Abstract
Stevens Johnsons syndrome (SJS) is a mucocutaneous disorder caused by an autoimmune response most commonly to medications. Unless it is properly managed in the acute setting, this entity can affect the ocular surface causing chronic cicatrizing conjunctivitis with limbal stem cell deficiency and lid anomalies which ultimately result in corneal opacities that may limit patients' visual acuity. When this stage is reached, some patients might need to undergo some form of corneal and/or limbal stem cell transplantation that exposes an already sensitized immune system to a new alloantigen. While the innate immunity plays a role in corneal graft survival, adaptive immune responses play a major part in corneal graft rejection and failure, namely through CD4+ T cell lymphocytes. Hence, the management of the immune response to surgical transplant procedures in SJS patients, involves a dual approach that modulates the inflammatory response to a new alloantigen in the context of an autoimmune sensitized patient. This review will explore and discuss current perspectives and future directions in the field of ocular immunology on how to manage SJS immune responses to ocular surgical procedures, reviewing systemic and local immunosuppressive therapies and protocols to adequately manage this debilitating condition.
Highlights
Stevens Johnsons syndrome (SJS) is a vesiculobullous disorder that affects the mucocutaneous tissues, which are generally triggered by an autoimmune response to different medications, commonly cold medications, anticonvulsants, and sulfa drugs [1]
After 43.4 months of mean follow-up, almost 80% of patients required a subsequent keratoplasty to improve visual acuity [21]. These findings suggest that even though SJS is a condition with poor ocular prognosis, combined corneal and limbal transplant require an aggressive and diligent immunosuppression with a group of drugs that includes a T cell inhibitor, and that Tacrolimus is favored over Cyclosporine as a T cell inhibitor
It commonly affects the ocular surface to the extent that it may need to undergo corneal surface reconstruction, with possible corneal or limbal stem cell transplantation to improve visual acuity
Summary
Stevens Johnsons syndrome (SJS) is a vesiculobullous disorder that affects the mucocutaneous tissues, which are generally triggered by an autoimmune response to different medications, commonly cold medications, anticonvulsants, and sulfa drugs [1]. After 43.4 months of mean follow-up, almost 80% of patients required a subsequent keratoplasty to improve visual acuity [21] These findings suggest that even though SJS is a condition with poor ocular prognosis, combined corneal and limbal transplant require an aggressive and diligent immunosuppression with a group of drugs that includes a T cell inhibitor, and that Tacrolimus is favored over Cyclosporine as a T cell inhibitor. Our group recently proposed a combinational strategy of BETi combined with T-reg expansion therapy and in murine models of allogeneic hematopoietic stem cell transplant, did not interfere with one another but together suppressed GVHD [43] In the future, this combinatorial platform could be considered for application to SJS patients to downregulate allo and auto immune responses following transplant (Figure 2)
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