Keywords
Autoimmunity
Early diagnosis
Autoimmune polyendocrinopathy
Diabetes Mellitus
Death
Antibodies
How to Cite
Abstract
Background: Autoimmune polyglandular syndrome (GPS) is a group of autoimmune disorders with insufficiency of at least two glands and a broad spectrum of clinical manifestations, presenting in childhood or adulthood.
Purpose: To present the case of GPS to understand the basics of this disease, its diagnosis and treatment, as well as its rare fatal outcome.
Case presentation: A 16-year-old patient presented with severe diabetic ketoacidosis and a history of myasthenia gravis diagnosed at one year of age, in addition to autoimmune thyroid disease. During his hospital stay, he had an ominous course, presenting with thrombocytopenia with purpura and renal failure, which were suggestive of autoimmune disease in the absence of bacterial pathogen isolates. After immunological screening, he received pulses of methylprednisolone and immunoglobulin to neutralize circulating antibodies due to his rapid clinical deterioration. After evaluation by pediatric rheumatology with positive ANA, anti-DNA, lupus anticoagulant, and anti-thyroid peroxidase antibodies, in addition to the presence of diabetes mellitus and myasthenia gravis, a diagnosis of autoimmune polyglandular syndrome type 3 was made. Despite multiple therapeutic efforts and extracorporeal membrane oxygenation therapy, the patient developed multiorgan failure, resulting in death.
Discussion and conclusion: GPS can have a variety of clinical manifestations and even trigger severe processes due to the poor auto-suppressive response of T lymphocytes. Early diagnosis is important to allow for a comprehensive approach with timely treatment and the prevention of fatal outcomes.
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