# HLA-DR1

HLA-DR1 (DR1) is a HLA-DR serotype that recognizes the DRB1*01 gene products. It has been observed to be common among centenarians.

## Serology

DR1 serotype recognition of Some DRB1*01 allele-group gene products :
| DRB1* | DR1 | DR103 | | Sample |
| allele | % | % | % | size (N) |
| *01:01 | 97% | | | 6317 |
| *01:02 | 95% | | | 2035 |
| *01:03 | 56% | 12% | | 1186 |
| *01:05 | >50% | | | 2 |
The serology for the most common DR1 alleles is excellent. The serology for alleles *01:04, *01:06, *01:09, *01:10, *0112, *0115, and *0116 is unknown.

## Disease associations
### By serotype

DR1 is associated with seronegative-rheumatoid arthritis, penicillamine-induced myasthenia, and schizophrenia. DR1 is increased in patients with systemic sclerosis and arthritis and in ulcerative colitis with patients that have articular manifestations.

### By allele

DRB1*01:01 is associated with rheumatoid arthritis, in anti-Jk(a) mediated hemolytic transfusion reactions, foliaceous pemphigus, HTLV-1-associated myelopathy/tropical spastic paraparesis, and lichen planus. In lyme disease arthritis, *01:01 appears to play a role in presentation of triggering microbial antigens.
DRB1*01:02 is associated with rheumatoid arthritis, in anti-Jk(a) mediated hemolytic transfusion reactions, psoriasis vulgaris, and recurrent respiratory papillomatosis
DRB1*01:03 is associated with colonic Crohn's disease and ulcerative colitis.

### By genotype

DRB1*01:01/*0404 and *01:01/*0401 increases risk of mortality in rheumatoid arthritis, with ischemic heart disease and smoking. these same genotypes are associated with rheumatoid vasculitis.

### By haplotype

DRB1*01:02:DQB1*05:01 is associated with psoriasis vulgaris and tubulointerstitial nephritis & uveitis syndrome, but is relatively protective against juvenile diabetes.
DR1-DQ5 is associated with tubulointerstitial nephritis & uveitis syndrome.
Diseases associated with HLA-DR and links to DR subpages(V - T) :
| Class | Disease | Associated DR | 2 | 3 | 4 |
| | alopecia areata | DR5 | | | |
| anemia | pernicious | DR15 | | | |
| | antiphospholipid syndrome, primary | DR5 | DR12 | | |
| aneurysm | coronary artery | DR16 | | | |
| arteritis | Takayasu's | DR16 | | | |
| arthritis, rheumatoid | juvenile | DR4 | DR5 | DR14 | DR15 |
| arthritis, rheumatoid | pauciarticular, juv. | DR8 | | | |
| arthritis, rheumatoid | Still's disease | DR12 | | | |
| arthritis, rheumatoid | iritis w/juv. arthritis | DR12 | | | |
| arthritis, rheumatoid | seropositive | DR1 | DR4 | DR10 | |
| arthritis, rheumatoid | w/systemic sclerosis | DR1 | | | |
| arthritis, rheumatoid | lyme disease induced | DR4 | | | |
| arthritis, rheumatoid | tiopronin intolerance | DR5 | DR11 | DR12 | |
| cardiomyopathy | hypertrophic | DR4 | DR17 | | |
| cardiomyopathy | T. cruzi induced | DR4 | DR7 | DR15 | |
| colitis | Crohn's | DR1 | | | |
| colitis | ulcerative | DR1 | | | |
| diabetes | juvenile (type 1) | DR3 | DR4 | DR17 | DR18 |
| diabetes | fatty liver (type 2) | DR8 | | | |
| encephalomyelitis | rabies vaccine-induced | DR17 | | | |
| encephalopathy | acute necrotizing | DR52 | | | |
| epilepsy | childhood | DR5 | | | |
| epilepsy | infantile/spasm | DR17 | | | |
| heart disease | rheumatic | DR16 | | | |
| hepatitis | autoimmune | DR2 | DR4 | DR17 | |
| hepatitis | primary biliary cirrhosis | DR2 | DR8 | | |
| hepatitis | chronic type C | DR11 | | | |
| | lichen planus | DR1 | DR10 | | |
| lupus, | systemic | DR3 | DR4 | DR52 | |
| lupus, | hydralazine-induced | DR4 | | | |
| lupus, | with Sjögren syndrome | DR15 | | | |
| lymphadenopathy | generalized | DR5 | | | |
| lymphoma, | mycosis fungoides | DR5 | | | |
| | melioidosis | DR16 | | | |
| myasthenia | gravis | DR3 | DR6 | DR13 | DR14 |
| myasthenia | penicillamine-induced | DR1 | | | |
| myositis | inflammatory inclusion body | DR17 | DR18 | DR52 | |
| | narcolepsy | DR2 | DR12 | | |
| nephritis, | tubulointerstitial | DR1 | | | |
| nephropathy | IgA-mediated | DR4 | | | |
| | polyglandular deficiency syndrome | DR5 | | | |
| pemphigus | foliaceous | DR1 | | | |
| pemphigus | vulgaris | DR4 | | | |
| psoriasis | vulgaris | DR1 | DR7 | | |
| papillomatosis, | respiratory | DR1 | | | |
| sarcoidosis | non-chronic | DR17 | DR52 | | |
| sclerosis, | multiple | DR2 | DR15 | DR53 | |
| sclerosis, | "bout onset" multiple | DR3 | | | |
| sclerosis, | systemic | DR4 | DR11 | DR16 | DR52 |
| sclerosis, | vulval lichen | DR12 | | | |
| schizophrenia | schizophrenia | DR1 | | | |
| susceptibility | leprosy | DR2 | | | |
| susceptibility | tuberculosis | DR2 | | | |
| susceptibility | ragweed Ra6 allergy | DR5 | | | |
| susceptibility | asthma, mite sensitive | DR11 | | | |
| susceptibility | 2ndary infection, AIDS | DR3 | | | |
| susceptibility | aspergillosis | DR15 | | | |
| susceptibility | Kaposi's sarcoma | DR5 | | | |
| susceptibility | thyroid carcinomas | DR8 | DR11 | | |
| susceptibility | ovarian/cervical cancer | DR10 | DR11 | DR15 | |
| susceptibility | grape induced anaphylaxis | DR11 | | | |
| susceptibility | Chlamydia pneumoniae | DR52 | | | |
| thyroiditis | Hashimoto's | DR3 | DR5 | | |
| thyroiditis | Graves' | DR3 | DR17 | DR52 | |
| uveitis | tubulointerstitial | DR1 | | | |
| $^*$references are provided on linked subpages ||||||

## Rheumatoid arthritis

DR1 are associated with rheumatoid arthritis, and while not the strongest association with the highest risk for early onset arthritis is within the DR4-bearing Native American population. There frequency of DR4-DQ8 haplotypes reach extreme nodal levels. Arthritis has been identified in a pre-Columbian remains from Italy, the affected individual bearing the DRB1*01:01 allele. DRB1*01:01 and most DR4 have in common a 'shared epitope'. In this hypothesis a common region of the beta chain, positions 67 to 74, are common and may be integral to presenting auto-immunological peptides.

## Genetic linkage

HLA-DR1 is not genetically linked to DR51, DR52 or DR53, but is linked to HLA-DQ1 and DQ5 serotypes.