Korean J Ophthalmol.  2013 Oct;27(5):331-340. 10.3341/kjo.2013.27.5.331.

Effect of Age and Early Intervention with a Systemic Steroid, Intravenous Immunoglobulin or Amniotic Membrane Transplantation on the Ocular Outcomes of Patients with Stevens-Johnson Syndrome

• Kim KH
• Park SW
• Kim MK
• Wee WR

• KMID: 1707286
• DOI: http://doi.org/10.3341/kjo.2013.27.5.331

Abstract

PURPOSE
This retrospective observational case series of fifty-one consecutive patients referred to the eye clinic with acute-stage Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) from 1995 to 2011 examines the effect of early treatment with a systemic corticosteroid or intravenous immunoglobulin (IVIG) on the ocular outcomes in patients with SJS or TEN.
METHODS
All patients were classified by age (< or =18 years vs. >18 years) and analyzed by treatment modality and early intervention with systemic corticosteroids (< or =5 days), IVIG (< or =6 days), or amniotic membrane graft transplantation (AMT) (< or =15 days). The main outcomes were best-corrected visual acuity (BCVA) in logarithm of the minimum angle of resolution (logMAR) and ocular involvement scores (OIS, 0-12), which were calculated based on the presence of superficial punctate keratitis, epithelial defect, conjunctivalization, neovascularization, corneal opacity, keratinization, hyperemia, symblepharon, trichiasis, mucocutaneous junction involvement, meibomian gland involvement, and punctal damage.
RESULTS
The mean logMAR and OIS scores at the initial visit were not significantly different in the pediatric group (logMAR = 0.44, OIS = 2.76, n = 17) or the adult group (logMAR = 0.60, OIS = 2.21, n = 34). At the final follow-up, the logMAR and OIS had improved significantly in the adult group (p = 0.0002, p = 0.023, respectively), but not in the pediatric group. Early intervention with IVIG or corticosteroids significantly improved the mean BCVA and OIS in the adult group (p = 0.043 and p = 0.024, respectively for IVIG; p = 0.002 and p = 0.034, respectively for corticosteroid). AMT was found to be associated with a significantly improved BCVA or OIS in the late treatment group or the group with a better initial OIS (p = 0.043 and p = 0.043, respectively for BCVA; p = 0.042 and p = 0.041, respectively for OIS).
CONCLUSIONS
Our findings suggest that patients with SJS or TEN who are aged 18 years or less have poorer ocular outcomes than older patients and that early treatment with steroid or immunoglobulin therapy improves ocular outcomes.

Keyword

Amniotic membrane graft transplantation; Immunoglobulins; Ocular complication; Steroids; Stevens-Johnson syndrome

MeSH Terms

Acute Disease
Adolescent
Age Factors
Amnion/*transplantation
Biopsy
Child
Child, Preschool
Corneal Diseases/etiology/pathology/*therapy
Female
Follow-Up Studies
Glucocorticoids/*administration & dosage
Humans
Immunoglobulins, Intravenous/*administration & dosage
Infant
Male
Retrospective Studies
Stevens-Johnson Syndrome/complications/pathology/*therapy
Time Factors
Treatment Outcome
*Visual Acuity
Glucocorticoids
Immunoglobulins, Intravenous

Figure

• 

  Fig. 1 Changes in visual acuities and ocular involvement score (OIS) with respect to patient age. (A) Mean logarithms of the minimal angle of resolution (logMAR) values in the pediatric group were similar at initial and final visits. In contrast, the mean logMAR of the adult patients improved significantly over the same period. (B) The pediatric group also showed no significant difference in mean OIS between initial and final visits. However, the adult group showed a significant improvement in mean OIS. There was no significant between group differences in each visits. Pediatric group = who are aged 18 years or less; adult group = who are over 18 years old. *Wilcoxon's signed rank test.

• 

  Fig. 2 Changes in visual acuities of the adult group with respect to treatment modality and time of treatment initiation. Early treatment of adult patients with intravenous immunoglobulin (IVIG) or with systemic corticosteroids was found to be associated with a significantly improved logMAR at final visit. The mean logMAR of patients treated with amniotic membrane graft transplantation (AMT) improved significantly by the final visit if patients were treated 15 days after disease onset or if their ocular involvement score was less than 6 at the initial visit. There was no significant between group differences in each visits. Early group = patients with treatment initiation ≤6 days for IVIG, ≤5 days for corticosteroid, or ≤15 days for AMT. Late group = treatment initiation >6 days for IVIG, >5 days for corticosteroid, or >15 days for AMT. Better group = patients with ocular involvement scores (OIS) of less than 6 at initial visit. Worse group = patients with OIS over 6 at initial visit. *Wilcoxon's signed rank test.

• 

  Fig. 3 Changes in ocular involvement score (OIS) of the adult group with respect to treatment modality and time of treatment initiation. Early treatment of adult patients with intravenous immunoglobulin (IVIG) or with systemic corticosteroids was found to be associated with a significantly improved OIS at final visit. The mean OIS of patients who were treated with amniotic membrane graft transplantation (AMT) improved significantly in the late group and worse group. There was no significant between group differences in each visits. Early group = patients with treatment initiation ≤6 days for IVIG, ≤5 days for corticosteroid, or ≤15 days for AMT. Late group = treatment initiation >6 days for IVIG, >5 days for corticosteroid, or >15 days for AMT. Better group = patients with OIS of less than 6 at initial visit. Worse group = patients with OIS over 6 at initial visit. *Wilcoxon's signed rank test.

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