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Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights

Oct 21, 2021 12:36 pm

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are among the most severe drug reactions, as they come with substantial mortality and morbidity.  A review of European centers dealing with SJS and TEN shows that despite best treatment practices, the 6-week mortality rate was 21%.

This retrospective review of 13 referral centers belonging to the ToxiTEN ERN-skin subgroup identified 212 adults with SJS/TEN between 2015 and 2019. They assess offending agents, treatments and outcomes.

Among SJS/TEN patients the mean body surface area detachment was 27% and primary culprit drugs were lead by antibiotics (21.2%), then anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%).

Treatment approaches included:

  • Supportive care only (38.2%)
  • Systemic glucocorticoids (35.4%)
  • Intravenous immunoglobulins (23.6%)
  • Cyclosporine (10.4%)
  • Tumor necrosis factor agents (3.3%).

Nearly two-thirds (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Acute complications was linked to body surface area detachment ≥30% (OR 2.49) and a SCORTEN greater than or equal to 2 was significantly mortality (OR, 10.30; P < .001).

Cyclosporine was associated with a >20% increase in body surface area detachment and an increased risk of infections (adjOR, 7.16). Glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40). Clearly the most severe patients received the most potent treatments.

The authors called for prospective therapeutic studies and registries to be developed.

The author has no conflicts of interest to disclose related to this subject

Rheumatologists’ Comments

Eugene Ui-Chong Lim

| Oct 21, 2021 6:56 pm

Anecdotally, I found that Tofacitinib 10mg bd worked fast and very well in some patients (targeting IL15 and other cytokines), while others do better with TNFi

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