Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency

KIYKIM, AYÇA; Ogulur, Ismail; Dursun, Esra; Charbonnier, Louis; Nain, Ercan; ÇEKİÇ, ŞÜKRÜ; ÖZCAN, DİLEK; KARACA, NESLİHAN; Cogurlu, Mujde; Bilir, Ozlem; Cansever, Murat; Kapakli, Hasan; Baser, Dilek; Kasap, Nurhan; Kutlug, Seyhan; ALTINTAŞ, DERYA; Al-Shaibi, Ahmad; Agrebi, Nourhen; Kara, Manolya; Guven, Ayla; SOMER, Ayper; Aydogmus, Cigdem; AKTAY AYAZ, Nuray; Metin, Ayse; AYDOĞAN, METİN; UNCUOĞLU, AYŞEN; Patiroglu, Turkan; Yıldıran, Alişan; GÜNER, ŞÜKRÜ; KELEŞ, SEVGİ; REİSLİ, İSMAİL; AKSU, GÜZİDE; KÜTÜKÇÜLER, NECİL; KILIÇ GÜLTEKİN, SARA; Yilmaz, Mustafa; AYDINER, ELİF; Lo, Bernice; ÖZEN, AHMET; Chatila, Talal; BARIŞ, SAFA

doi:10.1016/j.jaip.2019.06.011

Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency

KIYKIM A., Ogulur I., Dursun E., Charbonnier L. M., Nain E., ÇEKİÇ Ş., ...More

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

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
Ondokuz Mayıs 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.