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      Treatment of Addison’s disease during pregnancy

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          Summary

          Addison’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.

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          Most cited references4

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          What is the best diagnostic and therapeutic management strategy for an Addison patient during pregnancy?

          A new diagnosis of primary adrenal insufficiency (PAI) during pregnancy is extremely rare and difficult to recognize as signs and symptoms such as nausea, fatigue and hypotension may resemble features of normal pregnancy. However, if the diagnosis is overlooked and steroid replacement delayed, subsequent adrenal crisis triggered by hyperemesis gravidarum, fever or delivery can cause severe maternal and foetal morbidity and even mortality. In case of clinical suspicion of PAI, we recommend to measure paired samples of cortisol and ACTH and, if clinically feasible, a short synacthen test. We propose trimester-specific pass cut-offs for the short synacthen test that take into account the rise of total and also free cortisol during pregnancy. Empirical hydrocortisone treatment should never be delayed if the clinical suspicion is high. All pregnant women with PAI should be monitored by a team of endocrine and obstetric specialists. The third trimester is physiologically associated with a rise not only in total but also free cortisol and thus requires regular adjustment of the glucocorticoid dose. Mineralocorticoid requirements may change during pregnancy due to the anti-mineralocorticoid properties of progesterone. As plasma renin physiologically increases in pregnancy, monitoring is limited to clinical assessment including blood pressure and serum electrolytes. It is crucial that a pregnant woman with PAI and her partner are well educated regarding the adjustment of glucocorticoid dose in intercurrent illness and that both are trained in hydrocortisone emergency injection techniques. The obstetric staff should be provided with clear and written guidance for hydrocortisone cover during labour and delivery. With the appropriate replacement therapy, PAI patients can expect to have an uneventful pregnancy and deliver a healthy infant. © 2012 Blackwell Publishing Ltd.
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            Mineralocorticoid substitution and monitoring in primary adrenal insufficiency.

            Patients with primary adrenal insufficiency usually show pronounced impairment of aldosterone secretion and, therefore, require also mineralocorticoid replacement for full recovery. Clinical signs of mineralocorticoid deficiency comprise hypotension, weakness, salt craving and electrolyte disturbances (hyperkalemia, hyponatremia). Mineralocorticoid deficiency is confirmed by demonstration of profoundly decreased aldosterone and highly elevated plasma renin activity (PRA). Standard replacement consists of 9α-fluorocortisol (fludrocortisone) given once daily as a single oral dose (0.05-0.2 mg). Monitoring of mineralocorticoid replacement consists of clinical assessment (well-being, physical examination, blood pressure, electrolyte measurements) and measurement of PRA aiming at a PRA level in the upper normal range. Current replacement regimens may often be associated with mild hypovolemia. Dose adjustments are frequently needed in pregnancy to compensate for the anti-mineralocorticoid activity of progesterone and in high ambient temperature to avoid sodium depletion. In arterial hypertension a dose reduction is usually recommended, but monitoring for hyperkalemia is required.
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              Addison's disease in women is a risk factor for an adverse pregnancy outcome.

              Autoimmune Addison's disease (AAD) tends to affect young and middle-aged women. It is not known whether the existence of undiagnosed or diagnosed AAD influences the outcome of pregnancy.
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                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                12 April 2018
                2018
                : 2018
                : 17-0179
                Affiliations
                [1]Endocrinology , Diabetes and Metabolism Department, Coimbra Hospital and University Center, Coimbra, Portugal
                Author notes
                Correspondence should be addressed to D Oliveira Email: dianapontooliveira@ 123456gmail.com
                Article
                EDM170179
                10.1530/EDM-17-0179
                5900459
                50154f59-ace3-42be-93bd-a1a1fff62221
                © 2018 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 06 March 2018
                : 23 March 2018
                Categories
                Error in Diagnosis/Pitfalls and Caveats

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