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Symptoms ⬇

Acute pain, blurred vision, and photophobia. Associated systemic complaints may include lower back, cervical, or heel pain (typically worse on awakening), arthritis, oral ulcers, pain with urination, gastrointestinal complaints, and rashes.

Signs ⬆ ⬇

Critical

Recurrent, unilateral (or alternating bilateral) nongranulomatous anterior uveitis.

Other

Severe anterior chamber reaction with cell, flare, and fibrin. Most common cause of unilateral hypopyon. Tendency to form posterior synechiae early. Ciliary flush. More common in men than women.

Differential Diagnosis ⬆ ⬇

  • Other hypopyon uveitides: Behçet disease (posterior involvement more common than in HLA-B27), infectious endophthalmitis, retinoblastoma, metastatic tumors, drug-induced (e.g., rifabutin), sarcoidosis, and masquerade syndromes.
  • Idiopathic anterior uveitis.

Types of HLA-B27 Disease

  • HLA-B27-associated uveitis without systemic disease.
  • Ankylosing spondylitis: Young adult men, often with lower back pain or stiffness, abnormalities on sacroiliac spine radiographs, increased ESR, positive HLA-B27, and negative rheumatoid factor (seronegative spondyloarthropathy).
  • IBD: Crohn disease and ulcerative colitis. Chronic diarrhea, bloody stool, and crampy abdominal pain. Patients who have IBD and are HLA B27-negative may be more likely to get sclerokeratitis or peripheral ulcerative keratitis than uveitis.
  • Reactive arthritis (Reiter syndrome): Young adult men, conjunctivitis, urethritis, polyarthritis, occasionally keratitis, increased ESR, and positive HLA-B27. May have recurrent episodes. Arthritis tends to involve the lower extremities.
  • Psoriatic arthritis: Characteristic skin findings with arthritis typically involving the upper extremities.
NOTE:

Over half of patients presenting with HLA-B27-positive acute anterior uveitis have an underlying seronegative spondyloarthropathy, and of those, over half are diagnosed only after the onset of uveitis.

Work Up ⬆ ⬇

Workup
  1. HLA-B27 to confirm the diagnosis.
  2. Ankylosing spondylitis: Sacroiliac spine radiographs or CT scan show sclerosis and narrowing of the joint spaces; ESR often elevated but nonspecific.
  3. IBD: A medicine or gastroenterology consult.
  4. Reactive arthritis: Conjunctival and urethral swabs for chlamydia if indicated. Consult medicine or rheumatology.
  5. Psoriatic arthritis: A rheumatology or dermatology consult.

Treatment ⬆

See 12.1, ANTERIOR UVEITIS (IRITIS/IRIDOCYCLITIS). Patients with HLA-B27 uveitis often suffer multiple recurrences. For particularly severe relapsing cases, consider longer-term steroid-sparing immunomodulatory therapy, often in conjunction with rheumatology.