Abstract：
Isolated adrenocorticotropic hormone deficiency (IAD) is considered to be a rare disease. Due to the nonspecific clinical presentation, precise data on the prevalence and incidence are lacking. In this systematic review, we aimed to analyse the clinical characteristics, association with autoimmune diseases, and management of acquired idiopathic IAD cases. A structured search was conducted after developing a search strategy combining terms for acquired (idiopathic) IAD. Articles describing an adult case with a diagnosis of ACTH deficiency using dynamic testing, no deficiency of other pituitary axes, and MRI of the brain/pituitary protocolled as normal, were included. Exclusion criteria were cases describing congenital IAD, cases with another aetiology for IAD, and articles where full text was not available. In total 42 articles were included, consisting of 85 cases of acquired idiopathic IAD. Distribution by sex was approximately equal (F:M; 47:38). Lethargy was the most common presenting symptom (38%), followed by weight loss (25%), anorexia (22%), and myalgia/arthralgia (12%). Eight cases (9.5%) presented with an Addison crisis. 31% of cases had an autoimmune disease at diagnosis of which Hashimoto hypothyroidism was the most frequent. Data about follow-up was scarce; dynamic testing was repeated in 4 cases of which 2 showed recovery of the adrenal axis. We report the largest case series of acquired idiopathic IAD to date. Our systematic review highlights the lack of a clear definition and diagnostic work-up. Based on the findings in this review a proposition is made for a flowchart to diagnose acquired idiopathic IAD.
摘要：
孤立性促肾上腺皮质激素缺乏（IAD）被认为是一种罕见的疾病。由于非特异性临床表现，缺乏关于患病率和发病率的精确数据.在这次系统审查中，我们的目的是分析临床特征，与自身免疫性疾病相关，和获得性特发性IAD病例的处理。在开发结合获得性（特发性）IAD术语的搜索策略后，进行了结构化搜索。文章描述了使用动态测试诊断ACTH缺乏症的成人病例，没有其他垂体轴的缺乏，脑/垂体的MRI正常，包括在内。排除标准是描述先天性IAD的病例，具有其他病因的IAD的病例，以及没有全文的文章。共包括42篇文章，85例获得性特发性IAD。按性别划分的分布大致相等(F:M;47:38)。嗜睡是最常见的症状（38％），其次是体重减轻（25%），厌食症（22%），和肌痛/关节痛（12％）。8例（9.5％）出现了艾迪生危机。31％的病例在诊断时患有自身免疫性疾病，其中桥本甲状腺功能减退症最常见。有关随访的数据很少；在4例中重复了动态测试，其中2例显示肾上腺轴恢复。我们报告了迄今为止最大的获得性特发性IAD病例系列。我们的系统审查强调缺乏明确的定义和诊断工作。根据这篇综述的发现，提出了诊断获得性特发性IAD的流程图。
