Autoimmune Polyglandular Syndrome Type 1

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