Atypical Presentation of Autoimmune Polyglandular Syndrome Type II: The Tip of the Iceberg at the Interface between Type 2 and Type 3 Multiple Autoimmune Syndrome

Background: Autoimmune polyglandular syndrome type 2 is a rare condition characterized by at least two induced autoimmune glandular disorders that always includes Addison's disease, as well as type 1 diabetes mellitus and/or autoimmune thyroid diseases. Clinical Case: A 55-year-old patient with a personal medical history of gout and gastroduodenal ulcer, was admitted to the Internal Medicine department of the University Hospital Center Point G in Bamako for polyarthralgia and long-term fever, in whom the initial diagnosis of seronegative rheumatoid arthritis had been made in the face of an ultrasound scan of the metacarpophalangeal and interphalangeal joints showing signs of synovitis without effusion associated with the ACR/EULAR 2010 criteria; associated with an arthritic disease. During her evolution, she presented with adrenal insufficiency syndrome, after emergency management with hydrocortisone hemisuccinate 100 mg IV, the immunological assessment showed positivity for anti-21-alpha hydroxylase autoantibodies and anti-TSH receptor autoantibodies; leading us to a final diagnosis of autoimmune polyglandular syndrome type II made up of Addison's disease - rheumatoid arthritis and thyroid autoimmunity; in a context of very probable multiple autoimmune syndrome. His evolution was favorable after adequate management with methotrexate associated with folic acid and hydrocortisone; and therapeutic education. Conclusion: Autoimmune polyglandular syndrome should be considered in all patients with systemic and glandular autoimmune disease, particularly adrenal and thyroid; this in a context of polyautoimmunity.

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Publication Details

Journal
SAS Journal of Medicine
Published
2025-03-15
DOI
https://doi.org/10.36347/sasjm.2025.v11i03.012
Primary Topic
Adrenal Hormones and Disorders
Type
article
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article

Atypical Presentation of Autoimmune Polyglandular Syndrome Type II: The Tip of the Iceberg at the Interface between Type 2 and Type 3 Multiple Autoimmune Syndrome

Sidi Touré, A. Koné, Mamadou Cissoko, Adama Sinayoko et al.
SAS Journal of Medicine
Adrenal Hormones and Disorders
article

Atypical Presentation of Autoimmune Polyglandular Syndrome Type II: The Tip of the Iceberg at the Interface between Type 2 and Type 3 Multiple Autoimmune Syndrome

Sidi Touré, A. Koné, Mamadou Cissoko, Adama Sinayoko, Kaly Keïta, Stéphane Loïque Djeugoue, Diallo Seydou, Ibrahima Amadou Dembélé, György Balla, Pamanta Sory Ibrahim, Tientcheu Toko Dorette
article en

Abstract

Background: Autoimmune polyglandular syndrome type 2 is a rare condition characterized by at least two induced autoimmune glandular disorders that always includes Addison's disease, as well as type 1 diabetes mellitus and/or autoimmune thyroid diseases. Clinical Case: A 55-year-old patient with a personal medical history of gout and gastroduodenal ulcer, was admitted to the Internal Medicine department of the University Hospital Center Point G in Bamako for polyarthralgia and long-term fever, in whom the initial diagnosis of seronegative rheumatoid arthritis had been made in the face of an ultrasound scan of the metacarpophalangeal and interphalangeal joints showing signs of synovitis without effusion associated with the ACR/EULAR 2010 criteria; associated with an arthritic disease. During her evolution, she presented with adrenal insufficiency syndrome, after emergency management with hydrocortisone hemisuccinate 100 mg IV, the immunological assessment showed positivity for anti-21-alpha hydroxylase autoantibodies and anti-TSH receptor autoantibodies; leading us to a final diagnosis of autoimmune polyglandular syndrome type II made up of Addison's disease - rheumatoid arthritis and thyroid autoimmunity; in a context of very probable multiple autoimmune syndrome. His evolution was favorable after adequate management with methotrexate associated with folic acid and hydrocortisone; and therapeutic education. Conclusion: Autoimmune polyglandular syndrome should be considered in all patients with systemic and glandular autoimmune disease, particularly adrenal and thyroid; this in a context of polyautoimmunity.

SAS Journal of MedicineVol. 11(03)
Openalex Percentile: Top 93%
Adrenal Hormones and Disorders
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