Chronic adrenal insufficiency in patients with APS 1 also manifests mainly in childhood or adolescence (up to 15 years). Hypocortisolism affects 22-93% of patients. As with isolated adrenal insufficiency, the disease can be latent or subclinical for a long time, manifesting itself unexpectedly in stressful situations.
The first clinical manifestation of APS 1 is chronic candidiasis, which usually develops in childhood (up to 5 years in most cases) and affects the skin, tongue, nails, and mucous membranes.
Autoimmune polyendocrine syndrome type 2 (APS 2) occurs predominantly sporadically, but familial forms inherited in an autosomal dominant manner with incomplete penetrance are often observed. The presence of a specific gene that determines the development of acyclovir has not yet been proven. As with many autoimmune diseases, the risk of developing APS 2 is associated with certain alleles of the major histocompatibility complex.
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