Bornstein Stefan R, Allolio Bruno, Arlt Wiebke, Barthel Andreas, Don-Wauchope Andrew, Hammer Gary D, Husebye Eystein S, Merke Deborah P, Murad M Hassan, Stratakis Constantine A, Torpy David J
Medizinische Klinik und Poliklinik III (S.R.B., A.B.), Universitätsklinikum Dresden, 01307 Dresden, Germany; Department of Endocrinology and Diabetes (S.R.B.), King's College London, London WC2R 2LS, United Kingdom; Department of Internal Medicine I (B.A.), Endocrine and Diabetes Unit, University Hospital Würzburg, 97080 Würzburg, Germany; Comprehensive Heart Failure Center (B.A.), University of Würzburg, 97080 Würzburg, Germany; Centre for Endocrinology, Diabetes, and Metabolism (W.A.), University of Birmingham, Birmingham B15 2TT, United Kingdom; Endokrinologikum Ruhr (A.B.), 44866 Bochum, Germany; Department of Pathology and Molecular Medicine (A.D.-W.), McMaster University, Hamilton, ON L8S 4L8, Canada; Hamilton Regional Laboratory Medicine Program (A.D.-W.), Hamilton, ON L8N 4A6, Canada; Department of Internal Medicine (G.D.H.), Division of Metabolism, Endocrinology, and Diabetes, and Cancer Center, University of Michigan, Ann Arbor, Michigan 48109; Department of Clinical Science, University of Bergen, and Department of Medicine, Haukeland University Hospital (E.S.H.), 5021 Bergen, Norway; National Institutes of Health Clinical Center (D.P.M.), Bethesda, Maryland 20814; Mayo Clinic, Division of Preventive Medicine (M.H.M.), Rochester, Minnesota 55905; Eunice Kennedy Shriver National Institute of Child Health and Human Development (C.A.S.), National Institutes of Health, Bethesda, Maryland 20892; and Endocrine and Metabolic Unit (D.J.T.), Royal Adelaide Hospital, University of Adelaide, Adelaide SA 5000, Australia.
J Clin Endocrinol Metab. 2016 Feb;101(2):364-89. doi: 10.1210/jc.2015-1710. Epub 2016 Jan 13.
This clinical practice guideline addresses the diagnosis and treatment of primary adrenal insufficiency.
The Task Force included a chair, selected by The Clinical Guidelines Subcommittee of the Endocrine Society, eight additional clinicians experienced with the disease, a methodologist, and a medical writer. The co-sponsoring associations (European Society of Endocrinology and the American Association for Clinical Chemistry) had participating members. The Task Force received no corporate funding or remuneration in connection with this review.
This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to determine the strength of recommendations and the quality of evidence.
The evidence used to formulate recommendations was derived from two commissioned systematic reviews as well as other published systematic reviews and studies identified by the Task Force. The guideline was reviewed and approved sequentially by the Endocrine Society's Clinical Guidelines Subcommittee and Clinical Affairs Core Committee, members responding to a web posting, and the Endocrine Society Council. At each stage, the Task Force incorporated changes in response to written comments.
We recommend diagnostic tests for the exclusion of primary adrenal insufficiency in all patients with indicative clinical symptoms or signs. In particular, we suggest a low diagnostic (and therapeutic) threshold in acutely ill patients, as well as in patients with predisposing factors. This is also recommended for pregnant women with unexplained persistent nausea, fatigue, and hypotension. We recommend a short corticotropin test (250 μg) as the "gold standard" diagnostic tool to establish the diagnosis. If a short corticotropin test is not possible in the first instance, we recommend an initial screening procedure comprising the measurement of morning plasma ACTH and cortisol levels. Diagnosis of the underlying cause should include a validated assay of autoantibodies against 21-hydroxylase. In autoantibody-negative individuals, other causes should be sought. We recommend once-daily fludrocortisone (median, 0.1 mg) and hydrocortisone (15-25 mg/d) or cortisone acetate replacement (20-35 mg/d) applied in two to three daily doses in adults. In children, hydrocortisone (∼8 mg/m(2)/d) is recommended. Patients should be educated about stress dosing and equipped with a steroid card and glucocorticoid preparation for parenteral emergency administration. Follow-up should aim at monitoring appropriate dosing of corticosteroids and associated autoimmune diseases, particularly autoimmune thyroid disease.
本临床实践指南阐述了原发性肾上腺皮质功能减退症的诊断与治疗。
特别工作组包括一名由内分泌学会临床指南小组委员会选出的主席、另外八名患有该疾病的临床医生、一名方法学家以及一名医学撰写人。共同赞助协会（欧洲内分泌学会和美国临床化学协会）有参与成员。特别工作组在此次审查中未接受任何企业资金或报酬。
本循证指南采用推荐分级、评估、制定与评价（GRADE）系统制定，以确定推荐强度和证据质量。
用于制定推荐的证据来自两项委托进行的系统评价以及特别工作组确定的其他已发表的系统评价和研究。该指南先后由内分泌学会临床指南小组委员会和临床事务核心委员会、对网络发布内容做出回应的成员以及内分泌学会理事会进行审查和批准。在每个阶段，特别工作组都会根据书面意见进行修改。
我们建议对所有有指示性临床症状或体征的患者进行诊断性检查，以排除原发性肾上腺皮质功能减退症。特别是，我们建议对急性病患者以及有易感因素的患者采用较低的诊断（和治疗）阈值。对于原因不明的持续性恶心、疲劳和低血压的孕妇也建议如此。我们建议采用短程促肾上腺皮质激素试验（250μg）作为确诊的“金标准”诊断工具。如果最初无法进行短程促肾上腺皮质激素试验，我们建议首先进行包括测定早晨血浆促肾上腺皮质激素和皮质醇水平的初步筛查程序。潜在病因的诊断应包括针对21-羟化酶的自身抗体的有效检测。在自身抗体阴性的个体中，应寻找其他病因。我们建议成人每日一次服用氟氢可的松（中位数为(0.1mg)）和氢化可的松（(15 - 25mg/d)）或醋酸可的松替代物（(20 - 35mg/d)），分两到三次服用。对于儿童，建议服用氢化可的松（约(8mg/m²/d)）。应告知患者应激剂量的相关知识，并为其配备类固醇卡和用于肠胃外紧急给药的糖皮质激素制剂。随访应旨在监测皮质类固醇的适当剂量以及相关的自身免疫性疾病，特别是自身免疫性甲状腺疾病。
