Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency.


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Kıykım A., Özen A. O. , Özen A. O. , Özen A. O. , Özen A. O. , Çekiç Ş., ...More

The journal of allergy and clinical immunology. In practice, vol.7, no.8, 2019 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 7 Issue: 8
  • Publication Date: 2019
  • Doi Number: 10.1016/j.jaip.2019.06.011
  • Journal Name: The journal of allergy and clinical immunology. In practice
  • Journal Indexes: Science Citation Index Expanded, Scopus
  • Keywords: LPS-responsive beige-like anchor, Immune dysregulation, Abatacept, T follicular helper cells, Autoimmunity, IMMUNE DYSREGULATION, CTLA-4 CHECKPOINT, MUTATIONS, POLYENDOCRINOPATHY, ENTEROPATHY, DISEASE

Abstract

BACKGROUND: LPS-responsive beige-like anchor (LRBA) deficiency presents with susceptibility to infections, autoimmunity, and lymphoproliferation. The long-term efficacy of cytotoxic T-lymphocyte-associated antigen 4-immunoglobulin (abatacept) as targeted therapy for its immune dysregulatory features remains to be established.