Table 1.
Characteristics of all studies included in this review
| Study | Study design | Study sample (N) | Country | Total BSA and SCORTEN | Treatment regimen (n) | Conclusion | |||
|---|---|---|---|---|---|---|---|---|---|
| Arévalo et al, 200017 | Retrospective case series | 17 | Spain | Mean total BSA 83±17%; SCORTEN not specified | • CsA 3 mg/kg/d enterally every 12 hours, for 2 weeks and then tapered gradually (11) • Cyclophosphamide (150 mg IV every 12 hours) and different doses of corticosteroids (≥1 mg/kg/d of 6-methyl-prednisolone) (6) |
CsA is safe and is associated with rapid re-epithelization and a lower rate of multi-organ failure, severe leukopenia, and death than treatment with cyclophosphamide and corticosteroids in patients with severe TEN | |||
| Firoz et al, 201218 | Prospective | 82* | USA | Mean total BSA 34.8±26.1%; mean SCORTEN 2.17 |
• CsA (regimen not specified) only used in patients with low BSA and SCORTEN of 0–1 (8) • IVIg 4 g/kg divided over 3 days if patients presented within 72 hours of blistering (23) • Supportive care for patients who presented ≥3 days of blistering (51) |
No significant difference in survival among all three treatment options (P=0.15, log-rank test). IVIg did not significantly alter mortality | |||
| Giudice et al, 201719 | Retrospective case series | 12 | Italy | Mean total BSA 76.7±12.3%;mean SCORTEN 4.3 |
Standardized treatment protocol: CsA IV 250 mg/d or 4 mg/kg/d in pediatric patients on day one, at day three, daptomycin and plasmapheresis were introduced. CsA continued for 15 days, daptomycin for 10 days, plasmapheresis consisted of 7 cycles spaced by 2 days each (12) | Standardized treatment protocol consisting of CsA and plasmapheresis is safe and efficacious in patients with severe TEN | |||
| González-Herrada et al, 201720 | Retrospective and prospective cases | 42 | Spain | Mean total BSA 43.5±26.9%; mean SCORTEN 2.39 |
• CsA 3 mg/kg/d until complete re-epithelialization and then gradual taper (26) • IVIg 0.75 g/kg/d for 4 days (11) • Prednisone-equivalent 37.5–100 mg/d for 9–12 days (2) • Supportive care (3) |
CsA offers mortality benefit for SJS/TEN patients | |||
| Kirchhof et al, 201410 | Retrospective case series | 64 | Canada | Mean total BSA 28.7±26.6%; mean SCORTEN 1.65 |
• Supportive care (12) • IVIg 1 g/kg/d for 3 days (35) • CsA 3–5 mg/kg/d orally or IV for an average of 7 days (15) • IVIg and CsA (2) |
Relative mortality benefit of CsA (SMR 0.42) over IVIg (SMR 1.43) in patients with SJS/TEN | |||
| Lee et al, 201721 | Retrospective case series | 44 | Singapore | Mean total BSA 29±25%; mean SCORTEN 2.5 |
• CsA 3 mg/kg/d for 10 days, then 2 mg/kg/d for 10 days, and finally 1 mg/kg/d for 10 days (24) • Supportive care (20) |
Relative mortality benefit of CsA (SMR 0.42) over supportive care (SMR 1.02) | |||
| Mohanty et al, 201722 | Retrospective case series | 28 | India | Mean total BSA 35.95±20.33%; mean SCORTEN 2.05 | • CsA 5 mg/kg/d in three divided doses for 10 days, along with supportivecare (19) • Supportive care (9) |
SMR of CsA group (0.32) nearly 3.3 times lower than the only supportive treatment group (1.06) | |||
| Rajaratnam et al, 201023 | Retrospective case series | 21 | UK | Mean total BSA 44%; mean SCORTEN 3.1 |
• CsA IV 2.5–4 mg/kg/d for 3–5 days (3) • IVIg 0.4–1.0 g/kg/d for 3–7 days (14) • Cyclophosphamide IV 2.5 mg/kg/for 3 days (2) |
Corticosteroids did not appear beneficial compared to IVIg or CsA | |||
| Reese et al, 20119 | Retrospective case series | 4 | USA | Mean total BSA 35.8%; mean SCORTEN 1.25 |
• CsA 5 mg/kg/d in two divided doses for 5 days to a month (4) | CsA is efficacious with rapid response and re-epithelization. Short-term use of CsA did not have adverse reactions or increased infections | |||
| Singh et al, 201311 | Retrospective case series | 11 | India | Mean total BSA 23.4±16.3%; mean SCORTEN 1.45 |
• CsA 3 mg/kg/d in three divided doses for 7 days, then 2 mg/kg/d in two divided doses for another 7 days (11) | Faster re-epithelization, shorter hospital stay and relative mortality benefit of CsA over corticosteroids. CsA was also well tolerated by all the patients | |||
| Szepietowski et al, 199724 | Retrospective case series | 3 | Poland | Not specified | • CsA 8–10 mg/kg/d for 10–21 days and corticosteroids (3) | Combined and monotherapy with CsA appear superior to monotherapy with corticosteroids. CsA is beneficial for TEN patients | |||
| Valeyrie-Allanore et al, 201025 | Open, Phase II trial | 29 | France | Mean total BSA 12.2±8.2%; mean SCORTEN 1.27 |
• CsA orally through NG tube, 1.5 mg/kg twice daily for 10 days, then 1 mg/kg twice daily for 10 days, and finally 0.5 mg/kg twice daily for 10 days (29) | CsA was well tolerated; 26 out of 29 patients completed the 1-month treatment. Lower than expected mortality and disease progression observed | |||
Note:
*
Expression of concern by journal editor and staff over possible data irregularities.
Abbreviations: BSA, body surface area; CsA, cyclosporine; IV, intravenous; IVIg, intravenous immunoglobulin; NG, nasogastric; SCORTEN, SCORe of toxic epidermal necrosis; SJS, Stevens–Johnson syndrome; SMR, standardized mortality ratio; TEN, toxic epidermal necrolysis.