| Polyglandular Autoimmune syndrome Type 3 (PAS 3) |
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Prepared by: Daniela Cihakova MD, PhD Definition: In PAS-3, autoimmune thyroiditis occurs with another organ-specific autoimmune disease, but not with autoimmune adrenalitis. Other autoimmune diseases can include diabetes mellitus, pernicious anemia, vitiligo, alopecia, myasthenia gravis, and Sjögren’s syndrome. Symptoms: Depends on the organ affected. Diagnosis: Depends on which organ is affected. Treatment involves life-long follow up and screening for another glandular failure in patients already diagnosed with PAS-3. Types: PAS-3 can be further classified into 3 categories: PAS-3A = autoimmune thyroiditis with immune-mediated diabetes mellitus. Patients with autoimmune thyroid disease, IMDM and positive adrenal antibodies should be also considering as PAS-3. The most common PAS-3 combination is autoimmune disease of thyroid gland and pernicious anemia. The second most common combination is IMDM with some form of autoimmune thyroid disease. 20-30 percent of patients with autoimmune thyroid disease have positive parietal cell antibodies, 30 percent of PA patients have thyroid disease. 20 percent of IMDM patients have thyroid disease. Incidence: PAS-3 is most frequent among middle-aged women. Treatment: Depends on which organ is affected. Reatment involves life-long follow up and screening for another glandular failure in patients already diagnosed with PAS-3. Pathogenesis: PAS-3 is a combination of autoimmune diseases as described above. There are other pieces of evidence that PAS-3 is an autoimmune disease: Circumstantial evidence:
Indirect evidence: Mice model for PAS -3 is BioBreeding/Worcester rats (BB/W). In diabetic BBW mice, lymphocytic thyroiditis is also found. |
