JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE, vol.7, pp.2790-2815, 2019 (SCI-Expanded)
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.