[1例罕见的老年自身免疫性多腺综合征2型]。

IF 0.6 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL
Medicina-buenos Aires Pub Date : 2025-01-01
Abrar-Ahmad Zulfiqar
{"title":"[1例罕见的老年自身免疫性多腺综合征2型]。","authors":"Abrar-Ahmad Zulfiqar","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Autoimmune polyglandular syndrome type 2 (APS-2) is a rare and complex clinical entity, whose etiology and evolution are not fully understood. We present the case of a 78-year-old male, followed and treated for Addison's disease for 45 years, in whom macrocytosis with a hemoglobin level of 11.8 g/dL was detected during a routine evaluation. The immunological vitamin B12 deficiency led to the diagnosis of Biermer's disease, while the thyroid, diabetic and liver immunological evaluations were negative. Treatment was started with periodic injections of vitamin B12 and hormone replacement therapy (glucocorticoids and mineralocorticoids) was adjusted, which produced a favorable clinical response with progressive normalization of macrocytosis and hemoglobin levels. Long-term follow-up confirmed clinical stability and effective control of the autoimmune evolution. APS-2 is defined as the coexistence of autoimmune Addison's disease with autoimmune thyroid diseases (such as Graves' disease or Hashimoto's thyroiditis) and/or type 1 diabetes mellitus, in the absence of hypoparathyroidism. Various genetic and environmental factors appear to contribute to its development, although its exact etiology remains unknown. This clinical case supports the recommendation of active screening for autoimmune disorders in patients with Addison's disease.</p>","PeriodicalId":18419,"journal":{"name":"Medicina-buenos Aires","volume":"85 4","pages":"872-875"},"PeriodicalIF":0.6000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[A rare case of autoimmune polyglandular syndrome type 2 in an elderly patient].\",\"authors\":\"Abrar-Ahmad Zulfiqar\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Autoimmune polyglandular syndrome type 2 (APS-2) is a rare and complex clinical entity, whose etiology and evolution are not fully understood. We present the case of a 78-year-old male, followed and treated for Addison's disease for 45 years, in whom macrocytosis with a hemoglobin level of 11.8 g/dL was detected during a routine evaluation. The immunological vitamin B12 deficiency led to the diagnosis of Biermer's disease, while the thyroid, diabetic and liver immunological evaluations were negative. Treatment was started with periodic injections of vitamin B12 and hormone replacement therapy (glucocorticoids and mineralocorticoids) was adjusted, which produced a favorable clinical response with progressive normalization of macrocytosis and hemoglobin levels. Long-term follow-up confirmed clinical stability and effective control of the autoimmune evolution. APS-2 is defined as the coexistence of autoimmune Addison's disease with autoimmune thyroid diseases (such as Graves' disease or Hashimoto's thyroiditis) and/or type 1 diabetes mellitus, in the absence of hypoparathyroidism. Various genetic and environmental factors appear to contribute to its development, although its exact etiology remains unknown. This clinical case supports the recommendation of active screening for autoimmune disorders in patients with Addison's disease.</p>\",\"PeriodicalId\":18419,\"journal\":{\"name\":\"Medicina-buenos Aires\",\"volume\":\"85 4\",\"pages\":\"872-875\"},\"PeriodicalIF\":0.6000,\"publicationDate\":\"2025-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Medicina-buenos Aires\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Medicina-buenos Aires","FirstCategoryId":"3","ListUrlMain":"","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0

摘要

自身免疫性多腺综合征2型(APS-2)是一种罕见而复杂的临床疾病,其病因和演变尚不完全清楚。我们报告一例78岁的男性,因Addison病随访和治疗了45年,在常规评估中检测到血红蛋白水平为11.8 g/dL的巨噬细胞增多症。免疫维生素B12缺乏导致诊断为Biermer病,而甲状腺、糖尿病和肝脏免疫评价均为阴性。治疗开始时,定期注射维生素B12,并调整激素替代疗法(糖皮质激素和矿化皮质激素),这产生了良好的临床反应,巨噬细胞和血红蛋白水平逐渐正常化。长期随访证实临床稳定,自身免疫进化得到有效控制。APS-2被定义为自身免疫性Addison病与自身免疫性甲状腺疾病(如Graves病或桥本甲状腺炎)和/或1型糖尿病共存,没有甲状旁腺功能低下。各种遗传和环境因素似乎有助于其发展,尽管其确切的病因尚不清楚。本临床病例支持对Addison病患者进行自身免疫性疾病主动筛查的建议。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[A rare case of autoimmune polyglandular syndrome type 2 in an elderly patient].

Autoimmune polyglandular syndrome type 2 (APS-2) is a rare and complex clinical entity, whose etiology and evolution are not fully understood. We present the case of a 78-year-old male, followed and treated for Addison's disease for 45 years, in whom macrocytosis with a hemoglobin level of 11.8 g/dL was detected during a routine evaluation. The immunological vitamin B12 deficiency led to the diagnosis of Biermer's disease, while the thyroid, diabetic and liver immunological evaluations were negative. Treatment was started with periodic injections of vitamin B12 and hormone replacement therapy (glucocorticoids and mineralocorticoids) was adjusted, which produced a favorable clinical response with progressive normalization of macrocytosis and hemoglobin levels. Long-term follow-up confirmed clinical stability and effective control of the autoimmune evolution. APS-2 is defined as the coexistence of autoimmune Addison's disease with autoimmune thyroid diseases (such as Graves' disease or Hashimoto's thyroiditis) and/or type 1 diabetes mellitus, in the absence of hypoparathyroidism. Various genetic and environmental factors appear to contribute to its development, although its exact etiology remains unknown. This clinical case supports the recommendation of active screening for autoimmune disorders in patients with Addison's disease.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Medicina-buenos Aires
Medicina-buenos Aires 医学-医学:内科
CiteScore
1.30
自引率
12.50%
发文量
0
审稿时长
6-12 weeks
期刊介绍: Information not localized
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信