Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

HLA Associations in Oligo- and Polyarticular RF (-) Juvenile Idiopathic Arthritis-Associated Uveitis.

Angeles-Han5,  Sheila, Zeft8,  Andrew S., Bohnsack8,  John F., Clifford7,  Bronte, Ponder1,  Lori, Prozonic3,  Jennifer, Brown3,  Milton

Children's Healthcare of Atlanta
Emory Children's Center, Atlanta, GA
Emory Children's Center
Emory Children's Center, Decatur, GA
Emory University, Atlanta, GA
Emory University School of Medicine, Atlanta, GA
Nationwide Children's Hospital Research Institute
University of Utah, Salt Lake City, UT


Juvenile idiopathic arthritis-associated uveitis (JIA-U) can lead to complications such as cataracts, glaucoma, and synechiae, and increase risk for vision loss and blindness. Retrospective studies show an association between polymorphisms of HLA class II genes and JIA-U, especially in females with the oligoarticular subtype and early disease. While HLA-DR11 and HLA-DR13 alleles increase susceptibility to disease, HLA-DR1 is protective. Most studies have not included children who have poly RF (-) JIA-U. We sought to validate previously reported HLA associations and investigate the association between HLA-DRB1 in oligo- and polyarticular RF (-) JIA-U. Most prior studies also have not examined the effect of carriage of two risk alleles, which we sought to do.


Thirty children of Northern European ancestry with oligo- or polyarticular JIA-U were followed in pediatric rheumatology and ophthalmology clinics. Chart reviews confirmed the diagnoses of JIA and uveitis. Controls were 100 healthy adults screened for several common autoimmune disorders. Cases and controls were matched for ethnicity. High resolution sequence based typing was performed to determine HLA-DRB1 genotypes. Statistical analysis was performed using chi-square or fisher's exact test for odds ratio (OR).


Of 30 subjects, 43.3% were females, 76.7% were ANA positive, 83.3% had oligo JIA and 16.7% had RF (-) poly JIA. Mean age of JIA diagnosis was 3.0 years (SD 2.4). Of 10 subjects, mean age of JIA-U diagnosis was 3.4 years (SD 2.5) with a mean of 20 months (SD 28.5) of arthritis before uveitis onset. In our cohort, we found the following DR13 alleles –*0101 (n=9), *1302 (n=2), and *1303 (n=2), and the following DR11 alleles- *1101 (n=7), and *1103 (n=3). There was evidence for an association between JIA-U and DRB1*13 (OR 2.18, p=0.08), and DRB1*11 (OR 6.71, p=0.0003). There was also an association between carriage of either DRB1*11 or DRB1*13 (OR 3.25, P=0.005). There was a very strong association between JIA-U and carrying two copies of risk alleles (DRB1*11 and 13) (OR 30, p=0.0000007). There was a decreased frequency of DRB1*01 among cases with JIA-U compared to controls, although this was not statistically significant, reflecting the modest sample size.


We have validated the reported associations between DRB1*13 and DRB1*11 in JIA-U. We have also demonstrated the strong association between JIA-U and the presence of two risk alleles. These HLA risk genotypes should be further investigated in a large prospective group of children with JIA-U to determine the risk for uveitis severity and associated complications.

To cite this abstract, please use the following information:
Angeles-Han, Sheila, Zeft, Andrew S., Bohnsack, John F., Clifford, Bronte, Ponder, Lori, Prozonic, Jennifer, et al; HLA Associations in Oligo- and Polyarticular RF (-) Juvenile Idiopathic Arthritis-Associated Uveitis. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :256
DOI: 10.1002/art.28025

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