In terms of immunodeficiency, up to 30% of DADA2 patients have panhypogammaglobulinemia (IgG, IgM, IgA) [3] and can present with a common variable immunodeficiency-like phenotype, including recurrent upper and lower respiratory tract infections, bronchiectasis and associated gastrointestinal involvement. This evidence concerns the gene CD40LG and immunodeficiency disease.