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Polyglandular Autoimmune Syndrome (Type 1, 2, 3)

Syndromes characterized by autoimmune destruction of more than one endocrine gland.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Internal Medicine department. Book Appointment →

What is Polyglandular Autoimmune Syndrome (Type 1, 2, 3)?

Polyglandular autoimmune syndromes (PAS) are syndromes in which autoimmune destruction in more than one endocrine gland coexists. Type 1 (APECED syndrome) is due to AIRE gene mutation and presents in childhood with the triad of mucocutaneous candidiasis, hypoparathyroidism, and adrenal insufficiency.

Type 2 (Schmidt syndrome) is the most common form and consists of a combination of Addison's disease + autoimmune thyroid disease (Hashimoto or Graves) ± type 1 diabetes. It is associated with HLA-DR3 and HLA-DR4.

Type 3 defines the coexistence of autoimmune thyroid disease with other autoimmune diseases (type 1 DM, pernicious anemia, vitiligo, celiac) without adrenal involvement.

Symptoms

Findings of involvement of more than one endocrine gland (in different combinations)
Chronic fatigue and weakness
Hyperpigmentation (in adrenal insufficiency)
Tetany and paresthesias (in hypoparathyroidism)
Polyuria-polydipsia (if type 1 DM develops)
Mucocutaneous candidiasis (in type 1 PAS)

Risk Factors

Family history (clustering of autoimmune disease)
Diagnosis of one autoimmune endocrine disease (others should be screened)
HLA-DR3 and HLA-DR4 haplotype
AIRE gene mutation (for type 1)
Female sex (type 2 and 3 — female predominance)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • If new organ involvement findings develop in a known autoimmune endocrinopathy
  • Unexplained hypotension + hypoglycemia (suspected adrenal crisis) → EMERGENCY
  • If there is family history of more than one autoimmune endocrine disease
  • If chronic candidiasis + hypocalcemia is detected in childhood

Treatment Methods

01
Specific hormone replacement for each affected endocrine gland
02
Addison: Hydrocortisone + fludrocortisone
03
Hypothyroidism: Levothyroxine
04
Type 1 DM: Insulin therapy
05
Hypoparathyroidism: Calcium + active vitamin D (calcitriol)
06
Regular screening (autoantibody screening for affected glands + glands at risk)

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.