Evaluation of Plasmapheresis vs Immunoglobulin as First Treatment After Ineffective Systemic Corticosteroid Therapy for Patients With Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Yuki Miyamoto, MD, MPH1,2; Hiroyuki Ohbe, MD, MPH2; Ryosuke Kumazawa, DPH2; et al

JAMA Dermatol. Published online March 8, 2023. doi:10.1001/jamadermatol.2023.0035

Key Points

Question Are the clinical outcomes of administering plasmapheresis therapy first better than those of administering intravenous immunoglobulin (IVIG) therapy first after ineffective systemic corticosteroid therapy in patients with Stevens-Johnson syndrome or toxic epidermal necrolysis (SJS/TEN)?

Findings This retrospective cohort study of 266 inpatients with SJS/TEN found no significant difference in mortality rates between the plasmapheresis-first and the IVIG-first groups. Patients who received plasmapheresis therapy first had longer hospitalization stays and incurred higher expenses.
Meaning The findings of this retrospective cohort study suggest that there is no clear benefit to administering plasmapheresis before IVIG therapy to patients with SJS/TEN unresponsive to systemic corticosteroids and plasmapheresis may be associated with higher cost and longer hospital stays.

Abstract
Importance Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are severe cutaneous adverse reactions, and patients with SJS/TEN frequently require intensive care. However, there is limited evidence on the clinical outcomes of immunomodulating therapy, including plasmapheresis and intravenous immunoglobulin (IVIG) in patients with SJS/TEN.
Objective To compare clinical outcomes of patients with SJS/TEN who were treated with plasmapheresis first vs IVIG first after ineffective systemic corticosteroid therapy.
Design, Setting, and Participants This retrospective cohort study used data from a national administrative claims database in Japan that included more than 1200 hospitals and was conducted from July 2010 to March 2019. Inpatients with SJS/TEN who received plasmapheresis and/or IVIG therapy after initiation of at least 1000 mg/d of methylprednisolone equivalent systemic corticosteroid therapy within 3 days of hospitalization were included. Data were analyzed from October 2020 to May 2021.
Exposures Patients who received IVIG or plasmapheresis therapy within 5 days after initiation of systemic corticosteroid therapy were included in the IVIG- and plasmapheresis-first groups, respectively.
Main Outcomes and Measures In-hospital mortality, length of hospital stay, and medical costs.
Results Of 1215 patients with SJS/TEN who had received at least 1000 mg/d of methylprednisolone equivalent within 3 days of hospitalization, 53 and 213 patients (mean [SD] age, 56.7 [20.2] years; 152 [57.1%] women) were included in the plasmapheresis- and IVIG-first groups, respectively. Propensity-score overlap weighting showed no significant difference in inpatient mortality rates between the plasmapheresis- and IVIG-first groups (18.3% vs 19.5%; odds ratio, 0.93; 95% CI, 0.38–2.23; P = .86). Compared with the IVIG-first group, the plasmapheresis-first group had a longer hospital stay (45.3 vs 32.8 days; difference, 12.5 days; 95% CI, 0.4–24.5 d; P = .04) and higher medical costs (US $34 262 vs $23 054; difference, US $11 207; 95% CI, $2789–$19 626; P = .009).
Conclusions and Relevance This nationwide retrospective cohort study found no significant benefit to administering plasmapheresis therapy first instead of IVIG first after ineffective systemic corticosteroid treatment in patients with SJS/TEN. However, medical costs and length of hospital stay were greater for the plasmapheresis-first group.