Tsinopoulou Vasiliki Rengina, Kolanis Savvas, Katsarou Dimitra, Kotanidou Eleni P, Galli-Tsinopoulou Assimina
2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, AHEPA University General Hospital, Thessaloniki, GRC.
Program of Postgraduate Studies Adolescent Medicine and Adolescent Health Care, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, AHEPA University General Hospital, Thessaloniki, GRC.
Cureus. 2025 Jun 18;17(6):e86299. doi: 10.7759/cureus.86299. eCollection 2025 Jun.
Autoimmune polyglandular syndrome type 2 (APS-2) is a rare complex autoimmune endocrinological entity and is reported even more rarely in children and adolescents. It consists of primary adrenal insufficiency (Addison's disease), autoimmune thyroiditis (AIT) and/or type 1 diabetes mellitus (T1DM). Treatment of the AIT component of the disease with levothyroxine as hormone replacement therapy has been reported rarely to cause adrenal crisis. This treatment adverse event is possibly explained due to the increase of basal metabolic rate and subsequently the increased demand and clearance of cortisol after levothyroxine therapy and should always be considered by physicians in order to avoid the development of adrenal crisis. We report the case of a 13-year-old adolescent girl with hypothyroidism due to Hashimoto's thyroiditis (HT) who developed signs of adrenal insufficiency after initial levothyroxine therapy. She was initially admitted with symptoms of hyperthyroidism, including weight loss, anxiety, and oligomenorrhea. A more thorough examination revealed pallor, signs of dehydration, tachycardia, and additionally serum electrolyte imbalance and extremely low cortisol levels in combination with very high adrenocorticotropic hormone (ACTH) levels. The patient received emergency treatment for adrenal insufficiency and recovered well. Due to positive adrenal autoantibodies, APS-2 was confirmed. This case report highlights the necessity of careful adrenal function screening in patients with hypothyroidism and HT before initiating thyroid therapy in order to avoid the rare occurrence of adrenal crisis.
2型自身免疫性多腺体综合征（APS-2）是一种罕见的复杂自身免疫性内分泌疾病，在儿童和青少年中报道更为罕见。它由原发性肾上腺功能不全（艾迪生病）、自身免疫性甲状腺炎（AIT）和/或1型糖尿病（T1DM）组成。据报道，用左甲状腺素作为激素替代疗法治疗该疾病的AIT成分很少会导致肾上腺危象。这种治疗不良事件可能是由于基础代谢率增加，随后左甲状腺素治疗后皮质醇的需求和清除增加所致，医生应始终考虑到这一点，以避免肾上腺危象的发生。我们报告了一例13岁青少年女性因桥本甲状腺炎（HT）导致甲状腺功能减退，在初始左甲状腺素治疗后出现肾上腺功能不全体征的病例。她最初因甲状腺功能亢进症状入院，包括体重减轻、焦虑和月经过少。更全面的检查发现面色苍白、脱水体征、心动过速，此外还有血清电解质失衡以及极低的皮质醇水平，同时促肾上腺皮质激素（ACTH）水平非常高。患者接受了肾上腺功能不全的紧急治疗，恢复良好。由于肾上腺自身抗体呈阳性，确诊为APS-2。本病例报告强调了在对甲状腺功能减退和HT患者开始甲状腺治疗前仔细筛查肾上腺功能的必要性，以避免罕见的肾上腺危象发生。
