Shock Syndromes II
ii.
CyclosporineβInformation from individual case series suggests benefit at a dose of 3 mg/kg/d.
There are no formal recommendations for routine use.
iii.
CyclophosphamideβEarly case reports suggested benefit, but cyclophosphamide is not
recommended.
iv.
Colony-stimulating factorβMay be used in conjunction with cyclosporine in patients with
neutropenia and TEN
IVIG
| (a) | IVIG for SJS and TEN is controversial. |
|---|---|
| (b) | In vitro data support that immunoglobulin G antibodies against Fas-FasL proteins may |
decrease keratinocyte apoptosis.
| (c) | Many retrospective single-group and cohort studies suggest benefit (usual dosage, |
|---|
1 g/kg/d for 3 days) over SCORTEN-estimated mortality rates and similar control groups,
respectively.
| (d) | Given the rare incidence and logistical difficulty of designing a multicenter prospective |
|---|
study, available prospective studies have been small and single center. Results from these
studies, however, have shown no benefit to trends of worse outcomes.
| (e) | A 2015 study examining the impact of early systemic immunomodulatory treatment |
|---|
identified prognostic factors for chronic ocular complications in patients with SJS and
TEN (Ophthalmology. 2015;122(2):254-264). Conducted as a retrospective, comparative,
multicenter study across 3 university hospitals in Korea, it included 43 patients diagnosed
with SJS or TEN. The treatments were categorized into 5 groups: systemic steroids, IVIG,
combined steroids and IVIG, systemic pulse steroids, and supportive care only. The study
measured outcomes according to BCVA and chronic ocular surface complications score
(COCS) at the final follow-up. Despite the varied treatments, there were no significant
differences in final BCVA and COCS between the groups after adjusting statistically.
High COCS was notably associated with female sex and the use of amniotic membrane
transplantation during the acute stage. In addition, severe acute ocular and systemic
involvement scores were linked to worse COCS, which correlated strongly with final BCVA.
The study concluded that systemic immunomodulatory treatments did not significantly
affect the final visual outcomes or COCS in patients, indicating that factors such as sex
and the severity of initial symptoms may be more critical in predicting long-term ocular
complications in SJS and TEN.
| (f) | A 2005 study focused on the efficacy of high-dose IVIG in reducing acute ocular |
|---|
complications in patients with TEN (Eye (Lond). 2005;19(8):846-853). This retrospective,
historically controlled study compared 10 consecutive patients treated with high-dose
IVIG at Tan Tock Seng Hospital, Singapore, from July 2001 to June 2002, against 18
historical cohort patients diagnosed with TEN between July 1995 and June 2001 who did
not receive IVIG. The treated group received 2 g/kg of IVIG over 2 days. The analysis
showed that 90% of the patients treated with IVIG experienced ocular involvement, with
varying severity: mild complications like lid edema or mild conjunctival injection in 25%,
moderate complications such as pseudomembranes in 50%, and severe complications
including nonhealing epithelial defects with visual loss and symblepharon in 25%. In
contrast, 55.6% of the historical cohort experienced ocular issues, predominantly mild to
moderate, with no severe cases reported. The study concluded that IVIG treatment did not
significantly reduce the severity of visually significant ocular complications in patients
with TEN, suggesting a need for larger studies to confirm these findings.
| (g) | A systematic review and meta-analysis of use in patients with TEN found no benefit over |
|---|
standard of care.