Review of Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

MedicalResearch.com Interview with:
Prof. Dr. Maja Mockenhaupt

Dept. of Dermatology
Medical Center – University of Freiburg
Deutschland / Germany

MedicalResearch.com: What is the background for this study?

Response: Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are severe cutaneous adverse reactions that are associated with high morbidity and mortality. Primarily due to their rareness, therapeutic effects are often studied in observational settings. An evidence-based standardized treatment protocol for SJS/TEN is still missing.

MedicalResearch.com: What are the main findings?

Response: The comprehensive overview on proposed therapies for SJS/TEN revealed a series of different treatment options that is of immediate help for any treating physician. Besides supportive case, applied systemic immunomodulating therapies include glucocorticosteroids, intravenous immunoglobulins, cyclosporine, plasmapheresis, thalidomide, cyclophosphamide, hemoperfusion, tumor necrosis factor- alpha inhibitors and granulocyte colony-stimulating factor.

According to the results combining published data, glucocorticosteroids and cyclosporine are the most promising immunomodulating therapies for SJS/TEN to reduce mortality. Nevertheless, further research is needed for final conclusions and consistent guidelines in the treatment of SJS/TEN-patients.

MedicalResearch.com: What should readers take away from your report?

Response: Readers should get an overview on proposed therapies for SJS/TEN and on their therapeutic effects on mortality in SJS/TEN-patients.

1. Overview on proposed therapies:
Applied therapies are supportive care only or systemic immunomodulating therapies including glucocorticosteroids, intravenous immunoglobulins, cyclosporine, plasmapheresis, thalidomide, cyclophosphamide, hemoperfusion, tumor necrosis factor- alpha inhibitors and granulocyte colony-stimulating factor.

2. Therapeutical effects:
Glucocorticosteroids and cyclosporine showed both significant survival benefits for SJS/TEN-patients (odds ratios<1). No beneficial effects were observed for other immunomodulating treatments including intravenous immunoglobulins. Thus, glucocorticosteroids and cyclosporine are the most promising immunomodulating therapies for SJS/TEN. Further evaluation in prospective studies is warranted.

MedicalResearch.com: What recommendations do you have for future research as a result of this study?

Response: Multinational efforts to facilitate prospective interventional studies of high-quality are needed. Double-blind randomized therapeutic trials are very difficult to perform in rare diseases that may occur absolutely unexpectedly like SJS/TEN, and well-controlled treatment studies using the same inclusion criteria may be more feasible and should be encouraged. Especially studies with Cyclosporine and Glucocorticosteroids should be performed to confirm the positive results of our systematic review.

MedicalResearch.com: Is there anything else you would like to add?

Response: Immunomodulating treatments may help to stop disease progression, when applied early in the course of the reaction. Cyclosporine seems to be the most promising candidate for that, since studies included in our analysis demonstrated that progression of skin detachment could be stopped, whereas an unspecific overall benefit could be shown for glucocorticosteroids. Nevertheless, supportive care by a multidisciplinary team is crucial and can never be replaced by immunomodulatig therapy alone.

MedicalResearch.com: Thank you for your contribution to the MedicalResearch.com community.

Citation:
Zimmermann S, Sekula P, Venhoff M, Motschall E, Knaus J, Schumacher M, Mockenhaupt M. Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis A Systematic Review and Meta-analysis. JAMA Dermatol. Published online March 22, 2017. doi:10.1001/jamadermatol.2016.5668

Note: Content is Not intended as medical advice. Please consult your health care provider regarding your specific medical condition and questions.

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