HLA-DR4

HLA-DR4 (DR4) is a HLA-DR serotype that recognizes the DRB1*04 gene products. The DR4 serogroup is large and has a number of moderate frequency alleles spread over large regions of the world.

=Serology=

The serological identification of DR4 is good. The serology of DRB1*0417 to *0460 is unknown.

=Disease associations=

By serotype
DR4 is associated extraarticular rheumatoid arthritis, hydralazine-induced female systemic lupus erythematosus ,pemphigoid gestationis , phemphigus foliaceus , obstructive hypertrophic cardiomyopathy , IgA nephropathy and 'shared syndrome'-systemic schlerosis/rheumatoid arthritis.

By allele
DRB1*04 is associated with increased risk for alopecia areata.

DRB1*0401 is associated with multiple sclerosis, rheumatoid arthritis , type 1 diabetes , lyme disease induced arthritis

DRB1*0402: drug-triggered /idiopathic pemphigus vulgaris, type 1 diabetes , SLE associated anti-cardiolipin and anti-β2 glycoprotein I

DRB1*0403: polycystic ovary syndrome, SLE associated anti-cardiolipin and anti-β2 glycoprotein I

DRB1*0404: anti-citrullinated fibrinogen in rheumatoid arthritis, autoimmune hepatitis

DRB1*0405: rheumatoid arthritis, Autoimmune hepatitis , type 1 diabetes

DRB1*0406: caspase-8 autoantibodies silicosis-systemic sclerosis (SSc)-systemic lupus erythematosus (SLE)

DRB1*0409: T. cruzi infection with cardiomyopathy

By haplotype
DR4-DQ8 is a risk factor for papillary thyroid carcinoma.

DRB1*04:DQA1*0303-DQB1*0401 haplotype: type III autoimmune polyglandular syndrome, autoimmune hepatitis , autoimmune pancreatitis DRB1*04:DQA1*03-DQB1*0302 haplotype: Type 1 diabetes with DRB1*0401, *0405, *0402 increasing risk when DQ8 is present.

By genotype
DRB1*0101/*0404 and *0101/*0401 increases risk of mortality in rheumatoid arthritis, with eschemic heart disease and smoking. these same genotypes are associated with rheumatoid vasculitis.

By haplotype
HLA DR4-DQ8 juvenile diabetes, coeliac disease, rheumatoid arthritis

DRB1*04 allele group 68 Alleles: 60 proteins DR4 Serotype: *0401 to *0416 Serotype unknown: *0417 to *460

=Genetic Linkage=

=References=