{"title":"晚发型施密特综合征伴严重低钠血症:病例报告。","authors":"Stella Pigni, Giacomo Cristofolini, Simona Jaafar, Giulia Maida, Erika Grossrubatscher, Paolo Dalino, Emanuela Carioni, Gherardo Mazziotti, Andrea Lania, Benedetta Zampetti, Iacopo Chiodini","doi":"10.2174/0118715303323053240611075428","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Schmidt's syndrome (SS) is a subtype of polyglandular autoimmune syndrome type-2 combining autoimmune thyroiditis (AIT) and autoimmune Addison's disease (aAD). It occurs most frequently in young adult females, and aAD is the most common initial manifestation [1]. We present a rare case of SS with late-onset aAD and severe hyponatremia as the first sign.</p><p><strong>Case report: </strong>A 73-year-old woman presented to the emergency department (ED) with a 10-day history of vomiting, diarrhea, and altered mental status. Her past medical history was remarkable for AIT and hypokinetic cardiomyopathy. Moreover, she had recently undergone a 2-week course of corticosteroid therapy for vertiginous symptoms, reporting subjective well-being. In ED, she appeared confused and hypotensive. Blood tests revealed a sodium level of 99 mEq/l with normal potassium. Initial treatment with saline infusions were started, followed by ex juvantibus intravenous hydrocortisone awaiting hormone results, which proved consistent with primary adrenal insufficiency (ACTH 1314 pg/ml, cortisol 4.72 ug/dL). Replacement therapy with both hydrocortisone and fludrocortisone was then implemented, with substantial clinical improvement and normalization of sodium levels. However, the patient later developed right heart failure and hypokalemia, which were likely caused by overreplacement and resolved after adjusting the treatment regimen. The final diagnosis of aAD was confirmed by positive adrenal autoantibodies.</p><p><strong>Conclusions: </strong>aAD should be suspected in each case of severe hyponatremia [2], especially in patients with AIT independent of age. Furthermore, caution is needed in managing high-dose glucocorticoids along with fludrocortisone in elderly patients with cardiac disease to limit the risk of excessive mineralocorticoid activity and heart failure [3].</p>","PeriodicalId":94316,"journal":{"name":"Endocrine, metabolic & immune disorders drug targets","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Late-onset Schmidt's Syndrome Presenting with Severe Hyponatremia: A Case Report.\",\"authors\":\"Stella Pigni, Giacomo Cristofolini, Simona Jaafar, Giulia Maida, Erika Grossrubatscher, Paolo Dalino, Emanuela Carioni, Gherardo Mazziotti, Andrea Lania, Benedetta Zampetti, Iacopo Chiodini\",\"doi\":\"10.2174/0118715303323053240611075428\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Schmidt's syndrome (SS) is a subtype of polyglandular autoimmune syndrome type-2 combining autoimmune thyroiditis (AIT) and autoimmune Addison's disease (aAD). It occurs most frequently in young adult females, and aAD is the most common initial manifestation [1]. We present a rare case of SS with late-onset aAD and severe hyponatremia as the first sign.</p><p><strong>Case report: </strong>A 73-year-old woman presented to the emergency department (ED) with a 10-day history of vomiting, diarrhea, and altered mental status. Her past medical history was remarkable for AIT and hypokinetic cardiomyopathy. Moreover, she had recently undergone a 2-week course of corticosteroid therapy for vertiginous symptoms, reporting subjective well-being. In ED, she appeared confused and hypotensive. Blood tests revealed a sodium level of 99 mEq/l with normal potassium. Initial treatment with saline infusions were started, followed by ex juvantibus intravenous hydrocortisone awaiting hormone results, which proved consistent with primary adrenal insufficiency (ACTH 1314 pg/ml, cortisol 4.72 ug/dL). Replacement therapy with both hydrocortisone and fludrocortisone was then implemented, with substantial clinical improvement and normalization of sodium levels. However, the patient later developed right heart failure and hypokalemia, which were likely caused by overreplacement and resolved after adjusting the treatment regimen. The final diagnosis of aAD was confirmed by positive adrenal autoantibodies.</p><p><strong>Conclusions: </strong>aAD should be suspected in each case of severe hyponatremia [2], especially in patients with AIT independent of age. Furthermore, caution is needed in managing high-dose glucocorticoids along with fludrocortisone in elderly patients with cardiac disease to limit the risk of excessive mineralocorticoid activity and heart failure [3].</p>\",\"PeriodicalId\":94316,\"journal\":{\"name\":\"Endocrine, metabolic & immune disorders drug targets\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-15\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Endocrine, metabolic & immune disorders drug targets\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.2174/0118715303323053240611075428\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Endocrine, metabolic & immune disorders drug targets","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.2174/0118715303323053240611075428","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
背景:施密特综合征(SS)是结合自身免疫性甲状腺炎(AIT)和自身免疫性阿狄森病(aAD)的多腺自身免疫综合征2型的一种亚型。该病多发于青壮年女性,而阿狄森病是最常见的首发症状[1]。我们报告了一例罕见的 SS 病例,该病例以晚发 aAD 和严重低钠血症为首发症状:一名 73 岁的妇女因呕吐、腹泻和精神状态改变 10 天来急诊就诊。她的既往病史有明显的 AIT 和低运动性心肌病。此外,她最近还因眩晕症状接受了为期两周的皮质类固醇治疗,主观感觉良好。在急诊室,她显得神志不清,血压过低。血液检查显示钠含量为 99 mEq/l,钾含量正常。开始使用生理盐水输液进行初步治疗,随后静脉注射氢化可的松,等待激素检测结果,结果证明她患有原发性肾上腺功能不全(促肾上腺皮质激素 1314 pg/ml,皮质醇 4.72 ug/dL)。随后,患者接受了氢化可的松和氟氢可的松的替代治疗,临床症状得到明显改善,血钠水平也趋于正常。然而,患者后来出现了右心衰竭和低钾血症,这很可能是过度补充造成的,在调整治疗方案后症状得到缓解。结论:对于每例严重低钠血症[2],尤其是与年龄无关的AIT患者,都应怀疑AAD。此外,对于患有心脏病的老年患者,在使用大剂量糖皮质激素和氟氢可的松时应谨慎,以限制矿化皮质激素活性过高和心力衰竭的风险[3]。
Late-onset Schmidt's Syndrome Presenting with Severe Hyponatremia: A Case Report.
Background: Schmidt's syndrome (SS) is a subtype of polyglandular autoimmune syndrome type-2 combining autoimmune thyroiditis (AIT) and autoimmune Addison's disease (aAD). It occurs most frequently in young adult females, and aAD is the most common initial manifestation [1]. We present a rare case of SS with late-onset aAD and severe hyponatremia as the first sign.
Case report: A 73-year-old woman presented to the emergency department (ED) with a 10-day history of vomiting, diarrhea, and altered mental status. Her past medical history was remarkable for AIT and hypokinetic cardiomyopathy. Moreover, she had recently undergone a 2-week course of corticosteroid therapy for vertiginous symptoms, reporting subjective well-being. In ED, she appeared confused and hypotensive. Blood tests revealed a sodium level of 99 mEq/l with normal potassium. Initial treatment with saline infusions were started, followed by ex juvantibus intravenous hydrocortisone awaiting hormone results, which proved consistent with primary adrenal insufficiency (ACTH 1314 pg/ml, cortisol 4.72 ug/dL). Replacement therapy with both hydrocortisone and fludrocortisone was then implemented, with substantial clinical improvement and normalization of sodium levels. However, the patient later developed right heart failure and hypokalemia, which were likely caused by overreplacement and resolved after adjusting the treatment regimen. The final diagnosis of aAD was confirmed by positive adrenal autoantibodies.
Conclusions: aAD should be suspected in each case of severe hyponatremia [2], especially in patients with AIT independent of age. Furthermore, caution is needed in managing high-dose glucocorticoids along with fludrocortisone in elderly patients with cardiac disease to limit the risk of excessive mineralocorticoid activity and heart failure [3].