Unlike ALPS, organ-specific autoimmunity was commonly observed in ALPS-like patients, such as autoimmune thyroiditis in PIK3R1 or STAT1 GOF patients (39, 40), autoimmune polyarthritis in STK4 patients (22) autoimmune enteropathy in XIAP, STAT3 GOF, CTLA4, LRBA and IL2RB patients (16, 41–46) or autoimmune hepatitis in STAT1 GOF or LRBA patients (40, 47) (Table 1). Here, PIK3R1 is linked to autoimmune enteropathy.