Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline
Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline
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DOI：
10.1210/jc.2015-1710
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发表时间：
2016-02-01
影响因子：
5.8
通讯作者：
Torpy, David J.
中科院分区：
文献类型：
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作者：
Bornstein, Stefan R.;Allolio, Bruno;Torpy, David J.
目的：本临床实践指南涉及原发性肾上腺皮质功能不全的诊断和治疗。
参与者：工作组包括一名由内分泌学会临床指南小组委员会选定的主席、另外八名对该疾病有经验的临床医生、一名方法学家和一名医学撰稿人。共同赞助的协会（欧洲内分泌学会和美国临床化学协会）有参与成员。工作组在本次审查中未接受任何企业资金或报酬。
证据：本基于证据的指南是使用推荐分级、评估、制定和评价（GRADE）系统制定的，以确定推荐强度和证据质量。
共识过程：用于制定推荐的证据来自两项委托进行的系统综述以及工作组确定的其他已发表的系统综述和研究。该指南依次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，工作组都根据书面意见进行修改。
结论：我们建议对所有有提示性临床症状或体征的患者进行原发性肾上腺皮质功能不全的排除性诊断试验。特别是，我们建议在急性病患者以及有诱发因素的患者中采用较低的诊断（和治疗）阈值。对于有不明原因的持续性恶心、疲劳和低血压的孕妇也建议如此。我们推荐短效促肾上腺皮质激素试验（250μg）作为确立诊断的“金标准”诊断工具。如果一开始无法进行短效促肾上腺皮质激素试验，我们建议采用包括测量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。对潜在病因的诊断应包括对21 - 羟化酶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）以及氢化可的松（15 - 25mg/d）或醋酸可的松替代治疗（20 - 35mg/d），分2 - 3次每日剂量给药。对于儿童，推荐氢化可的松（约8mg/m²/d）。应教育患者有关应激剂量的知识，并为其配备类固醇卡片和用于胃肠外紧急给药的糖皮质激素制剂。随访应旨在监测糖皮质激素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。
Objective: This clinical practice guideline addresses the diagnosis and treatment of primary adrenal insufficiency.Participants: 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.Evidence: 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.Consensus Process: 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.Conclusions: 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 mu g) 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 (similar to 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.