Anterior Uveitis

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Symptoms

  • Pain, redness, photophobia, tearing, possible reduction of visual acuity

Medical history

  • First attack or recurrent?
  • Systemic diseases/ concomitant rheumatological diseases?
  • Extended medical history:
    • Symptoms: Joint pain, back pain, skin lesions, discolouration/ numbness of hands and feet, diarrhoea, night sweats, fever, weight loss, dry mouth/ eyes, herpes blisters, ulcers in the mouth/ genital area.
    • Other: travel history, ancestry, animals, tick bite, smoking/drinking habits, drug use, sexual history, medication, occupation, family history.

Aetiology

  • Non-granulomatous
    • idiopathic (approx. 50%)
    • autoimmune: HLA-B27 associated: Ankylosing spondylitis (Bechterew’s disease), reactive arthritis, psoriatic arthritis, inflammatory bowel disease, Behcet’s disease (HLA-B51), juvenile rheumatoid arthritis, Kawasaki syndrome, other autoimmune diseases (lupus erythematosus, polychondritis, Wegener’s granulomatosis, interstitial nephritis)
    • others: postoperative, trauma, UGH syndrome
  • granulomatous
    • infectious: herpes simplex virus, varicella zoster virus, syphilis, tuberculosis, leprosy, brucellosis, toxoplasmosis, propionibacterium acnes, fungi (cryptococcus, aspergillus), HIV
    • Immunological: sarcoidosis, VKH syndrome, sympathetic ophthalmia, lens-associated uveitis (phacoanaphylactic uveitis).
    • others: Fuchs heterochromic iridocyclitis, glaucomatocyclitic crisis (Posner-Schlossmann syndrome).

Findings

  • Ciliary vascular injection
  • Reactive miosis
  • Anterior chamber cells / flare, fibrin, hypopyon
  • Endothelial precipitates
    • fine (“stellate”): very fine precipitates, often entire endothelium
    • small, non-granulomatous
    • granulomatous (“mutton-fat”): large, “fatty” precipitates, often inferior endothelium
  • Posterior synechiae
  • Iris nodules (Busacca/Koeppe), iris atrophy (diffuse, focal, segmental), heterochromia
  • Vitreous cells (in case of spill-over)
  • Increased intraocular pressure (-> HSV, Posner-Schlossmann, Fuchs’ heterochromic iridocyclitis)

Examination

  • Distinguish whether granulomatous or non-granulomatous
    • Non-granulomatous: small, fine, dust-like endothelial precipitates.
    • Granulomatous: somewhat larger precipitates, “greasy”, partly confluent often inferiorly in Arlt’s triangle
      • small stellate precipitates distributed over the entire endothelium (also granulomatous) in Fuchs’ heterochromic cyclitis
  • Corneal epithelium
    • Dendrites: HSV
      • tree-like ulceration, sunken in epithelium, blunt ends with “terminal bulbs” 1 2
      • central fluorescein staining
    • Pseudodendritica: VZV
      • “stuck-on” appearance, rather pointed ends without terminal bulbs 3
      • little to no fluorescein staining
  • Intraocular pressure
    • elevated: typically in granulomatous: HSV, VZV, Fuchs’ heterochromic cyclitis, sarcoidosis
      • very high IOP in glaucomatocyclitic crisis (Posner-Schlossmann syndrome)
      • low: typically in severe uveitis with inflammation of the ciliary body, e.g. in HLA-B27+ (non-granulomatous)
  • Iris
    • Atrophy
      • Diffuse (patchy) iris transillumination: HSV, Fuchs’ heterochromic cyclitis
        • Fuchs’: affected iris is usually lighter colored (=heterochromia)
          • CAVE: if the iris is very bright, it can also be darker than the opposite side.
      • Sector-shaped iris transillumination: VZV
    • Granulomas: nodules in granulomatous uveitis (sarcoidosis, tuberculosis, Fuchs’ heterochromic cyclitis)
      • Koeppe: at pupillary margin
      • Busacca: iris stroma
      • Berlin: in the anterior chamber angle, typically in sarcoidosis
  • Lens
    • posterior subcapsular cataract: heterochromic cyclitis
  • Typically unilateral: Fuchs’ heterochromic cyclitis

Work-up

  • In case of a mild first episode with no signs of systemic disease: no further work-up needed
  • In case of a severe first manifestation or recurrent uveitis:
    • Differential blood count, CRP, BSR, ANA, Syphilis, Tuberculosis, HLA-B 27, Lyme disease, ACE + Lysozyme + IL-2
    • Chest X-ray / Chest CT if necessary
  • Consider anterior chamber puncture (e.g. in the case of segmental iris atrophy (HSV?) or in the absence of a response to therapy).
  • Consider rheumatology consult

Therapy

  • Pred Forte gtt (Prednisolon) initially usually hourly (depending on anterior chamber cells) + consider additional Ultracortenol ointment (Prednisolon) at night; reduce slowly if response is good.
    • Possible regimen:
      Pred Forte hourly for 1 week, then two-hourly for 1 week, then 5x/d for 1 week, then 4x/d for 1 week,
      then 3x/d for 1 week, then 2x/d for 1 week, then 1x/d for 1 week, then STOP
  • Scopolamine gtt 2x/d if marked anterior chamber cells (to prevent posterior synechiae)
  • in case of long persistent anterior chamber cells/ pronounced inflammation:
    • Consider systemic therapy with Spiricort (Prednisolon), initially 1mg/kg or subconjunctival application of Triamcinolon (Kenakort -> subconjunctivally after local anaesthesia, 10mg superior and 10mg inferior or 20mg inferior).
    • CAVE: rule out infectious causes before starting therapy with systemic steroids!

Sources