Autoimmune polyglandular syndrome (APS) type 1 is a rare autoimmune disorder inherited in an autosomal recessive pattern due to loss of function of the AIRE gene and defective removal of self-reactive T-lymphocytes during the process of thymic T cell maturation. Its manifestation starts early in life with the cardinal clinical disorders being one of muco-cutaneous candidiasis, Addison's disease, and hypoparathyroidism. Recognizing the syndromic nature of one autoimmune disease will facilitate an active search for other conditions which would allow early detection, management, follow-up, and most importantly patient education and counselling to avoid potential complications. We present the case of a young immigrant with multiple endocrinopathies and mucocutaneous candidiasis who presented with features of adrenal insufficiency. Our aim was to briefly review APS type 1 as a disease entity and to highlight the importance of patient education in its management.
J Community Hosp Intern Med Perspect
Nwosu, I., Oladiran, O., Ogbonna-Nwosu, C., & Anyata, A. (2019). Autoimmune polyglandular syndrome type 1: a case report and brief review.. J Community Hosp Intern Med Perspect, 9 (3), 252-254. https://doi.org/10.1080/20009666.2019.1616523