Yanes et al. presents evidence-based guidelines from the Australasian Society of Clinical Immunology and Allergy to support clinicians in selecting patients with suspected inborn errors of immunity for genomic testing. The work provides practical recommendations to improve clinical decision-making, delivery of diagnostic genomic testing, and patient outcomes.

A report in this issue of JHI deepens our understanding of human disease associated with loss of CTLA-4.

The hyper IgE syndrome (formerly also called Job syndrome or Buckley syndrome) is a rare and syndromic primary immunodeficiency. It is an autosomal-dominant disorder caused by heterozygous dominant-negative mutations in the transcription factor STAT3. Here, we propose clinical management guidelines for this multisystem condition based on the experience and management of these patients in the last 25+ years.