PMID- 11836294
OWN - NLM
STAT- MEDLINE
DCOM- 20020301
LR  - 20220331
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 87
IP  - 2
DP  - 2002 Feb
TI  - Autoimmune adrenocortical failure in Norway autoantibodies and human leukocyte 
      antigen class II associations related to clinical features.
PG  - 618-23
AB  - Autoimmune destruction of the adrenal cortex is the most common cause of primary 
      adrenocortical insufficiency (Addison's disease) in industrialized countries. We 
      have investigated a large Norwegian cohort of patients with Addison's disease in 
      terms of clinical manifestations, autoantibodies, and human leukocyte antigen 
      (HLA) class II haplotypes. The study comprised 94 patients (54 females) of ages 
      6-85 yr (mean 45 yr) with, either isolated Addison's disease or part of 
      autoimmune polyendocrine syndrome type II. Among those diagnosed before the age 
      of thirty, 53% were men, while among those diagnosed at 30 or above, 30% were 
      men. Altogether 77 (82%) of the 94 patients had autoantibodies against 
      21-hydroxylase (21OH). Thirty-eight of the 40 patients with disease duration 5 yr 
      or less had such autoantibodies. This frequency fell to 60% among patients with a 
      disease duration greater than 35 yr. Five women had gonadal failure. This failure 
      correlated with antibodies against side-chain cleavage enzyme (P = 0.03). 
      Antibodies against glutamic acid decarboxylase and IA2 correlated with the 
      presence of type 1 diabetes (P < 0.005 and P = 0.003, respectively). The 
      frequency of the HLA DRB1*03-DQA1*05-DQB1*02 (DR3-DQ2) and 
      DRB1*04-DQA1*03-DQB1*0302 (DR4-DQ8) haplotypes were positively correlated to 
      Addison's disease, whereas the DRB1*01-DQA1*0101-DQB1*0501 (DR1-DQ5) haplotype 
      was negatively correlated. In addition, the DRB1*04 subtype DRB1*0404 was 
      increased among Addison patients relative to controls. We verify that 
      autoimmunity is the main cause of Addison's disease in our cohort. In younger 
      patients, the disease is equally common in men and women. Measurement of 
      autoantibodies against 21OH is a valuable tool in establishing the etiological 
      diagnosis, especially in patients with a short disease duration. Addison's 
      disease is associated with the DR3-DQ2 and DR4 (0404)-DQ8 haplotypes. A 
      particularly high risk for disease development is observed when these occur in a 
      heterozygous combination (DR3-DQ2/DR4-DQ8).
FAU - Myhre, Anne Grethe
AU  - Myhre AG
AD  - Department of Pediatrics, Akershus Central Hospital, N-1474 Nordbyhagen, Norway. 
      Anne.Myhre@med.uib.no
FAU - Undlien, Dag E
AU  - Undlien DE
FAU - Lovas, Kristian
AU  - Lovas K
FAU - Uhlving, Sverre
AU  - Uhlving S
FAU - Nedrebo, Bjorn G
AU  - Nedrebo BG
FAU - Fougner, Kristian J
AU  - Fougner KJ
FAU - Trovik, Thor
AU  - Trovik T
FAU - Sorheim, Jan I
AU  - Sorheim JI
FAU - Husebye, Eystein S
AU  - Husebye ES
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 0 (Autoantibodies)
RN  - 0 (Histocompatibility Antigens Class II)
SB  - IM
MH  - Addison Disease/complications/immunology/*physiopathology
MH  - Adolescent
MH  - Adrenal Cortex/immunology/*physiopathology
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Animals
MH  - Autoantibodies/analysis
MH  - Cattle
MH  - Child
MH  - Cohort Studies
MH  - Diabetes Mellitus, Type 1/complications
MH  - Female
MH  - Gonads/physiopathology
MH  - Histocompatibility Antigens Class II/analysis
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Norway
MH  - Random Allocation
MH  - Reference Values
MH  - Thyroid Diseases/complications
EDAT- 2002/02/12 10:00
MHDA- 2002/03/02 10:01
CRDT- 2002/02/12 10:00
PHST- 2002/02/12 10:00 [pubmed]
PHST- 2002/03/02 10:01 [medline]
PHST- 2002/02/12 10:00 [entrez]
AID - 10.1210/jcem.87.2.8192 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2002 Feb;87(2):618-23. doi: 10.1210/jcem.87.2.8192.