Autoimmune Polyendocrinopathy (Polyglandular) Syndrome Type II is more common and diverse than APS Type I.


Synonym: Schmidt's Syndrome with Addison's Disease plus hypothyroidism


Onset: variable, evolves with age


First degree family members show multiple endocrine disorders, but the type and severity may differ between individuals.


Endocrine disorders:

(1) Addison's disease

(2) chronic thyroiditis

(3) type 1A diabetes mellitus and/or intermittent hypoglycemia


Some patients present with one endocrine disorder, with other disorders appearing as the patient gets older.


Clinical findings:

(1) hypotension (associated with Addison's disease)

(2) hyperpigmentation and/or vitiligo

(3) intermittent, severe fatigue


HLA types: HLA-DQ2, HLA-DQ8, HLA-DRB1*0404


Autoantibodies may include (and may appear before clinically symptomatic):

(1) 21-hydroxylase autoantibodies

(2) related to autoimmune diabetes

(3) related to autoimmune thyroiditis



(1) autoimmune polyendocrinopathy type I

(2) other polyendocrine disorders


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