We recently described two siblings with autosomal recessive (AR) complete programmed death 1 (PD-1) deficiency, both of whom had type 1 diabetes (T1D) and thyroiditis in childhood and died from autoimmune pneumonitis at the ages of 3 and 11 years (Ogishi et al., 2021). The gene discussed is PDCD1; the disease is hyperinsulinemic hypoglycemia, familial, 4.