Long-term follow-up of autoimmune polyendocrine syndrome type 1 in Norway
Authors' conclusion
APS-1 should be considered in patients diagnosed with PAI before age 20, and AIRE sequencing is recommended for diagnostic confirmation. The presence of IFN-ω autoantibodies, a proinflammatory cytokine profile, and increased soluble IFN receptor levels further support the role of dysregulated interferon responses in APS-1 pathogenesis.
Study context
- Population
- All known Norwegian patients with APS-1, Norwegian Registry of Organ-specific Autoimmune Diseases
- Participants
- 71 patients
- Exposure
- Classical vs nonclassical APS-1
- Classicaln = 49
- Nonclassicaln = 22
- Outcome
- Most frequent clinical manifestations
Methodological detailspopulation-based, long-term follow-up (1996-2025)
- Population-based
- Long-term follow-up (1996-2025)
Main finding
As reported in the abstract
- Classical APS-1: chronic mucocutaneous candidiasis, enamel hypoplasia and primary adrenal insufficiency (PAI) most frequent
- Nonclassical APS-1: vitiligo, hypothyroidism and PAI most common
Observational data: associations, not effects.
Further findings
Cytokine profile
- Broad proinflammatory cytokine signature in classical APS-1, with increased soluble interferon (IFN)-α/β receptor levels
Original publication
Long-term follow-up of autoimmune polyendocrine syndrome type 1 in Norway. J Clin Endocrinol Metab. Published 12 February 2026. doi:10.
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