Type 1 diabetes and polyglandular autoimmune syndrome: A review

Table 5 Polyglandular autoimmune syndromes[13,68,78,161-166]

	PAS Type I	PAS Type II-IV	IPEX
Onset	Childhood	Adulthood	Infancy
Incidence	< 1:100000/yr	1–2:100000/yr	Extremely rare
Male/Female ratio	3:04	1:03	Male >> Female
Genetics	Monogenetic (AIRE)	Polygenetic	X-linked (FOXP3)
Autoantibodies	Anti-interferon-α/ω antibodies 100%, additional Abs	Organ-specific Abs depending on the autoimmune components	ANA (42%) SSA (25%) TG Abs (25%)
Prevalence of T1D	2%-33%	40%-60%	80%
Additional autoimmune endocrine components	Hypoparathyroidism (80%-85%) Addison’s disease (60%-70%) Hypogonadism (12%) Autoimmune thyroid disease (10%)	Autoimmune thyroid disease (70%-75%) Addison’s disease (40%-50%) Hypoparathyroidism (0%-5%) Hypogonadism (0%-3%) Hypopituitarism (0%-2%)	Autoimmune thyroid disease (25%)
Concomitant non-endocrine diseases	Mucocutaneous candidiasis (70%-80%); autoimmune hepatitis; autoimmune gastritis; alopecia areata; vitiligo; keratoconjunctivitis	Autoimmune gastritis; pernicious anemia; neurodermitis; alopecia areata; myasthenia gravis; systemic lupus erythematosus; rheumatoid arthritis; autoimmune hepatitis	Malabsorption; autoimmune skin diseases; multiple sclerosis

Abs: Antibodies; AIRE: Autoimmune regulatory gene; ANA: Anti-nuclear antibodies; FOXP3: Forkhead box protein P3; IPEX: Immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome; PAS: Polyglandular autoimmune syndrome; TG: Transglutaminase; T1D: Type 1 diabetes.