title: Clinical and Immunological Characteristics of Autoimmune Addison Disease: A Nationwide Swedish Multicenter Study.
abstract: Studies of the clinical and immunological features of autoimmune Addison disease (AAD) are needed to understand the disease burden and increased mortality.
  
  
  
  To provide upgraded data on autoimmune comorbidities, replacement therapy, autoantibody profiles, and cardiovascular risk factors.
  
  
  
  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.
  
  
  
  The endpoints were the prevalence of autoimmune comorbidities and cardiovascular risk factors. Autoantibodies against 13 autoantigens were determined.
  
  
  
  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).
  
  
  
  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.
authors.affiliation: Centre for Molecular Medicine, Department of Medicine (Solna).|Science for Life Laboratory, Department of Medical Sciences, and.
authors.forename: Frida
authors.initials: F
authors.lastname: Dalin
authors.affiliation: Department of Molecular Medicine and Surgery, and.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Nordling Eriksson
authors.affiliation: Department of Public Health and Clinical Medicine and.
authors.forename: Per
authors.initials: P
authors.lastname: Dahlqvist
authors.affiliation: Centre for Molecular Medicine, Department of Medicine (Solna).
authors.forename: Åsa
authors.initials: Å
authors.lastname: Hallgren
authors.affiliation: Division of Endocrinology, Department of Medical and Health Sciences, Faculty of Health Sciences, and.
authors.forename: Jeanette
authors.initials: J
authors.lastname: Wahlberg
authors.affiliation: Department of Pediatrics, Institute of Clinical Sciences.
authors.forename: Olov
authors.initials: O
authors.lastname: Ekwall
authors.affiliation: Department of Public Health and Clinical Medicine and.
authors.forename: Stefan
authors.initials: S
authors.lastname: Söderberg
authors.affiliation: Departments of Immunology, Genetics and Pathology.
authors.forename: Johan
authors.initials: J
authors.lastname: Rönnelid
authors.affiliation: Department of Laboratory Medicine and.
authors.forename: Per
authors.initials: P
authors.lastname: Olcén
authors.affiliation: Translational Immunology, Department of Medicine (Solna), and.
authors.forename: Ola
authors.initials: O
authors.lastname: Winqvist
authors.affiliation: Department of Molecular Medicine and Surgery, and.|Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Karolinska Institutet, Stockholm SE-17176, Sweden.
authors.forename: Sergiu-Bogdan
authors.initials: SB
authors.lastname: Catrina
authors.affiliation: Institution of Clinical Science, Pediatrics, Umeå University, Umeå SE-90736, Sweden.
authors.forename: Berit
authors.initials: B
authors.lastname: Kriström
authors.affiliation: Department of Public Health and Clinical Medicine and.
authors.forename: Maria
authors.initials: M
authors.lastname: Laudius
authors.affiliation: Medical Sciences, and.
authors.forename: Magnus
authors.initials: M
authors.lastname: Isaksson
authors.affiliation: Women's and Children's Health, Uppsala University, Uppsala SE-75236, Sweden.
authors.forename: Maria
authors.initials: M
authors.lastname: Halldin Stenlid
authors.affiliation: Women's and Children's Health, Uppsala University, Uppsala SE-75236, Sweden.
authors.forename: Jan
authors.initials: J
authors.lastname: Gustafsson
authors.affiliation: Women's and Children's Health, Uppsala University, Uppsala SE-75236, Sweden.
authors.forename: Gennet
authors.initials: G
authors.lastname: Gebre-Medhin
authors.affiliation: Department of Molecular Medicine and Surgery, and.|Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Karolinska Institutet, Stockholm SE-17176, Sweden.
authors.forename: Sigridur
authors.initials: S
authors.lastname: Björnsdottir
authors.affiliation: Department of Women's and Children's Health, Karolinska Institutet, Stockholm SE-17176, Sweden.
authors.forename: Annika
authors.initials: A
authors.lastname: Janson
authors.affiliation: Department of Laboratory Medicine and.
authors.forename: Anna-Karin
authors.initials: AK
authors.lastname: Åkerman
authors.affiliation: Department of Pediatrics, Faculty of Medicine and Health, Örebro University, Örebro SE-70281, Sweden.
authors.forename: Jan
authors.initials: J
authors.lastname: Åman
authors.affiliation: Division of Pediatrics, Department of Clinical and Experimental Medicine, Linköping University, Linköping SE-58183, Sweden.
authors.forename: Karel
authors.initials: K
authors.lastname: Duchen
authors.affiliation: Department of Internal Medicine and Clinical Nutrition, Institute of Medicine, and.|Department of Endocrinology, Sahlgrenska University Hospital, Sahlgrenska Academy, University of Gothenburg, Gothenburg SE-40530, Sweden.
authors.forename: Ragnhildur
authors.initials: R
authors.lastname: Bergthorsdottir
authors.affiliation: Department of Internal Medicine and Clinical Nutrition, Institute of Medicine, and.|Department of Endocrinology, Sahlgrenska University Hospital, Sahlgrenska Academy, University of Gothenburg, Gothenburg SE-40530, Sweden.
authors.forename: Gudmundur
authors.initials: G
authors.lastname: Johannsson
authors.affiliation: Department of Pediatrics, Institute of Clinical Sciences.
authors.forename: Emma
authors.initials: E
authors.lastname: Lindskog
authors.affiliation: Department of Endocrinology, Skåne University Hospital, Lund SE-22362, Sweden; and.
authors.forename: Mona
authors.initials: M
authors.lastname: Landin-Olsson
authors.affiliation: Department of Pediatrics, Pediatric Endocrinology, Clinical Sciences, Lund University, Lund SE-22362, Sweden.
authors.forename: Maria
authors.initials: M
authors.lastname: Elfving
authors.affiliation: Department of Endocrinology, Skåne University Hospital, Lund SE-22362, Sweden; and.
authors.forename: Erik
authors.initials: E
authors.lastname: Waldenström
authors.affiliation: Department of Molecular Medicine and Surgery, and.
authors.forename: Anna-Lena
authors.initials: AL
authors.lastname: Hulting
authors.affiliation: Centre for Molecular Medicine, Department of Medicine (Solna).|Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Karolinska Institutet, Stockholm SE-17176, Sweden.
authors.forename: Olle
authors.initials: O
authors.lastname: Kämpe
authors.affiliation: Department of Molecular Medicine and Surgery, and.|Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Karolinska Institutet, Stockholm SE-17176, Sweden.
authors.forename: Sophie
authors.initials: S
authors.lastname: Bensing
mesh_terms: D000224:Addison Disease / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology*; D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000453:epidemiology / Q000276:immunology; D002318:Cardiovascular Diseases / Q000453:epidemiology / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003430:Cross-Sectional Studies; D004334:Drug Administration Schedule; D005260:Female; D020249:Hormone Replacement Therapy / Q000379:methods; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D012042:Registries; D012307:Risk Factors; D013548:Sweden / Q000453:epidemiology; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D006854:Hydrocortisone