Autoimmune Polyendocrinopathies (Schmidt Syndrome)

Autoimmune diseases affecting multiple endocrine organs. Type I is characterized by childhood onset and chronic mucocutaneous candidiasis (CANDIDIASIS, CHRONIC MUCOCUTANEOUS), while type II exhibits any combination of adrenal insufficiency (ADDISON'S DISEASE), lymphocytic thyroiditis (THYROIDITIS, AUTOIMMUNE;), HYPOPARATHYROIDISM; and gonadal failure. In both types organ-specific ANTIBODIES against a variety of ENDOCRINE GLANDS have been detected. The type II syndrome differs from type I in that it is associated with HLA-A1 and B8 haplotypes, onset is usually in adulthood, and candidiasis is not present.
Also Known As:
Schmidt Syndrome; Schmidt's Syndrome; Autoimmune Polyendocrinopathy; Polyendocrinopathies, Autoimmune; Autoimmune Polyglandular Syndrome Type I; Autoimmune Polyglandular Syndrome Type II; Autoimmune Polyglandular Syndrome, Type 1; Polyglandular Autoimmune Syndrome, Type 1; Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy; Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophies; Polyendocrinopathy, Autoimmune; Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophies, Autoimmune; Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophy, Autoimmune; Schmidts Syndrome; Syndrome, Schmidt's; Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophy; Autoimmune Syndrome Type I, Polyglandular; Autoimmune Syndrome Type II, Polyglandular; Polyglandular Type I Autoimmune Syndrome; Polyglandular Type II Autoimmune Syndrome
Networked: 141 relevant articles (4 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Hypoparathyroidism
2. Hypothyroidism
3. Thyroiditis
4. Osteoporosis
5. Addison Disease (Addison's Disease)


1. Peterson, Pärt: 3 articles (12/2014 - 01/2002)
2. Kämpe, Olle: 3 articles (01/2012 - 06/2002)
3. Perheentupa, J: 3 articles (12/2009 - 12/2000)
4. Perheentupa, Jaakko: 3 articles (03/2009 - 01/2002)
5. Anderson, Mark S: 3 articles (05/2008 - 11/2006)
6. Strassburg, C P: 3 articles (07/2005 - 05/2000)
7. Manns, M P: 3 articles (07/2005 - 05/2000)
8. Peterson, P: 3 articles (07/2000 - 06/2000)
9. Lehman, Heather K: 2 articles (09/2015 - 04/2008)
10. Kisand, Kai: 2 articles (12/2014 - 12/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autoimmune Polyendocrinopathies:
1. AntibodiesIBA
2. Thyroxine (Levothyroxine)FDA LinkGeneric
3. Parathyroid Hormone (Parathormone)IBA
4. Ketoconazole (Nizoral)FDA LinkGeneric
5. Hydrocortisone (Cortisol)FDA LinkGeneric
6. HormonesIBA
7. Thyrotropin (Thyroid-Stimulating Hormone)FDA Link
8. CortisoneIBA
9. type 1 Autoimmune polyendocrinopathy syndromeIBA
10. AutoantibodiesIBA
01/01/2002 - "Autoimmune polyglandular syndrome type 1 (APS-1) is a disease associated with defects of the autoimmune regulator gene and is characterized by autoimmune lesions of several tissues, predominantly endocrine glands, with multiple autoantibodies. "
07/01/1996 - "There have been only four previous reports of Schmidt's syndrome with sarcoidosis, and a possible fifth with negative autoantibodies. "
05/11/1991 - "Among the patients with autoimmune polyendocrinopathy, the prevalence of systemic hypertension was higher in those with cardiac autoantibodies than in those without (5/28 [18%] vs 2/80 [3%]; p = 0.01); the same was true for a family history of hypertension (11 [42%] vs 5 [7%]; p = 0.0001). "
08/01/1986 - "A stimulation test with 15 mU bTSH/kg was performed in three patients with Schmidt's syndrome (coexisting Addison's disease and manifest primary hypothyroidism), 15 patients with either subclinical hypothyroidism or increased titres of thyroid autoantibodies, 10 patients without thyroid involvement, and 10 normal controls. "
06/01/2013 - "Gain-of-function mutations, on the other hand, result in the hypocalcemic disorders of autosomal dominant hypocalcemia and Bartter syndrome type V. Moreover, autoantibodies directed against the extracellular domain of the CaSR have been found to be associated with FHH in some patients, and also in some patients with hypoparathyroidism that may be part of autoimmune polyglandular syndrome type 1. Studies of disease-causing CASR mutations have provided insights into structure-function relationships and highlighted intra-molecular domains that are critical for ligand binding, intracellular signaling, and receptor trafficking. "

Therapies and Procedures

1. Transplants (Transplant)
2. Liver Transplantation
3. Plasmapheresis
4. Laminectomy
5. Subcutaneous Injections