From the Guidelines
Treatment for Stevens-Johnson Syndrome (SJS) should prioritize immediate hospitalization, withdrawal of the suspected causative agent, and supportive care, with consideration of systemic corticosteroids, IVIG, or cyclosporine in select cases, as these interventions may improve morbidity, mortality, and quality of life outcomes. The management of SJS requires a multidisciplinary approach, including dermatology, ophthalmology, and critical care specialists. Initial management focuses on supportive care similar to burn treatment, including:
- Fluid replacement with crystalloids like normal saline at 2-3 mL/kg/% body surface area affected
- Wound care with gentle cleansing with sterile saline and application of non-adherent dressings
- Pain management with opioids, such as morphine 0.1 mg/kg IV every 4 hours as needed
- Prevention of complications, including infection and sepsis
Ophthalmologic consultation is essential, as eye involvement can lead to permanent damage; treatment may include:
- Lubricating drops
- Topical antibiotics
- Corticosteroid eye drops, as recommended by the U.K. guidelines for the management of SJS/TEN in adults 2016 1
- Daily ophthalmological review during the acute illness, with a focus on ocular hygiene and prevention of corneal exposure
While the evidence for systemic corticosteroids, IVIG, or cyclosporine is controversial, some cases may benefit from these interventions, particularly when started early in the disease course 1. The use of systemic corticosteroids, such as prednisone 1-2 mg/kg/day for 3-5 days, may be considered in select cases, although the evidence is largely based on retrospective studies and case series. IVIG and cyclosporine may also be considered, although the evidence for their use is limited.
The mortality rate for SJS is 5-10%, with recovery typically taking 2-6 weeks 1. Patients should be educated about avoiding the triggering medication in the future and carrying medical alert identification. Overall, the management of SJS requires a careful balance of supportive care, wound management, and consideration of systemic interventions to improve morbidity, mortality, and quality of life outcomes.
From the Research
Treatment Options for Stevens-Johnson Syndrome
- The treatment of Stevens-Johnson syndrome (SJS) involves the use of various immunomodulatory therapies, including systemic corticosteroids, cyclosporine, intravenous immunoglobulin, cyclophosphamide, plasmapheresis, and tumor necrosis factor-α inhibitors 2.
- Corticosteroids are beneficial in the treatment of SJS and may be lifesaving in some patients, with a recommended dose of prednisone 40 mg daily to methylprednisolone 750 mg daily 3.
- A study found that steroid pulse therapy at disease onset is of great therapeutic importance in preventing ocular complications, with a dose of methylprednisolone 500 or 1000 mg/day for 3 to 4 days 4.
Management Guidelines
- The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL) recommends prompt withdrawal of the culprit drug, meticulous supportive care, and judicious and early initiation of moderate to high doses of oral or parenteral corticosteroids 2.
- A multidisciplinary approach in the management of SJS patients is helpful due to the systemic nature of the disease 2.
- The use of cyclosporine (3-5 mg/kg/day) for 10-14 days may also be used either alone or in combination with corticosteroids 2.
Comparison of Treatment Options
- A systematic review found that etanercept (TNF-alpha inhibitor) may reduce disease-specific mortality compared to corticosteroids, but the certainty of the evidence is low 5.
- The review also found that the certainty of the evidence for disease-specific mortality is very low for the following comparisons: corticosteroids versus no corticosteroids, IVIG versus no IVIG, and cyclosporin versus IVIG 5.
- Topical betamethasone shows great promise for preventing corneal epithelial stem cell loss in the limbal region and cicatricial changes 4.