Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency

Kiykim, A; Ogulur, I; Dursun, E; Charbonnier, LM; Nain, E; Cekic, S; Dogruel, D; Karaca, NE; Cogurlu, MT; Bilir, OA; Cansever, M; Kapakli, H; Baser, D; Kasap, N; Kutlug, S; Altintas, DU; Al-Shaibi, A; Agrebi, N; Kara, M; Guven, A; Somer, A; Aydogmus, C; Ayaz, NA; Metin, A; Aydogan, METİN; Uncuoglu, AYŞEN; Patiroglu, T; Yildiran, A; Guner, SN; Keles, S; Reisli, I; Aksu, G; Kutukculer, N; Kilic, SS; Yilmaz, M; Karakoc-Aydiner, E; Lo, B; Ozen, A; Chatila, TA; Baris, S

doi:10.1016/j.jaip.2019.06.011

Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency

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Kiykim A., Ogulur I., Dursun E., Charbonnier L., Nain E., Cekic S., ...More

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE, vol.7, no.8, pp.2790-2815, 2019 (SCI-Expanded)

Publication Type: Article / Article
Volume: 7 Issue: 8
Publication Date: 2019
Doi Number: 10.1016/j.jaip.2019.06.011
Journal Name: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Page Numbers: pp.2790-2815
Keywords: LPS-responsive beige-like anchor, Immune dysregulation, Abatacept, T follicular helper cells, Autoimmunity, IMMUNE DYSREGULATION, CTLA-4 CHECKPOINT, MUTATIONS, POLYENDOCRINOPATHY, ENTEROPATHY, DISEASE
Kocaeli University Affiliated: Yes

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.