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Recovery of adrenal function in a patient with confirmed Addison's disease

机译:已确认艾迪生氏病的患者的肾上腺功能恢复

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SummaryAddison's disease is a condition characterised by immune-mediated destruction of the adrenal glands leading to a requirement of lifelong replacement therapy with mineralocorticoid and glucocorticoid. We present a case of a 53-year-old man who presented at the age of 37 years with nausea, fatigue and dizziness. He was found to have postural hypotension and buccal pigmentation. His presenting cortisol level was 43?nmol/l with no response to Synacthen testing. He made an excellent response to conventional replacement therapy with hydrocortisone and fludrocortisone and then remained well for 16 years. On registering with a new endocrinologist, his hydrocortisone dose was revised downwards and pre- and post-dose serum cortisol levels were assessed. His pre-dose cortisol was surprisingly elevated, and so his dose was further reduced. Subsequent Synacthen testing was normal and has remained so for further 12 months. He is now asymptomatic without glucocorticoid therapy, although he continues on fludrocortisone 50?μg daily. His adrenal antibodies are positive, although his ACTH and renin levels remain elevated after treatment. Addison's disease is generally deemed to lead to irreversible cell-mediated immune destruction of the adrenal glands. For this reason, patients receive detailed counselling and education on the need for lifelong replacement therapy. To our knowledge, this is the third reported case of spontaneous recovery of the adrenal axis in Addison's disease. Recovery may therefore be more common than previously appreciated, which may have major implications for the treatment and monitoring of this condition, and for the education given to patients at diagnosis.Learning pointsPartial recovery from Addison's disease is possible although uncommon.Patients with long-term endocrine conditions on replacement therapy still benefit from regular clinical and biochemical assessment, to revisit optimal management.As further reports of adrenal axis recovery emerge, this may influence the counselling given to patients with Addison's disease in the future.
机译:总结艾迪生氏病是一种以免疫介导的肾上腺破坏为特征的疾病,导致需要使用盐皮质激素和糖皮质激素进行终身替代治疗。我们介绍了一个53岁的男性患者,该患者在37岁时出现恶心,疲劳和头晕。发现他患有体位性低血压和颊色素沉着。他目前的皮质醇水平为43?nmol / l,对Synacthen测试无反应。他对使用氢化可的松和氟可的​​松的传统替代疗法做出了出色的反应,然后保持了16年。向新的内分泌科医生报名后,他的氢化可的松剂量向下调整,并评估了给药前和给药后的血清皮质醇水平。他的服药前皮质醇异常升高,因此他的剂量进一步降低。随后的Synacthen测试正常,并已保持12个月。尽管他每天继续服用氟可的松50μg,但他现在没有糖皮质激素治疗就没有症状。尽管治疗后他的ACTH和肾素水平仍然升高,但他的肾上腺抗体是阳性的。通常认为艾迪生氏病会导致不可逆的肾上腺细胞介导的免疫破坏。因此,患者需要接受终身替代疗法的详细咨询和教育。据我们所知,这是第三例报道的阿迪森氏病肾上腺轴自发恢复的病例。因此,康复可能比以前意识到的更为普遍,这可能对这种状况的治疗和监测以及诊断时对患者的教育产生重大影响。学习要点尽管从艾迪生病中恢复并不常见,但可能会部分康复。替代疗法的内分泌情况仍可从常规临床和生化评估中受益,以重新获得最佳治疗。随着更多关于肾上腺轴恢复的报道的出现,这可能会影响未来对艾迪生病患者的咨询。

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