This section describes PID with autoimmunity including monogenetic diseases of the immune system affecting self-tolerance by thymic impairment (e.g., APECED) and/or other T-lymphocyte function deficiencies (ZAP70), syndromic diseases (JAK1 GOF) or metabolic diseases (Prolidase deficiency) (Table 2). Here, JAK1 is linked to prolidase deficiency.