ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2019) 63 P383 | DOI: 10.1530/endoabs.63.P383

Clinical presentation of patients with Autoimmune Polygranular Syndrome III (APS III)

Ageliki Sapera1, Georgios Papadakis2, Dimitra Tampouratzi1, Styliani Kalaitzidou1, Chrysi Karavasili1, Michail Kotis1, Ageliki Aravantinou1, Zoi Roumpidaki1, Anna Dracopoulou1, Victoria Kaltzidou1, Irini Veniou1 & Athanasia Tertipi1


1Metaxa Anticancer Hospital, Piraeus Athens, Greece; 2STEPS Stoffwechselzentrum, Biel/Bienne, Switzerland.


Background: The autoimmune polyglandular syndromes are clusters of endocrine abnormalities that occur in discreet patterns in subjects with immune dysregulation and that permit treatment and anticipation of associated systemic or other hormonal deficiencies. Three major entities are recognized, APS I, APS II and APS III. They are considered rare syndromes, but they are possibly not always thoroughly investigated. APS III, in contrast to APS I and II, does not involve the adrenal cortex. In APS III, autoimmune thyreoiditis occurs with another organ-specific autoimmune disease.

Patients and methods: We present 12 cases of patients who present with autoimmune thyreoiditis and APS III.

1. Woman 76 years old:

Atrophic gastritis and gastric NET G1

Rheumatoid arthritis, at 45 years old

Vitiligo at 40 years old

Positive islet cell and anti-GAD autoantibodies

2. Woman 61 years old:

Alopecia areata at 58 years old

Premature ovarian failure (last menstruation at 38 years)

3. Woman 61 years old:

Alopecia areata at 5 years old

Vitiligo at 36 years old

Atrophic gastritis

4. Woman 55 years old:

Alopecia areata at 4 years old

LADA, positive islet cell and anti-GAD antibodies at 54 years old

5. Woman 72 years old:

Autoimmune gastritis at 63 years old.

6. Woman 46 years old:

Graves’ disease at 43 years old

LADA, positive islet cell and anti-GAD antibodies at 45 years old

7. Woman 60 years old:

Sjögren syndrome at 50 years old

Systemic lupus erythematosus at 50 years old

Atrophic gastritis and hyperplasia of gastric neuroendocrine cells at 48 years old

8. Woman 69 years old:

Coeliac disease at 65 years old

9. Woman 74 years old:

Vitiligo at 30 years old

10. Woman 60 years old:

Autoimmune hepatitis at 59 years old

11. Male 66 years old:

Chronic atrophic gastritis with positive antibodies to parietal cells and intrinsic factor at 60 years old

12. Woman 35 years old:

Premature ovarian failure at 20 years old.

Conclusion: Endocrinologists often treat patients with autoimmune thyroid disease. It is important, when indicated, to investigate for other autoimmune diseases in order to detect an APS III, which may cause serious illnesses, such as diabetic ketoacidosis or neuroendocrine tumors of the stomach due to atrophic gastritis.