Addison-Krise – Risiko erkennen und rasch behandeln

Abstract

An adrenal crisis (Addisonian crisis) is an acute life-threatening complication of adrenal insufficiency. It occurs when hydrocortisone demand is not met by supplementation in the context of an infection – often gastrointestinal, fever, trauma, acute psychological or physical stress. Symptoms of weakness, nausea, muscle/joint pain and drowsiness may develop out of robust health within few hours. If overlooked, treated too late, with insufficient dose or route of application, there exists a considerable risk of mortality. Treatment consists of immediate parenteral hydrocortisone (100 mg bolus) and subsequent continuous infusion until the underlying precipitating cause is cured and/or when regular supplementation can be continued. Physicians and nurses must not underestimate the condition, since diagnosis and treatment must not be delayed. Patients, relatives and spouses need to be trained in prevention and early recognition through educational programs and emergency cards.

Die Addison-Krise ist eine seltene Komplikation der Nebenniereninsuffizienz mit Mortalitätsrisiko. Sie wird durch Kortisolmangel ausgelöst und oft zu spät und verzögert behandelt. Eine schnelle und ausreichende Hydrokortison-Substitution parenteral ist auch bei besonders gefährdeten Patienten lebensrettend. Schulungsprogramme dienen der Prävention und sorgen für die nötige Sicherheit im Umgang mit der Selbstinjektion von Hydrokortison im Notfall.

Schlüsselwörter

Nebenniereninsuffizienz - autoimmun polyglanduläres Syndrom - Kortisolmangel

Key words

adrenal insufficiency - autoimmune polyglandular syndrome - cortisol deficiency

• Literatur

• 1 Saeger W. Adrenalitis. Pathologe 2016; 37: 238-244 . doi:10.1007/s00292–016–0163-y
• 2 Arlt W, Allolio B. Adrenal insufficiency. Lancet 2003; 361: 1881-1893 . doi:10.1016/S0140–6736(03)13492–7
• 3 Allolio B. Extensive expertise in endocrinology. Adrenal crisis. Eur J Endocrinol 2015; 172: R115-R124 . doi:10.1530/EJE-14–0824
• 4 Meyer G, Badenhoop K, Linder R. Addison’s disease with polyglandular autoimmunity carries a more than 2. 5-fold risk for adrenal crises: German Health insurance data 2010–2013. Clin Endocrinol (Oxf) 2016; 85: 347-353 . doi:10.1111/cen.13043
• 5 Hahner S, Loeffler M, Bleicken B. et al. Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies. Eur J Endocrinol 2010; 162: 597-602 . doi:10.1530/EJE-09–0884
• 6 Erichsen MM, Lovas K, Skinningsrud B. et al. Clinical, immunological, and genetic features of autoimmune primary adrenal insufficiency: observations from a Norwegian registry. J Clin Endocrinol Metab 2009; 94: 4882-4890 . doi:10.1210/jc.2009–1368
• 7 Ono Y, Ono S, Yasunaga H. et al. Clinical features and practice patterns of treatment for adrenal crisis: a nationwide cross-sectional study in Japan. Eur J Endocrinol 2017; 176: 329-337 . doi:10.1530/EJE-16–0803
• 8 Bornstein SR, Allolio B, Arlt W. et al. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2016; 101: 364-389 . doi:10.1210/jc.2015–1710
• 9 Husebye ES, Allolio B, Arlt W. et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014; 275: 104-115 . doi:10.1111/joim.12162
• 10 Burger-Stritt S, Hahner S. Adrenal Insufficiency. Dtsch Med Wochenschr 2016; 141: 1740-1742 . doi:10.1055/s-0042–118276
• 11 Leblicq C, Rottembourg D, Deladoey J. et al. Are guidelines for glucocorticoid coverage in adrenal insufficiency currently followed?. J Pediatr 2011; 158: 492-498.e1 . doi:10.1016/j.jpeds.2010.08.021
• 12 Hahner S, Hemmelmann N, Quinkler M. et al. Timelines in the management of adrenal crisis – targets, limits and reality. Clin Endocrinol (Oxf) 2015; 82: 497-502 . doi:10.1111/cen.12609