Hodé Annelie Kérékou, Dédjan Hubert
Department of Endocrinology, Metabolism and Nutrition, CNHU-HKM Cotonou, Bénin.
Arch Clin Cases. 2021 Oct 27;6(4):109-111. doi: 10.22551/2019.25.0604.10163. eCollection 2019.
Autoimmune polyendocrinopathies are rare diseases characterized by the coexistence of at least two endocrine diseases linked to an autoimmune mechanism, however sometimes are associated with non-endocrine autoimmune diseases. They are divided into two main subgroups: autoimmune polyendocrinopathy type I and polyendocrinopathies type II-IV. We report a case of a 53-year-old female patient followed for 2 years for Hashimoto's thyroiditis. On admission, she was complaining of polyuropolydipsic syndrome, asthenia, weight loss, abdominal pain and vomiting. The clinical examination noted a dehydrated patient in poor general condition, without fever, tachycardic at 104 beats/min, and polypneic at 24 cycles/min. Laboratory tests revealed hyperglycemia (4.7 g/l), glucosuria, acetonuria, anti-GAD>2000 UI/l antibody, normal TSH. The 8-hour cortisol level and anti-21 hydroxylase antibodies level were normal. In this context, the patient was diagnosed with diabetes type 1 associated with Hashimoto's thyroiditis (autoimmune polyendocrinopathy type III). In conclusion, the autoimmune polyendocrinopathy type III is a rare syndrome, predominantly affecting females. In our patient's case, the initial presentation of the disease was dominated by the autoimmune thyroiditis, which is the most frequent endocrine autoimmunity diagnosed in adults with polyglandular autoimmune syndrome. Therefore, the recommended treatment is based on hormonal substitution.
自身免疫性多内分泌腺病是罕见疾病,其特征为至少两种与自身免疫机制相关的内分泌疾病并存,不过有时也与非内分泌自身免疫性疾病相关。它们主要分为两个亚组:自身免疫性多内分泌腺病I型和多内分泌腺病II - IV型。我们报告一例53岁女性患者,因桥本甲状腺炎接受随访2年。入院时,她主诉有多尿多饮综合征、乏力、体重减轻、腹痛和呕吐。临床检查发现患者脱水,一般状况较差,无发热,心率104次/分钟,心动过速,呼吸频率24次/分钟,呼吸急促。实验室检查显示血糖升高(4.7 g/l)、糖尿、酮尿、抗谷氨酸脱羧酶抗体>2000 UI/l,促甲状腺激素正常。8小时皮质醇水平和抗21羟化酶抗体水平正常。在此情况下,该患者被诊断为1型糖尿病合并桥本甲状腺炎(自身免疫性多内分泌腺病III型)。总之,自身免疫性多内分泌腺病III型是一种罕见综合征,主要影响女性。在我们患者的病例中,疾病的初始表现以自身免疫性甲状腺炎为主,这是在患有多腺体自身免疫综合征的成年人中最常诊断出的内分泌自身免疫病。因此,推荐的治疗方法是激素替代治疗。