Endocrine Abstracts (2015) 38 P430 | DOI: 10.1530/endoabs.38.P430

Autoimmune polyendocrine syndrome type 4

Shang Shaho


Barts Health, London, UK.


Autoimmune Polyendocrine Syndromes (APS) are heterogenous organ-specific autoimmune disorders affecting multiple endocrine glands, although non-endocrine organs can also be affected. We describe a 58 year old gentleman with a background of type 1 diabetes mellitus (T1DM), Addison’s disease and primary autoimmune hypothyroidism, who presented with a two day history of vomiting. He had no associated diarrhoea, abdominal pain, or fever and there was no history of recent travel, contact with unwell persons or consumption of unusual foods. He subsequently developed a sore throat and hoarse voice and was investigated with a gastroscopy, which revealed extensive candida infection of the vocal cords and oesophagus. APS are categorized into four types, based on the combination of endocrine glands that are affected. APS type 1, characterised by hypoparathyroidism, mucocutaneous candidiasis and Addison’s disease, is frequently seen in childhood. For a more common APS type 2 to be diagnosed, Addison’s disease together with autoimmune thyroiditis and/or together with diabetes mellitus type 1 must be present. The third type of autoimmune polyendocrine syndrome APS type 3 involves the same disorder of endocrine glands as type 2 but usually without any defect of adrenal cortex. If the autoimmune polyendocrinopathies do not fulfil the criteria of APS 1 to 3, the disease may be categorized as autoimmune polyendocrine syndrome type 4. Although our patient had features most consistent with autoimmune polyendocrine syndrome type 2, he presented with mucocutanous candidiasis as well. This combination leads us to a diagnosis of APS type 4. This case highlights the importance of being vigilant about new components of a syndrome developing at different times though a patient’s life.

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