THERAPY OF ENDOCRINE DISEASE: Perspectives on the management of adrenal insufficiency: clinical insights from across Europe

Abstract

BackgroundConventional glucocorticoid (GC) replacement for patients with adrenal insufficiency (AI) is inadequate. Patients with AI continue to have increased mortality and morbidity and compromised quality of life despite treatment and monitoring.Objectivesi) To review current management of AI and the unmet medical need based on literature and treatment experience and ii) to offer practical advice for managing AI in specific clinical situations.MethodsThe review considers the most urgent questions endocrinologists face in managing AI and presents generalised patient cases with suggested strategies for treatment.ResultsOptimisation and individualisation of GC replacement remain a challenge because available therapies do not mimic physiological cortisol patterns. While increased mortality and morbidity appear related to inadequate GC replacement, there are no objective measures to guide dose selection and optimisation. Physicians must rely on experience to recognise the clinical signs, which are not unique to AI, of inadequate treatment. The increased demand for corticosteroids during periods of stress can result in a life-threatening adrenal crisis (AC) in a patient with AI. Education is paramount for patients and their caregivers to anticipate, recognise and provide proper early treatment to prevent or reduce the occurrence of ACs.ConclusionsThis review highlights and offers suggestions to address the challenges endocrinologists encounter in treating patients with AI. New preparations are being developed to better mimic normal physiological cortisol levels with convenient, once-daily dosing which may improve treatment outcomes.

Adrenal Crisis: Still a Deadly Event in the 21st Century

“…The introduction of a pan-European Emergency card with clear instructions on the necessity of early treatment will hopefully empower allied health care providers and physicians to institute treatment. 46 For physicians who are less familiar with this uncommon condition, it is prudent to listen to patients and their families who often do know best, and in times of uncertainty, heed patients' requests for more hydrocortisone. 47 Increased 33 ‡Ensure patients are given the same "first on the list" priority for scheduled surgery as patients with insulin-dependent diabetes.…”

“…The new European Emergency Card provides information in both English and 8 different local languages. 46 Emergency numbers and directions to hospitals should be identified before travel, and they should be discouraged from traveling to places where medical care is limited. Appropriate vaccinations should be administered, and caution should be exercised in prevention of gastrointestinal infections.…”

Recent Advances in the Pharmacotherapy of Infantile Spasms

“…Physicians should be aware that adrenal insufficiency may become longstanding and even permanent in exceptional cases [55]. Even minor degrees of adrenal insufficiency can be fatal in the stressed host [56].…”

Nebenniereninsuffizienz und Nebenniereninzidentalome

“…Ein Anstieg des Cortisolwertes auf >18 μg/dL (>500 nmol/L bzw 180 ng/mL) gilt als beweisend für eine suffiziente Nebennierenfunktion. Dieser Test wird klinisch sehr gut vertragen und kann nahrungsunabhängig zu jeder Tageszeit durchgeführt werden [3,4] …”