Soule S
Department of Medicine, University of Cape Town, Cape Town, South Africa.
Clin Endocrinol (Oxf). 1999 Jan;50(1):115-20. doi: 10.1046/j.1365-2265.1999.00625.x.
Addison's disease may present with diverse and non-specific clinical and biochemical features. Contentious issues include the appropriate criteria for the interpretation of the ACTH stimulation test and the necessary extent of investigation to identify a specific aetiology for the hypoadrenalism. The experience of Addison's disease at a South African teaching hospital was reviewed to (1) record the aetiology and spectrum of presentation, (2) examine the performance of the ACTH stimulation test and (3) determine the utility of adrenal CT scan and biopsy.
Retrospective study of patients admitted to a South African teaching hospital from 1980 to 1997 with a diagnosis of acute Addison's disease.
Fifty patients presenting with acute Addison's disease were identified by a search of hospital records. Pretreatment biochemical and haematological parameters were recorded. The cortisol response at 20 and 60 min to an intravenous injection of 250 micrograms synacthen (Cortrosyn) was analysed. In a subgroup of affected subjects, the bone mineral density (BMD) in the lumbar spine and femoral neck was measured during long-term follow-up.
Presenting features included hyperpigmentation (86%), weight loss (67%), abdominal pain (20%) and diarrhoea (16%). Thirty-nine patients (78%) were hyponatraemic, while 26 (53%) were hyperkalaemic. Nine patients (18%) were hypoglycaemic and 21% had hypercalcaemia. The mean basal cortisol was 148 nmol/l (range 10-487) and 16 patients (40%) had a normal basal cortisol. The mean cortisol 20 min after ACTH stimulation was 172 nmol/l (range 19-588). There was no significant increase in serum cortisol following ACTH stimulation (P > 0.05). Adrenal CT scans were performed in only 24 patients (48%) and were normal in 10, while abnormalities were detected in 14 patients (bilateral enlargement in 11, calcification in two and atrophic adrenals in one). Eight patients had a DEXA scan performed during follow-up--four were osteopaenic in the lumbar spine and five at the femoral neck. The probable aetiology of Addison's was idiopathic in 42%, related to active TB in 18%, old TB in 16%, autoimmune in 12% and malignancy with metastases in 6%--single cases were due to sarcoid, iron overload and adrenoleukodystrophy. Adrenal biopsy was performed in two patients and was diagnostic of malignancy in both cases. The mortality within the first month after hospitalization was 12%.
In our experience, Addison's disease is frequently idiopathic, presents with protean manifestations and should be considered in patients with unexplained hyperpigmentation or gastrointestinal complaints, particularly when associated with hyponatraemia and hyperkalaemia. A normal basal cortisol does not exclude the diagnosis which requires ACTH stimulation testing.
艾迪生病可能呈现出多样且非特异性的临床和生化特征。存在争议的问题包括促肾上腺皮质激素（ACTH）刺激试验解读的适当标准以及为确定肾上腺功能减退的特定病因所需的检查范围。回顾了一家南非教学医院的艾迪生病病例，以（1）记录病因及临床表现范围，（2）检查ACTH刺激试验的表现，（3）确定肾上腺CT扫描和活检的效用。
对1980年至1997年入住一家南非教学医院且诊断为急性艾迪生病的患者进行回顾性研究。
通过查阅医院记录确定了50例急性艾迪生病患者。记录治疗前的生化和血液学参数。分析静脉注射250微克合成促皮质素（考的松）后20分钟和60分钟时的皮质醇反应。在一部分受影响的受试者中，在长期随访期间测量腰椎和股骨颈的骨密度（BMD）。
临床表现包括色素沉着（86%）、体重减轻（67%）、腹痛（20%）和腹泻（16%）。39例患者（78%）血钠降低，26例（53%）血钾升高。9例患者（18%）血糖降低，21%血钙升高。基础皮质醇平均为148纳摩尔/升（范围10 - 487），16例患者（40%）基础皮质醇正常。ACTH刺激后20分钟皮质醇平均为172纳摩尔/升（范围19 - 588）。ACTH刺激后血清皮质醇无显著升高（P > 0.05）。仅24例患者（48%）进行了肾上腺CT扫描，其中10例正常，14例发现异常（11例双侧增大，2例钙化，1例肾上腺萎缩）。8例患者在随访期间进行了双能X线吸收法扫描——4例腰椎骨质减少，5例股骨颈骨质减少。艾迪生病的可能病因中，特发性占42%，与活动性结核病相关的占18%，陈旧性结核病相关的占16%，自身免疫性的占12%，恶性肿瘤伴转移的占6%——个别病例分别由结节病、铁过载和肾上腺脑白质营养不良引起。2例患者进行了肾上腺活检，均诊断为恶性肿瘤。住院后第一个月内的死亡率为12%。
根据我们的经验，艾迪生病常为特发性，表现形式多样，对于不明原因色素沉着或胃肠道不适的患者，尤其是伴有低钠血症和高钾血症时，应考虑该病。基础皮质醇正常不能排除诊断，仍需进行ACTH刺激试验。
