Background: Addisons disease is characterised by immune mediated destruction of the adrenal glands. This process is widely deemed to be irreversible. We present a case of confirmed Addisons disease where adrenal function appears to have spontaneously recovered.
Case report: A 37-year-old presented in 1997 with classical Addisons disease: pigmentation, dizziness, weight loss, and nausea. There was no relevant family history. Random cortisol was 43 and 56 nmol/l at 30 min post synacthen. Adrenal antibodies, ACTH and renin were not checked. Treatment with hydrocortisone 20 mg waking and 10 mg mid-afternoon, and fludrocortisone 50 μg daily led to an excellent recovery.
On subsequent registration with a new endocrinologist, gradual hydrocortisone dose reduction was suggested. Pre- and post-dose cortisols were also checked, and the pre-dose level found to be surprisingly high: 359 nmol/l. The hydrocortisone dose was therefore reduced further. Repeat Synacthen testing provoked a rise in cortisol to 596 nmol/l, and so hydrocortisone was cautiously withdrawn, with no impact on symptoms, electrolytes or blood pressure. Off treatment, 0900 h ACTH was initially elevated at 86 ng/l but has fallen to 13 ng/l, Synacthen testing remains normal, and the patient remains well after 6 months off treatment, 16 years after his initial diagnosis. Renin remains elevated so fludrocortisone has been continued. Adrenal antibody tests are now positive and aldosterone levels are pending.
Discussion: This is the third and most clear cut reported case of spontaneous recovery from Addisons. However, we have no models to predict which patients are more likely to recover than others, and so vigilance in the follow-up and monitoring of Addisons is vital. Finally, this case demonstrates the importance of patient involvement in the management of long term conditions, and has absolutely relied on detailed educational input from a specialist nurse to maintain his safety.