Treatment of Addison’s disease during pregnancy

Abstract

SummaryAddison’s disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant patient with Addison’s disease, referred to Obstetrics-Endocrinology specialty consult at 14 weeks gestation. She had been to the emergency department of her local hospital various times during the first trimester presenting with a clinical scenario suggestive of glucocorticoid under-replacement (nausea, persistent vomiting and hypotension), but this was interpreted as normal pregnancy symptoms. Hydrocortisone dose was adjusted, and the patient maintained regular follow-up. No complications were reported for the remainder of gestation and delivery. Pregnant patients with Addison’s disease should be monitored during gestation and in the peripartum period by multidisciplinary teams. Adjustments in glucocorticoid and mineralocorticoid replacement therapy are often necessary, and monitoring should be based mainly on clinical findings, which becomes increasingly difficult during pregnancy. Patient education and specialized monitoring are key to avoiding complications from under- or over-replacement therapy in this period.Learning points:An increase in glucocorticoid replacement dose is expected to be necessary during pregnancy in a woman with Addison’s disease.Patient education regarding steroid cover and symptoms of acute adrenal crisis are fundamental.Monitoring in this period is challenging and remains mainly clinical.The increase in hydrocortisone dose often obviates the need to increase fludrocortisone dose.

Diagnosing and managing adrenal crisis in pregnancy: A case report

“…There is typically a low clinical suspicion of new adrenal insufficiency in pregnancy, given the overlapping signs of symptoms with normal pregnancy, nausea and vomiting in pregnancy, and hyperemesis gravidarum such as fatigue, nausea, vomiting, and mild hypotension [ 7 ]. In fact, only 100 cases of adrenal insufficiency have been reported to be diagnosed during pregnancy [ 8 ].…”

Autoimmune Polyglandular Syndrome Type 2 and Pregnancy

“…In developed countries, the most common reason for this (about 70% of all cases) is autoimmune adrenalitis. 3,8,9 Women with Addison's disease have markedly reduced fertility with multifocal genesis, especially when combined with another autoimmune disorder such as Hashimoto's disease and/or type 1 diabetes mellitus. On the other hand, adrenal insufficiency leads to the loss of androgens, which affects the fertility, libido and sexual activity.…”

“…Considering this fact, pregnancies in patients with Addison's disease are rare. 3,8,9 The course of pregnancy in patients with Addison's disease is associated with a number of complications and the risk of an adrenal crisis. Quite often, the main symptoms that mark the onset of an Addison's crisis may be linked to pregnancy-related complaints, such as severe fatigue, nausea and vomiting.…”

“…It is very important to maintain adequate glucocorticoid replacement therapy to avoid the adverse effects of over-treatment (gestational diabetes mellitus, weight gain, arterial hypertension) and of under-treatment (low birth weight, adrenal crisis in the mother, electrolyte imbalance). 8,9 There is no ideal replacement dose of glucocorticoid therapy for pregnant women with Addison's disease. Most of them require a 20-40% increase in the baseline dose after 24 th week.…”

“…This may lead to augmenting therapy with mineralocorticoid in some pregnant patients with Addison's disease. 9,8 Fludrocortisone is administrated according to the electrolyte profile and the general condition. The patient should be informed about potential benefits and risks of using the medication during pregnancy.…”