Table 2 summarizes the spectrum of autoimmunity described amongst APDS patients reported in the literature. Autoimmune cytopenias and gastrointestinal manifestations are significantly more common than renal or rheumatological diseases or endocrinopathies (4). Variable autoantibody production in APDS patients has also been reported, including ANA, perinuclear ANCA (p-ANCA), PR3-ANCA, and anti-Smith, anti-Ro/SSA, anti-cardiolipin, anti-dsDNA, anti-ribonucleoprotein, and anti-smooth muscle antibodies (16, 23–25, 37, 38, 40, 42). This evidence concerns the gene TRIM21 and activated PI3K-delta syndrome.