Autoimmune Polyglandular Syndrome Type 1

One of the rarest autoimmune diseases is the autoimmune polyglandular syndrome type 1 which is caused by defects in the AIRE gene leading to affection of several endocrine glands. Two out of three criteria are needed for diagnosis including hypoparathyroidism, adrenal insufficiency along with other non-endocrinal symptoms such as mucocutaneous candidiasis. Management of the condition is multidisciplinary with regular long-life follow-ups. Generally, there is a paucity of illustrative articles in literature about the pathogenesis and the main signs and symptoms of autoimmune polyglandular syndrome type 1 in a simplified manner which lead to the need for such articles. This article attempts to fill that void as an illustrative overview for the purpose of education and awareness about this condition because the early the treating physician can diagnose and accordingly treat the patient, the better the prognosis of the disease becomes with less morbidity and mortality rates.