HLA-B27 Associated Uveitis

HLA-B27 associated uveitis is the commonest anterior uveitis, typically acute, unilateral, alternating between eyes, and recurrent. It often accompanies spondyloarthritis.

• HLA-B27 is present in about 8 percent of white people and about 50 percent of patients with acute anterior uveitis.
• Associated disease: ankylosing spondylitis (lifetime uveitis risk about 30 to 40 percent), reactive arthritis, psoriatic arthritis, enteropathic arthritis (inflammatory bowel disease), and undifferentiated spondyloarthritis.
• Many patients present with uveitis before the diagnosis of arthritis; about 50 percent have undiagnosed spondyloarthritis at first uveitis presentation.
• Typical features: male predominance (about 3:1), age 20 to 40 years.

• Sudden painful red eye with photophobia, usually with marked inflammation: heavy cells and flare, fibrin, hypopyon (in about 15 to 25 percent), posterior synechiae, fine keratic precipitates; usually only the anterior segment is involved, but cystoid macular oedema and vitritis can occur.
• Characteristic alternating pattern: one eye affected at a time, with episodes separated by weeks to years.
• Systemic: inflammatory back pain (onset under 40 years, insidious, morning stiffness over 30 minutes, improvement with exercise, night pain in the second half), buttock pain, enthesitis (heel pain), dactylitis, peripheral arthritis, psoriasis, urethritis or diarrhoea (reactive arthritis), abdominal symptoms (inflammatory bowel disease).
• Complications: posterior synechiae, cataract, glaucoma, macular oedema, hypotony.

• Clinical diagnosis: HLA-B27 testing (supports the diagnosis, high positive predictive value in recurrent unilateral non-granulomatous uveitis).
• Spondyloarthritis assessment: ask about back pain; examine spine and sacroiliac joints; pelvis X-ray and magnetic resonance imaging of sacroiliac joints (sacroiliitis), CRP and ESR.
• Exclude other causes when atypical: syphilis serology, chest X-ray, interferon gamma release assay.
• Examine for psoriasis, and ask about bowel symptoms (faecal calprotectin when suggestive).

• Acute episode: topical prednisolone acetate 1 percent hourly tapering over 4 to 6 weeks, cycloplegia (cyclopentolate 1 percent three times daily), and subconjunctival or sub-Tenon steroid or short oral prednisolone course for severe disease.
• Prevention of recurrences in patients with 2 or more episodes in 12 months, bilateral or severe disease, or active arthritis:
  • Adalimumab 80 mg loading then 40 mg every 2 weeks, reducing recurrences by about 50 to 60 percent and also treating arthritis.
  • Infliximab 5 mg/kg at weeks 0, 2 and 6, then every 8 weeks.
  • Sulfasalazine 500 mg daily increasing to 1.5 to 2 g twice daily, an option for peripheral arthritis and recurrent anterior uveitis.
  • Methotrexate 15 to 25 mg weekly if anti-tumour necrosis factor therapy is unsuitable.
• Etanercept is less effective for uveitis and may be associated with new onset uveitis; avoid it for patients with uveitis.
• Rheumatology care: non-steroidal anti-inflammatory drugs (naproxen 500 mg twice daily, etoricoxib 60 to 90 mg daily), physiotherapy, and biologic therapy for spondyloarthritis.
• Complications: pressure management, cataract surgery after at least 3 months of quiet inflammation with perioperative steroid cover, treat macular oedema with sub-Tenon triamcinolone 40 mg or acetazolamide.
• Counsel patients to attend urgently for any red painful eye to limit synechiae and damage; recurrences are more frequent in the first 3 to 5 years and decline with time.