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. 2020 Aug 17;43(9):1–9. doi: 10.1007/s40618-020-01229-1

Table 1.

Definitions of polyglandular autoimmune syndrome type II and type III across the studies included

Article Polyglandular autoimmune syndrome type II Polyglandular autoimmune syndrome type III
Abrar-Ahmad [29] AD and AITD and/or T1DM and other autoimmunities

The absence of adrenal insufficiency

III/a-AITD and T1DM and sarcoidosis or coeliac disease

III/b-AITD and pernicious anemia

III/c-AITD and vitiligo or alopecia

Ben-Skowronek et al. [27] AD and AITD and/or T1DM

AITD plus other endocrinopathy except for AD

III/A-AITD and T1DM

III/B-AITD and pernicious anemia

III/c-AITD and vitiligo and/or alopecia and/or other organ-specific autoimmune diseases

Betterle et al. [31] AD and AITD and/or T1DM AITD and other autoimmune diseases excluding AD and/or hypoparathyroidism
Cruz et al. [20] AD and AITD and/or T1DM PAS type III differs from type II by the absence of AD
Dittmar and Kahaly [21] AD and AITD and/or T1DM Apart from the absence of AD, there is no clinical difference between PAS type II and type III
Horie et al. [23] a AITD and T1DM
Papadopoulos and Hallengren [25] AD and AITD and/or T1DM without hypoparathyroidism or chronic mucocutaneous candidiasis a
Papadopoulos et al. [26] AD and AITD and/or T1DM AITD and T1DM and/or pernicious anemia and/or vitiligo/alopecia
Piatkowska and Szalecki [22] a AITD and at least one of autoimmune disease: T1DM, pernicious anemia, vitiligo, alopecia areata, coeliac disease, hypogonadism, myasthenia gravis and others excluding AD
Sastre-Garriga [28] AD and AITD and/or T1DM for primary criteria, secondary criteria can be gonadal failure, vitiligo, alopecia and pernicious anemia a
Storz et al. [35] AD and AITD and/or T1DM AD excluded
Choudhuri et al. [19], Handa and Dogra [34], Karagüzel et al. [32], Karavanaki et al. [33], Kondonouri et al. [26], Renzullo et al. [3], Teufel et al. [30] a a

aNo definition given in the study, we characterized the subgroups, as follows: polyglandular autoimmune syndrome type II: Addison’s disease and autoimmune thyroid disease and/or type I diabetes mellitus; polyglandular autoimmune syndrome type III: autoimmune thyroid disorders and other autoimmune diseases except Addison’s disease

AD Addison’s disease, AITD autoimmune thyroid disorders, PAS polyglandular autoimmune syndrome, T1DM type 1 diabetes mellitus