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CVID patients can also manifest rheumatologic diseases (eg, arthritis, Sjogren's disease, systemic lupus erythematosus, vasculitis, Behçet's syndrome) and gastrointestinal autoimmune disorders (eg, ulcerative colitis, autoimmune atrophic gastritis, celiac disease). This latter may pose a particular diagnostic challenge, as celiac-specific autoantibodies can be absent in CVID patients. Understanding the molecular basis and the clinical impact of autoimmunity in CVID patients might help manage CVID, thus reducing its diagnostic delay and p