SEVERE ANEMIA AS AN INITIAL MANIFESTATION OF TYPE III B AUTOIMMUNE POLYGLANDULAR SYNDROME: CASE REPORT

Abstract

Introduction: Autoimmune polyglandular syndromes (APS) are the association of two or more autoimmune endocrine diseases. The development of these conditions depends on both genetic and environmental factors. They are classified as type I, known as juvenile-onset PAS, and adult-onset PAS, which can be subdivided into types II to IV. These conditions are underdiagnosed, with a higher prevalence of adult-onset PAS in females and a peak diagnosis between the 2nd and 5th decades of life. Clinical manifestations depend on the association between the autoimmune endocrinopathies, and treatment consists of correction of endocrine dysfunctions, hormone replacement therapy when indicated, symptomatic control, and management of potential complications. Objective: To report a case of type III polyglandular autoimmune syndrome, with severe acute anemia as the initial manifestation. Report: A 58-year-old, previously healthy female patient presented with asthenia, dizziness, weight loss (approximately 10 kg in 2 years), and chronic pain in the lower limbs. She was admitted for management of severe anemia, with evidence of hemoglobin of 4.6 g/dL. During the investigation, tests showed thyroid alterations compatible with primary hypothyroidism (TSH: 12.5 μIU/mL; free T4: 0.82 ng/dL), in addition to significant hypovitaminosis B12. Following the investigation, atrophic gastritis and significant elevation of anti-TPO (>1300 IU/mL) were identified, allowing for the diagnosis of Polyglandular Syndrome type IIIB, due to autoimmune thyroiditis and pernicious anemia. The patient subsequently continued outpatient follow-up, with a good response to management of hormonal disorders. She also underwent further investigation to determine the possibility of new autoimmune disorders over the years. Conclusion: Autoimmune polyglandular syndrome type III is a condition with low incidence due to underdiagnosis. Early diagnosis and appropriate management are essential due to the impact on morbidity and mortality of the underlying conditions.

DOI:https://doi.org/10.56238/sevened2025.031-023