Which human leukocyte antigens are associated with increased risk for Stevens-Johnson syndrome (SJS)?

Updated: Jan 17, 2019
  • Author: C Stephen Foster, MD, FACS, FACR, FAAO, FARVO; Chief Editor: Andrew A Dahl, MD, FACS  more...
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There is strong evidence for a genetic predisposition to severe cutaneous adverse drug reactions such as Stevens-Johnson syndrome. Carriage of the following human leukocyte antigens has been associated with increased risk:

  • HLA-B*1502

  • HLA-B*5801

  • HLA-B*44

  • HLA-A29

  • HLA-B12

  • HLA-DR7

  • HLA-A2

  • HLA-B*5801

  • HLA-A*0206

  • HLA-DQB1*0601

Certain of these HLA alleles are associated with an increased probability of developing Stevens-Johnson syndrome upon exposure to specific drugs. The US Food and Drug Administration (FDA) and Health Canada advise screening for HLA-B*1502 in patients of southeastern Asian ethnicity before starting treatment with carbamazepine. (The risk is much lower in other ethnic populations, making screening impractical in them). HLA-B*5801 confers a risk of allopurinol-related reactions. [25] Pretreatment screening is not readily available. [26]

Whites with HLA-B*44 appear to be more susceptible to develop Stevens-Johnson syndrome. HLA-A29, HLA-B12, and HLA-DR7 are frequently associated with sulfonamide-induced Stevens-Johnson syndrome, while HLA-A2 and HLA-B12 are often encountered in Stevens-Johnson syndrome induced by nonsteroidal anti-inflammatory drugs (NSAIDs).

HLA-A*0206 and HLA-DQB1*0601 allele have been shown to be was strongly associated with Stevens-Johnson syndrome with ocular disease. [27, 28]

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