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SAS Journal of Medicine | Volume-11 | Issue-03
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
Ibrahima Amadou Dembélé, Stéphane Loique Djeugoue, Kaly Keïta, Mamadou Cissoko, Adama Sinayoko, Pamanta Sory Ibrahim, Seydou Diallo, Sidi Touré, Tientcheu Toko Dorette, Amadou Koné, Grace Anicia Balla
Published: March 15, 2025 |
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Pages: 199-205
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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.