study. If there was no classification in the study, we classified according to the classification of Roujeau and Stern, 1999 from the raw data as SJS:detachment of TBSA <10%, 10% < SJS /TEN <30%, and <30%: TEN.
Review articles were not listed in this table.