DISEASE SCANNER

Global Incurable Diseases Tracker

Back to Globe
Autoimmune Disease

Autoimmune Polyglandular Syndrome

HIGH SEVERITY

A group of disorders characterized by autoimmune destruction of multiple endocrine glands. Type 1 (AIRE gene): mucocutaneous candidiasis, hypoparathyroidism, adrenal insufficiency. Type 2: adrenal insufficiency, autoimmune thyroid disease, type 1 diabetes.

Global Affected

50.0K

Countries

22

Symptoms

Mucocutaneous candidiasis (APS-1)
Hypocalcemia
Adrenal insufficiency
Hypothyroidism
Type 1 diabetes
Alopecia
Vitiligo
Pernicious anemia

Treatment Options

Corticosteroids
Immunosuppressants
Biologic agents
DMARDs
Plasmapheresis
IVIG
Physical therapy
Symptom management

Risk Factors

1Female gender
2Family history
3Genetic predisposition
4Environmental triggers
5Infections
6Hormonal factors
7Smoking

Diagnostic Methods

  • 1Autoantibody testing
  • 2Inflammatory markers
  • 3Imaging
  • 4Biopsy
  • 5Clinical criteria
  • 6Exclusion of other causes

Prognosis

Variable; many autoimmune diseases are chronic with relapsing-remitting courses. Early treatment and modern biologics have significantly improved outcomes.

Prevention

  • No specific prevention
  • Smoking cessation
  • Avoidance of triggers
  • Early diagnosis and treatment
  • Regular monitoring

Research Status

Hormone replacement for each deficient gland. Antifungals for candidiasis. Regular monitoring for new autoimmune components. Genetic counseling. Lifelong management.

Affected Countries

United StatesUnited KingdomCanadaAustraliaGermanyFranceItalySpainJapanBrazilMexicoNigeriaSouth AfricaIndiaChinaFinlandSwedenNorwayEELVLTRussia

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK519046
  • https://www.ncbi.nlm.nih.gov/books
  • https://www.mayoclinic.org/diseases-conditions
  • https://rarediseases.org/rare-diseases

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.