Upala Sikarin, Yong Wai Chung, Sanguankeo Anawin
Department of Internal Medicine, Bassett Medical Center, Columbia University College of Physicians and Surgeons, New York, USA; Department of Preventive and Social Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Department of Internal Medicine, Bassett Medical Center, Columbia University College of Physicians and Surgeons, New York, USA.
N Am J Med Sci. 2016 May;8(5):226-8. doi: 10.4103/1947-2714.183014.
Autoimmune polyglandular syndrome is a rare condition that causes a variety of clinical symptoms due to autoimmune processes involving multiple endocrine organs. Its vague presentation can cause missed or delayed treatment for adrenal insufficiency, resulting in a life-threatening adrenal crisis.
A 21-yr-old man presented with lethargy, hypotension, hyponatremia, hypoglycemia, and an elevated thyroid-stimulating hormone level. He was binge drinking the day before presentation. No significant response to initial treatment with levothyroxine and dextrose occurred. Diagnostic workup later revealed primary adrenal insufficiency. All initial symptoms completely resolved following treatment with hydrocortisone, fludrocortisone, and levothyroxine.
Autoimmune polyglandular syndrome causes dysfunction of multiple endocrine organs such as the thyroid gland, adrenal gland, and pancreas. Initial diagnosis of APS is crucial and difficult because of its vague, acute presentation, which often involves hypothyroidism and adrenal insufficiency. Delayed treatment of adrenal insufficiency can result in a life-threatening adrenal crisis. A diagnostic workup for adrenal insufficiency should be performed in patients who do not respond to hypothyroidism treatment.
自身免疫性多腺体综合征是一种罕见疾病,由于多个内分泌器官发生自身免疫过程,导致出现多种临床症状。其表现不典型可导致肾上腺功能不全的诊断延误或漏诊,进而引发危及生命的肾上腺危象。
一名21岁男性出现嗜睡、低血压、低钠血症、低血糖以及促甲状腺激素水平升高。他在就诊前一天大量饮酒。初始使用左甲状腺素和葡萄糖治疗后无明显反应。后续诊断检查发现原发性肾上腺功能不全。使用氢化可的松、氟氢可的松和左甲状腺素治疗后,所有初始症状完全缓解。
自身免疫性多腺体综合征会导致甲状腺、肾上腺和胰腺等多个内分泌器官功能障碍。由于自身免疫性多腺体综合征的症状模糊且呈急性发作,常涉及甲状腺功能减退和肾上腺功能不全,因此其初始诊断至关重要且困难。肾上腺功能不全的治疗延迟可导致危及生命的肾上腺危象。对于甲状腺功能减退治疗无反应的患者,应进行肾上腺功能不全的诊断检查。