Autoimmune polyendocrine syndrome type 3
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| Autoimmune polyendocrine syndrome type 3 | |
|---|---|
| Other names | Autoimmune polyendocrinopathy type 3, Autoimmune polyglandular syndrome type 3, APS type 3, APS3, PAS3.[1] |
| Specialty | Endocrinology, Rheumatology |
| Symptoms | Any symptoms associated with its constituent diseases |
| Complications | Autoimmune thyroiditis (Always), Celiac Disease, Type I Diabetes, Autoimmune hypophysitis, Systemic Lupus Erythematosus, Sjögren's Syndrome, Vitiligo (May or May not be Present) |
| Usual onset | Any Age |
| Duration | Lifelong |
| Types | APS 3A: Autoimmune thyroiditis with Immune Mediated diabetes mellitus;
APS 3B: Autoimmune thyroiditis with Pernicious anemia; APS 3C: Autoimmune thyroiditis with Vitiligo and/or Alopecia and/or Another Organ Specific Disease |
| Causes | Combination of Genetic and Environmental Factors |
| Risk factors | Family History of APS III or other Autoimmune Diseases |
| Diagnostic method | Serum antibody assays; Autoimmune Thyroiditis Required for Diagnosis |
| Differential diagnosis | Autoimmune Polyendocrine Syndrome Type II, APECED, IPEX |
| Prevention | N/A |
| Treatment | Depends on Constituent Diseases |
| Prognosis | Normal Lifespan |
| Frequency | ~ 2-3% of population |
Autoimmune polyendocrine syndrome, type 3 is a condition characterized by the coexistence of autoimmune thyroiditis and at least one other autoimmune disease (excluding Addison's Disease).[2] Based on other organ-specific autoimmune involvement, there are multiple subtypes that are classified: type 3a shows thyroid autoimmune disease in conjunction with type 1 diabetes, type 3b shows thyroid autoimmune disease in conjunction with pernicious anemia (PA), and type 3c shows thyroid autoimmune disease in conjunction with alopecia, vitiligo, or other organ-specific autoimmune disease.[3]
The hallmark of autoimmune polyglandular syndromes (APS) is the existence of autoimmune reactions directed against multiple endocrine and non-endocrine organs. There have been described as four primary types:[4]
- APS-1 [Autoimmune-Polyendocrine-Candidiasis-Ectodermal-Dystrophy Syndrome (APECED)]: Chronic hypoparathyroidism, autoimmune Addison's disease, and chronic candidiasis (two conditions must exist for the term to be used).[4]
- APS-2 (Schmidt's disease): Autoimmune Addison's disease (constantly present), in conjunction with either type 1 diabetes mellitus (DM) or autoimmune thyroid disease.[4]
- APS-3: Type 1 diabetes, atrophic gastritis, pernicious anemia, vitiligo, alopecia, and myasthenia gravis, and autoimmune thyroid disease; Addison's disease and/or hypoparathyroidism are not included in this association.[4]
- APS-4: Combinations that weren't in the earlier groups.[4]
Signs and symptoms
Most patients with autoimmune polyendocrine syndrome type 3 have autoimmune thyroid diseases associated with only one other autoimmune disease; these associations are most frequently with either type 1 diabetes (20–30% of cases)[5] or chronic atrophic gastritis (39 percent of cases).[6] Other disorders associated with autoimmune polyendocrine syndrome type 3 are pernicious anemia, vitiligo, alopecia, and myasthenia gravis.[4]
Epidemiology
Autoimmune polyendocrine syndrome, type 3 has a frequency of 1.4 to 2 per 100,000 people, with no discernible ethnic group preference.[7]