HLA-B27 Associated Acute Anterior Uveitis
Uveitis is a common form of intraocular inflammation of the iris, ciliary body, or choroid, which presents predominantly as anterior uveitis (80-85%). Approximately 50% of acute anterior uveitis (AAU) cases are associated with the allele Human Leukocyte Antigen B27 (HLA-B27).
The HLA-B locus encodes a class I Major Histocompatibility Complex surface antigen which presents antigens to CD8+ T-cells.
- changes amino acids in the antigen-binding cleft
- highly polymorphic
- 105 subtypes, 132 genetic alleles
- Varies between ethnic populations
- Pawaia tribe of Papua New Guinea (53%)
- Haida natives of western Canada (50%)
- Chukotka Eskimos of eastern Russia (40%)
- Northern Scandinavia (14-16%)
- Caucasians (8-10%)
- HLA-B27 Prevalence in the United States:
- 7.5% in non-Hispanic whites
- 4.6% in Mexican-Americans
- 2-4% in African-Americans
- Age of onset: 20-40 years
- Males 1.5-2.5 times more likely than women
- Systemic associations: Psoriasis, Ankylosing Spondylitis, Inflammatory Bowel Disease, and Reactive Arthritis
- Recurrent, averaging 1-2 episodes per year
- Common episodes of inflammation alternate between eyes; rarely if ever bilateral simultaneously
- Usually resolves within two months
- Recurrence may become less frequent with longer duration
- Sudden onset
- Ocular pain
- Eye redness
- Severe inflammation potentially resulting in:
- Anterior chamber fibrin
Potential Ocular Complications
- Posterior synechiae
- Ocular hypertension or glaucoma
- Posterior subcapsular cataract
- Epiretinal membrane
- Less commonly:
- Cystoid macular edema
- Band keratopathy
- Ocular hypotony
- Mixed results whether complication rates differ between HLA-B27 and other uveitis etiologies
- Careful review of systems and systemic evaluation to look for joint, skin or bowel involvement
- HLA-B27 typing
- Consider Rheumatology referral and/or xray of sacroiliac joint
- Consider Syphilis serology, Chest xray, ACE, lysozyme to rule out other causes of uveitis
- Corticosteroids (topical drops, periocular/intraocular injection, oral) based on the amount of inflammation present; Start with high dose/frequency (such as prednisolone acetate 1% every 1-2 hours, or difluprednate 4-6 x/day or 1 mg/kg oral prednisone then taper over several weeks)
- Cycloplegia for comfort and to prevent posterior synechiae (cyclopentolate 1% twice daily for example)
- Systemic immunosuppressive medications in patients with multiple vision threatening flares and systemic diseases (anti-metabolites, biologics) 
- Adalimumab approved only for AAU patients with spondyloarthropathy
- FDA approval for non-infectious posterior or panuveitis (not anterior uveitis)
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