II型自身免疫多内分泌综合征:肾上腺危象后

Nuno Zarcos Palma, M. Cruz, L. Santos, Margarida Cruz, Filipe Cunha, Vítor Fagundes, L. Pires, M. Mesquita
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摘要

自身免疫性多内分泌综合征(APS)是一种罕见的自身免疫性内分泌疾病,其特征是两种或多种器官特异性疾病的相关性。II型自身免疫性多内分泌综合征(APS II)包括Addison病与甲状腺自身免疫性疾病和/或1型糖尿病的相关性。尽管典型的表现是症状性低血压,但它可以表现为肾上腺危象——一种危及生命的情况。我们报告了一例41岁的女性,患有长期乏力、皮肤色素沉着和静脉输液难治的症状性低血压。APS II被诊断为Addisonian危象,在氢化可的松发作后得到缓解。
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Autoimmune polyendocrine syndrome type II: After adrenal crisis
Autoimmune Polyendocrine Syndromes (APS) are rare autoimmune endocrinopathies, characterized by the association of two or more organ-specific disorders. Type II Autoimmune Polyendocrine Syndromes (APS II) comprises the association of Addison’s disease with thyroid autoimmune disease and/or type 1 diabetes mellitus. Although the classic presentation is symptomatic hypotension, it can manifest as an adrenal crisis - a life-threatening condition. We report a case of a 41-year-old woman with prolonged asthenia, cutaneous hyperpigmentation and symptomatic hypotension refractory to intravenous fluids. APS II was diagnosed with a presentation of an Addisonian crisis, resolved after the onset of hydrocortisone.
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