Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2002-2-11
pubmed:abstractText
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).
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0021-972X
pubmed:author
pubmed:issnType
Print
pubmed:volume
87
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
618-23
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed-meshheading:11836294-Addison Disease, pubmed-meshheading:11836294-Adolescent, pubmed-meshheading:11836294-Adrenal Cortex, pubmed-meshheading:11836294-Adult, pubmed-meshheading:11836294-Aged, pubmed-meshheading:11836294-Aged, 80 and over, pubmed-meshheading:11836294-Animals, pubmed-meshheading:11836294-Autoantibodies, pubmed-meshheading:11836294-Cattle, pubmed-meshheading:11836294-Child, pubmed-meshheading:11836294-Cohort Studies, pubmed-meshheading:11836294-Diabetes Mellitus, Type 1, pubmed-meshheading:11836294-Female, pubmed-meshheading:11836294-Gonads, pubmed-meshheading:11836294-Histocompatibility Antigens Class II, pubmed-meshheading:11836294-Humans, pubmed-meshheading:11836294-Male, pubmed-meshheading:11836294-Middle Aged, pubmed-meshheading:11836294-Norway, pubmed-meshheading:11836294-Random Allocation, pubmed-meshheading:11836294-Reference Values, pubmed-meshheading:11836294-Thyroid Diseases
pubmed:year
2002
pubmed:articleTitle
Autoimmune adrenocortical failure in Norway autoantibodies and human leukocyte antigen class II associations related to clinical features.
pubmed:affiliation
Department of Pediatrics, Akershus Central Hospital, N-1474 Nordbyhagen, Norway. Anne.Myhre@med.uib.no
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't