The aching red photophobic eye

Uveitis, which unsurprisingly means inflammation of the uveal tract. Note the typical history and important findings of a constricted slightly irregular pupil with ciliary flush (i.e. circumcorneal involvement of the diffusely injected conjunctiva).

Different terms may be used for uveitis depending which components of the uveal tractare involved, such as:

• iritis — inflammation of the iris
• cyclitis — ciliary body
• choroiditis — choroid

Alternatively uveitis may be referred to as:

• Anterior uveitis — aka iridocyclitis, which involves inflammation of the anterior portion of the uveal tract and usually affects young or middle-aged persons.
• Posterior uveitis — which includes vitritis, choroiditis, retinitis, chorioretinitis, or retinochoroiditis.
• Panuveitis — aka diffuse uveitis, which refers to both anterior and posterior involvement.

Uveitis can be acute or chronic/ recurrent.

There are many, many causes of uveitis!

There are 4 broad categories:

• inflammatory
• traumatic
• infectious
• drug-induced

Inflammatory causes —
HLA B-27 associated — this HLA antigen is present in 30—70% of patients with anterior uveitis, half of whom have an associated systemic disease such as:

• ankylosing spondylitis
• psoriatic arthritis
• reactive arthritis
• inflammatory bowel disease

Non-HLA-B27 associated uveitis may result from  numerous underlying systemic conditions such as:

• sarcoidosis
• juvenile idiopathic arthritis
• Behçet disease
• Kawasaki disease
• >multiple sclerosis
• Wegeners’ granulomatosis

Infectious causes:

• Parasitic — e.g. toxoplasmosis (accounts for 25% of retinochoroiditis in the US)
• Viral — e.g. cytomegalovirus (CMV), herpes viruses, adenovirus, measles, mumps, influenza
• bacterial — e.g. tuberculosis, leptospirosis, syphilis
• ricketsial disease — e.g. Lyme disease

Traumatic causes:

• Traumatic iritis, usually secondary to a direct blow from a blunt object

Drug-induced:

• e.g. rifabutin, cidofovir, sulfonamides, pamidronate

Uveitis can also occur secondary to other ocular conditions (e.g. lens-induced, post-operative, anterior segment ischemia, tumour).

History

• Anterior uveitis —
  Painful red eye — deep ache radiating to periorbital or temple area, worse with movement and accommodation.
  photophobia
  +/- blurred vision
• Posterior uveitis —
  “floaters” and visual changes — blind spots or flashes may be present with retinal involvement.
  Redness or significant pain are usually absent.
• Features of an underlying cause (see Q2) — inflammatory (HLA-B27 vs non-HLA-B27 associated conditions), infectious traumatic, or drugs.
• Duration and previous episodes?

Examination

• Visual acuity —often normal, but may be reduced in posterior uveitis
• Pupils —
  constricted, may be irregular, and is sluggish in response to light in comparison with the uninvolved eye.
  Direct and consensual photophobia (the latter may help differentiate anterior uveitis from from superficial causes of photophobia).
• Red reflex — may be normal or altered, e.g. secondary cataract.
• Slit lamp —
  Conjunctiva — diffusely injected conjunctiva with circumcorneal involvement aka ‘ciliary flush’ (no perlimbal sparing as occurs in conjunctivitis). Tearing, but no purulent discharge.
  Cornea — keratitic precipitates (deposits of white blood cells on the endothelium) may be visualized and are a hallmark of iritis. There are two types: nongranulomatous (small-medium) and granulomatous (large — e.g. sarcoidosis).
  Anterior chamber — cloudy anterior chamber due to inflammatory cells and proteinaceous flare. Synechieae (adhesions of the iris to the cornea or lens) may be present. Look for a hypopyon.
  Posterior chamber — cells and flare may be present in posterior uveitis.
  
• Tonometry — check for secondary glaucoma
• Fundoscopy — look for evidence of chorioditis and retiinits, or complications such as retinal detachment.

RootAtlas.com has nice brief videos of nongranulmatous and granulomatous KPs, as well as an excellent ‘must see’ video demonstrating ‘cells and flare’ in the anterior chamber.

Investigations are rarely required as part of the emergency management of uveitis, however they may expedite subsequent treatment.

Investigations may not be required if:

• known underlying cause (e.g. sarcoidosis or drugs)
• mild unilateral nongranulomatous disease with no evidence of systemic disease

Infectious uveitis, although rare, must be ruled out before treatment with immunosuppressive agents. Sometimes it is only suspected after a failure of treatment.

Investigations may include:

• identification of specific findings on ophthalmologic and general examination
• infectious work up — specific serology testing (e.g. syphilis), PCR, and cultures (e.g. STD screen), HIV tests
• HLA-B27 typing
• CXR and/or CT chest if pulmonary symptoms — e.g. sarcoidosis, tuberculosis
• Imaging of the sacroiliac joints if suspected spondyloarthropathy as a diagnosis.
• Colonoscopy if gastrointestinal symptoms or heme-positive stools — e.g. inflammatory bowel disease

Important complications include:

• cataracts
• glaucoma (e.g. due to synechiae)
• retinal detachment
• ophthalmitis

• Urgent referral to ophthalmology.
• Seek and treat underlying causes.
• Treat complications e.g. glaucoma
• Mydriatics (eg, sympathomimetics like phenylephrine HCl) —  prevent the formation of synechiae by pupillary dilation.
• Parasympatholytic agents (eg, atropine, cyclopentolate) — produce both mydriasis and cycloplegia  reducing pain and photophobia
• Treat with corticosteroids after consultation with an ophthalmologist and consideration of a possible infectious etiology:

• Topical corticosteroids (eg, prednisolone acetate 1%) — useful for anterior uveitis, but due to poor penetration are less effective for posterior uveitis.

• Systemic corticosteroid therapy — usually reserved for bilateral disease that is refractory to local medication or for patients with significant ocular disability or retinitis.

• Other immunosuppressants — may be used in steroid-dependent or refractory uveitis.

All cases of uveitis should be discussed with an ophthalmologist.

• Uncomplicated noninfectious uveitis — outpatient management with ophthalmology follow up within 24 hours.
• Refractory or infectious uveitis — usually require admission and further work up.

Bilateral recurrent alternating anterior uveitis

Hypopyon

With severe inflammation leukocytes can settle in the anterior chamber and form a hypopyon, which is a white or yellowish accumulation of purulent material.