Journal Article

Clinical and Immunological Characteristics of Autoimmune Addison Disease: A Nationwide Swedish Multicenter Study

Bensing, Sophie, Lindskog, Emma, Isaksson, Magnus, Halldin Stenlid, Maria, Rönnelid, Johan, Kriström, Berit, Ekwall, Olov, Kämpe, Olle, Dalin, Frida, Duchen, Karel, Björnsdottir, Sigridur, Hulting, Anna-Lena, Åman, Jan, Åkerman, Anna-Karin, Wahlberg, Jeanette, Catrina, Sergiu-Bogdan, Söderberg, Stefan, Bergthorsdottir, Ragnhildur, Landin-Olsson, Mona, Gebre-Medhin, Gennet, Nordling Eriksson, Gabriel, Dahlqvist, Per, Hallgren, Åsa, Olcén, Per, Elfving, Maria, Winqvist, Ola, Laudius, Maria, Gustafsson, Jan, Janson, Annika, Johannsson, Gudmundur, Waldenström, Erik

2017

Overview

Context:Studies of the clinical and immunological features of autoimmune Addison disease (AAD) are needed to understand the disease burden and increased mortality.Objective:To provide upgraded data on autoimmune comorbidities, replacement therapy, autoantibody profiles, and cardiovascular risk factors.Design, Setting, and Participants:A cross-sectional, population-based study that included 660 AAD patients from the Swedish Addison Registry (2008–2014). When analyzing the cardiovascular risk factors, 3594 individuals from the population-based survey in Northern Sweden, MONICA (monitoring of trends and determinants of cardiovascular disease), served as controls.Main Outcome Measures:The endpoints were the prevalence of autoimmune comorbidities and cardiovascular risk factors. Autoantibodies against 13 autoantigens were determined.Results:The proportion of 21-hydroxylase autoantibody-positive patients was 83%, and 62% of patients had ≥1 associated autoimmune diseases, more frequently coexisting in females (P < 0.0001). AAD patients had a lower body mass index (P < 0.0001) and prevalence of hypertension (P = 0.027) compared with controls. Conventional hydrocortisone tablets were used by 89% of the patients, with a mean dose of 28.1 ± 8.5 mg/d. The mean hydrocortisone equivalent dose normalized to the body surface was 14.8 ± 4.4 mg/m2/d. A greater hydrocortisone equivalent dose was associated with a greater incidence of hypertension (P = 0.046).Conclusions:Careful monitoring of AAD patients is warranted to detect associated autoimmune diseases. Contemporary Swedish AAD patients did not have an increased prevalence of overweight, hypertension, type 2 diabetes mellitus, or hyperlipidemia. However, high glucocorticoid replacement doses could be a risk factor for hypertension.Upgraded data regarding autoimmune comorbidities, autoantibody profiles, replacement therapy and cardiovascular risk factors revealing a need to carefully monitor AAD patients.

• Publisher: Endocrine Society,Copyright Oxford University Press,Oxford University Press
• Subject: Addison Disease - complications / Addison Disease - drug therapy / Addison Disease - epidemiology / Addison Disease - immunology / Addison's disease / Adolescent / Adult / Aged / Autoantibodies / Autoantibodies - blood / Autoantigens / Autoimmune diseases / Autoimmune Diseases - epidemiology / Autoimmune Diseases - immunology / Body mass index / Body weight / Cardiovascular diseases / Cardiovascular Diseases - epidemiology / Cardiovascular Diseases - etiology / Child / Child, Preschool / Clinical Medicine / Comorbidity / Cross-Sectional Studies / Diabetes mellitus / Diabetes mellitus (non-insulin dependent) / Disease / Drug Administration Schedule / Endocrine disorders / Female / Glucocorticoids / Health risk assessment / Hormone Replacement Therapy - methods / Humans / Hydrocortisone / Hydrocortisone - administration & dosage / Hydrocortisone - therapeutic use / Hydroxylase / Hyperlipidemia / Hypertension / Immunology / Infant / Infant, Newborn / Klinisk medicin / Male / Medical and Health Sciences / Medicin och hälsovetenskap / Middle Aged / Overweight / Population studies / Registries / Risk Factors / Sweden - epidemiology / Tablets / Young Adult