Autoimmune polyendocrine syndrome type 2

Autoimmune polyendocrine syndrome type 2
Classification and external resources
Specialty endocrinology
ICD-10 E31.0
ICD-9-CM 258.1
OMIM 269200
DiseasesDB 29690
eMedicine med/1868
MeSH D016884

Autoimmune polyendocrine syndrome type 2, a form of autoimmune polyendocrine syndrome also known as Schmidt's syndrome,[1] or APS-II, is the most common form of the polyglandular failure syndromes.[2] It is heterogeneous and has not been linked to one gene. Rather, patients are at a higher risk when they carry a particular human leukocyte antigen genotype (HLA-DQ2, HLA-DQ8 and HLA-DR4). APS-II affects women to a greater degree than men (75% of cases occur in women).[2]

Features of this syndrome are:


Symptoms of Addison's disease and Hashimoto's thyroiditis include:

Some researchers favour splitting this syndrome into three distinct syndromes (numbering 2, 3[4] and 4), but evidence for these distinct combinations is not convincing.


It is named for Martin Benno Schmidt (1863 1949), a German pathologist.


  1. Heuss D, Engelhardt A, Göbel H, Neundörfer B (June 1995). "Myopathological findings in interstitial myositis in type II polyendocrine autoimmune syndrome (Schmidt's syndrome)". Neurol. Res. 17 (3): 233–7. PMID 7643982.
  2. 1 2 Greenspan, Francis S.; Gardner, David C. (2004). Basic clinical endocrinology. New York: McGraw-Hill. p. 103. ISBN 0-07-140297-7.
  3. Betterle C, Zanchetta R (April 2003). "Update on autoimmune polyendocrine syndromes (APS)". Acta Biomed. 74 (1): 9–33. PMID 12817789.
  4. de Carmo Silva R, Kater CE, Dib SA, et al. (February 2000). "Autoantibodies against recombinant human steroidogenic enzymes 21-hydroxylase, side-chain cleavage and 17alpha-hydroxylase in Addison's disease and autoimmune polyendocrine syndrome type III". Eur. J. Endocrinol. 142 (2): 187–94. doi:10.1530/eje.0.1420187. PMID 10664529. Retrieved 2008-07-25.
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