Clinical and Immunological Characteristics of Autoimmune Addison Disease: A Nationwide Swedish Multicenter Study

Frida Dalin, Gabriel Nordling Eriksson, P. Dahlqvist, Å. Hallgren, J. Wahlberg, O. Ekwall, S. Söderberg, J. Rönnelid, P. Olcén, O. Winqvist, S. Catrina, B. Kriström, Maria Laudius, M. Isaksson, Maria Halldin Stenlid, J. Gustafsson, G. Gebre-Medhin, S. Björnsdóttir, A. Janson, A. Åkerman, J. Åman, K. Duchén, Ragnhildur Bergthorsdottir, G. Johannsson, E. Lindskog, M. Landin-Olsson, M. Elfving, E. Waldenström, A. Hulting, O. Kämpe, S. Bensing
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引用次数: 72

Abstract

Context: Studies of the clinical and immunological features of autoimmune Addison disease (AAD) are needed to understand the disease burden and increased mortality. Objective: To provide upgraded data on autoimmune comorbidities, replacement therapy, autoantibody profiles, and cardiovascular risk factors. Design, Setting, and Participants: A cross-sectional, population-based study that included 660 AAD patients from the Swedish Addison Registry (2008–2014). When analyzing the cardiovascular risk factors, 3594 individuals from the population-based survey in Northern Sweden, MONICA (monitoring of trends and determinants of cardiovascular disease), served as controls. Main Outcome Measures: The endpoints were the prevalence of autoimmune comorbidities and cardiovascular risk factors. Autoantibodies against 13 autoantigens were determined. Results: The proportion of 21-hydroxylase autoantibody-positive patients was 83%, and 62% of patients had ≥1 associated autoimmune diseases, more frequently coexisting in females (P < 0.0001). AAD patients had a lower body mass index (P < 0.0001) and prevalence of hypertension (P = 0.027) compared with controls. Conventional hydrocortisone tablets were used by 89% of the patients, with a mean dose of 28.1 ± 8.5 mg/d. The mean hydrocortisone equivalent dose normalized to the body surface was 14.8 ± 4.4 mg/m2/d. A greater hydrocortisone equivalent dose was associated with a greater incidence of hypertension (P = 0.046). Conclusions: Careful monitoring of AAD patients is warranted to detect associated autoimmune diseases. Contemporary Swedish AAD patients did not have an increased prevalence of overweight, hypertension, type 2 diabetes mellitus, or hyperlipidemia. However, high glucocorticoid replacement doses could be a risk factor for hypertension.
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自身免疫性阿狄森病的临床和免疫学特征:一项瑞典全国多中心研究
背景:需要对自身免疫性阿狄森病(AAD)的临床和免疫学特征进行研究,以了解疾病负担和死亡率增加。目的:提供有关自身免疫合并症、替代疗法、自身抗体谱和心血管危险因素的最新数据。设计、环境和参与者:一项横断面、基于人群的研究,包括来自瑞典Addison Registry(2008-2014)的660例AAD患者。在分析心血管危险因素时,来自瑞典北部MONICA(监测心血管疾病的趋势和决定因素)人群调查的3594人作为对照。主要结局指标:终点是自身免疫合并症和心血管危险因素的患病率。检测13种自身抗原的自身抗体。结果:21-羟化酶自身抗体阳性的患者比例为83%,62%的患者存在≥1种相关自身免疫性疾病,且多见于女性(P < 0.0001)。与对照组相比,AAD患者的体重指数(P < 0.0001)和高血压患病率(P = 0.027)均较低。89%的患者使用常规氢化可的松片,平均剂量为28.1±8.5 mg/d。体表标准化氢化可的松平均当量剂量为14.8±4.4 mg/m2/d。较高的氢化可的松当量剂量与较高的高血压发病率相关(P = 0.046)。结论:仔细监测AAD患者是必要的,以发现相关的自身免疫性疾病。当代瑞典AAD患者没有增加超重、高血压、2型糖尿病或高脂血症的患病率。然而,高剂量的糖皮质激素替代可能是高血压的危险因素。
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