Continuing education CET

Uveitis a practical approach

Teifi James describes the differential diagnosis, underlying associations and management options for various forms of uveitis. Module C12371, suitable for optometrists and DOs

ptometrists and opticians need to have a strategy for dealing with uveitis. This article will help you to develop a game plan before you are obliged to deal with the uveitis patient on the other side of the slit lamp in your consulting room. You dont need to be afraid of uveitis and it is important not to panic. If you are systematic and thoughtful, you can be amazingly helpful to the patient, GP and your local ophthalmology department. The first and most important question you must ask yourself when you discover intraocular inflammation should be is this an incidental finding at a routine sight test or a symptomatic presentation?. If you divide all uveitis patients into these two categories it will help you enormously. Differential diagnosis There are many different types of uveitis and the style of presentation will often point to a diagnosis. An asymptomatic adult patient with no particular complaints who attends for routine refraction with cells in the anterior chamber will probably have Fuchs uveitis syndrome (FUS). The absence of photophobia, the absence of posterior synechiae, the lack of redness, lack of pain and the presence of characteristic stellate KPs (star shaped keratic precipitates on the corneal endothelium, Figure 1) all help to confirm the diagnosis of FUS. Heterochromia is really quite rare and iris changes are often subtle or absent, so dont be flummoxed if the iris looks virtually the same in both eyes. Secondary glaucoma is also quite uncommon, so dont expect a high pressure in the affected eye. Unilateral posterior subcapsular cataract is commonly found in the visual axis of the affected eye. Vitritis can also be present and patients may confirm the presence of floaters when prompted. Fuchs is
18 | Optician | 23.10.09

Figure 1 Stellate KPs

almost always a uniocular condition and remains confined to the one eye through the course of the disease. Bilateral FUS can occur occasionally but both eyes are affected from the outset. Sequential involvement of the two eyes separated over time is unheard of in FUS. You can make a routine referral for these patients on a GOS18.
History

History For all other presentations of uveitis you must take a meticulous history and make a thorough examination. In the majority of cases the history will actually tell you whats going on and the examination can be used to confirm or refute your working diagnosis.
Systemic questions at presentation 2

History of first episode Painful, aching, tender Photophobic, watery Period of onset variable Vision blurred Absence of foreign body sensation
Systemic questions at presentation 1 Have you ever heard of: Ankylosing spondylitis? Reiters syndrome? Psoriasis? Ulcerative colitis/Crohns disease? Have you got any joint pains/ arthritis? What general problems have you? What medicines do you take?

Do you get low back pain? Is your back stiff when you awake? Do you get sore joints? Do you have sore feet? Any ulcers in mouth or elsewhere? Tick bites, cat scratches, vet On Rx for atypical mycobacteria? Clarithromycin and rifabutin?
Direct questions to ask exposure?

First or recurrent episode? Episode frequency and severity? Only ever been in one eye? Or both? Both simultaneously or at different Whats the vision like between
episodes? times?

opticianonline.net

18-21-uveitis-cet 18

26/10/09 11:40:50

CET Continuing education

Figure 2 Photophobia makes it difficult for patients to keep their eyes open

Spectrum of anterior uveitis

Acute First episode, unilateral First episode, bilateral Recurrent episodes cute presentation of chronic A Sub-acute and overspill Chronic/rare/weird/esoteric/arcane
condition

Figure 3 Mouth ulcer associated with uveitis

Direct questions to ask

First or recurrent episode? Episode frequency and severity? Only ever been in one eye? Or both? oth simultaneously or at different B hats the vision like between W
episodes? times?

Figure 4 Are the eyes red?

Here are some key questions you should ask. Have you ever had anything like this before? This question can save you a lot of time and effort. Find out if the patient has ever had anything similar before, and if so, did they attend the Hospital Eye Service? Has someone already made a diagnosis? You need to decide whether this is a first presentation or a recurrent problem. Ask what happened, when it happened, what was the sequence of events and ask if both eyes are affected or just one. Did the problems come on rapidly over one or two days? Or is it difficult to be precise about the onset of symptoms, which have developed over a few weeks or months? Is the eye painful what sort of pain? Does the pain increase on accommodation or in direct light? If the patient is photophobic, (Figure 2) ask if the photophobia is mild or severe. Is there a family history of arthritis or joint problems? Does the patient have any other diseases which may be associated with uveitis? Any joint problems, skin problems, bowel problems or frequent mouth ulcers (Figure 3)? Do they take any prescription medicines especially eye drops prescribed at the eye clinic or

by the GP? Ask specifically if the patient has ever heard of: Ankylosing spondylitis Psoriasis or psoriatic arthritis Reactive arthritis Reiters syndrome Sarcoid or sarcoidosis Behets disease Do they know anyone with a chronic cough or a diagnosis of TB? By now you should have arrived at a differential diagnosis. If somebody has a prior history of recurrent acute anterior uveitis, and they present with a painful

photophobic red eye, then ask if this episode feels just the same as the previous time(s). Foreign-body sensation implies corneal or conjunctival irritation and is most unusual in iritis or uveitis, so use this question to help differentiate between keratoconjunctivitis and intraocular inflammation. Categorise the patients presentation as one of the following: Incidental finding in a patient with few or no symptoms Recurrent episode of an acute presentation with pain and photophobia First ever presentation of a painful photophobic red eye Slow onset, subacute or chronic presentation with a known diagnosis Hitherto undiagnosed chronic uveitis without symptoms Hitherto undiagnosed chronic uveitis with vision affected. Signs Use your examination to corroborate, confirm or refute the working hypothesis you have developed with your history and direct questions. Are the eyes red? Is only the affected eye red? Is there the typical ciliary blush or circumciliary injection of acute anterior uveitis? (Figure 4). Specific signs to look for are keratic

Figure 5 Mutton fat KPs

Figure 6 Hypopyon
23.10.09 | Optician | 19

opticianonline.net

18-21-uveitis-cet 19

26/10/09 11:40:51

CET Continuing education

precipitates (KPs). Are there any KPs? Yes or no? If there are deposits on the corneal endothelium, are they suggestive of a diagnosis? A fine dusting of small KPs all over the endothelium suggests acute iritis, the stellate KPs of FUS are characteristic; larger blobby mutton fat KPs are typical of granulomatous uveitis which is usually chronic or subacute and may occur in a white eye. Mutton fat KPs (Figure 5) are rare in Britain in the 21st century and they suggest sarcoid or one of several other less common causes of uveitis. Make sure to swing the slit lamp beam around to try different styles of illumination and use the highest magnification while focused on the endothelium to get the best view of KPs. Next, focus within the aqueous. Have a general look around the anterior chamber (AC). Does the aqueous look normal and watery or is it plasmoid with a gloopy appearance. Is there a hypopyon (Figure 6)? You should always look to be certain because a small, subtle hypopyon can be overlooked. A neat trick is to switch to higher magnification and focus on the marginal tear meniscus adjusting the lower lid so that the marginal tear strip lies just over the inferior limbus. By using the meniscal tear strip as an improvised natural gonio-lens you can visualise a small hypopyonagainsttheinferiorendothelium which would otherwise be missed. Look for cells in the AC. Use a bright, narrow slit beam with high magnification with the illumination coming from the side for best visualisation of AC cells. First ask yourself if there are any cells present in the aqueous or not. Dont spend ages counting or grading how many plusses of cells there are. Cells are either absent or present. If there are cells decide if there are a few or lots. This will give you good information rapidly without having to worry about classifying the situation. If there are lots of cells, go back and double check for that hypopyon because it is more likely to be there when there are lots of cells in the AC. The pupil is extremely helpful in uveitis. Careful examination of the pupil margin will provide a lot of relevant information. Typically the pupil of the affected eye is miosed in acute anterior uveitis, so check to see if it is smaller than the unaffected (normal) eye. Look for posterior synechiae (PS). Dilating the pupils will better demonstrate PS and may break any fresh synechiae this is desirable. It is also good practice to examine the posterior segment of both eyes looking for vitreous cells, vitritis and posterior segment signs (Figures 7 and 8). If the pupil is completely stuck
20 | Optician | 23.10.09

Examination

Photophobia Red painful eye (s) Circumciliary injection/ciliary blush Keratic precipitates Cells/hypopyon Flare Aqueous appearance Miosed pupil
Possible immediate complications of acute anterior uveitis
Figure 7 Choroidal and retinal changes secondary to TB

Posterior synechiae Iris bomb Secondary glaucoma Angle closure Uveitic Steroid induced
Possible late complications of recurrent acute anterior uveitis

Band keratopathy Seclusio pupillae Secondary glaucoma damage dvanced glaucoma after repeated A ataract (Initially posterior subcapC Hypotony Phthisis
sular, eventually, white mature LO) episodes

Figure 8 Always examine the fundus in uveitis patients. This image shows pathognomonic candlewax dripping appearance of retinal phlebitis in sarcoidosis

down pupil, as established synechiae are unlikely to shift (Figure 10). Intermediate uveitis Intermediate uveitis (IU) is almost always a bilateral condition. The hallmark is the presence of cells in the anterior vitreous of both eyes. To see these cells you have to focus on the posterior lens then push forward very slightly with the slit-lamp joystick. You will see cells if they are there. There are often a few cells in the AC, but the diagnosis can be clinched by examining the inferior peripheral vitreous. Pale clumps of inflammatory cells hanging relatively immobile anchored in the vitreous close to the retinal surface are called snowballs and they are pathognomonic of IU. Occasionally there may be pale infiltrates in the inferior pars plana too, which is why IU is sometime called pars planitis. Decide whether the vitreous is clear or murky. Are the retinal details easily seen or are they unclear because of vitreous haze. Dont get hung up on a classification of the vitritis, but rather decide if it is mild or severe. Its best not to get too deeply involved in discussions about IU. Roughly 30 per cent of cases are idiopathic, 30 per cent are associated with sarcoid and 30 per cent associated with multiple sclerosis. The other 10 per

Figure 9 Slit-lamp appearance of iris bomb as a result of seclusio pupillae. (360-degree posterior synechiae). Discuss with on-call ophthalmologist immediately

down through 360, a situation called seclusio pupillae then you may see iris bomb (Figure 9). The iris looks like a ring doughnut seen from above. Although the intraocular pressure can be very high in acute iris bomb, normal or low pressure is often seen if the situation has been present for a while. Dont be surprised if dilating drops do not work effectively on a stuck

opticianonline.net

18-21-uveitis-cet 20

26/10/09 11:40:51

Continuing education CET

cent of cases are due to a whole ragbag of arcane diagnoses. A detailed discussion of posterior or panuveitis is outside the scope of this article. Most posterior segment inflammation will present with disturbed visual function and require prompt referral especially if there is cystoid macular oedema. Optometrist activity Finally, I have a special plea. Optometrists know far more ophthalmology than the doctors in the general A&E department. Sending an acute patient to a general A&E department is a complete waste of the patients time and the emergency doctors time too. If you have a patient with severe acute iritis, dilate the pupil as best you can and ring through to your local eye clinic or ask the hospital switchboard to contact the on-call ophthalmologist for you to arrange the most appropriate referral. If you are lucky enough to still have a local eye casualty department then you probably already have local guidelines for emergency referrals. Refer asymptomatic patients with an incidental finding of anterior uveitis as a routine referral in the standard way and please dont stipulate an urgent time scale for these patients on the GOS18. Patients with recurrent acute anterior uveitis experience on average six recurrent episodes in a lifetime. This mean value is extrapolated from Clive Edelstens data gathered in Ipswich, so roughly half of all RAAU patients will have six or more recurrences in a lifetime. Once someone has had more than two attacks of classic unilateral painful red photophobic iritis, they recognise the situation and can be trusted to accurately self diagnose. In Halifax and Huddersfield, I educate patients about their condition and give them permission to become self-starters. Because the timing of the next recurrence is utterly unpredictable, these patients are given an undated FP10 prescription for topical steroids and mydriatic drops stapled to an explanatory letter for the dispensing pharmacist. The patient or pharmacist can simply date the prescription when it is presented. This approach renders the next episode sub-acute because the patient self-diagnoses, obtains appropriate prescription treatment without having to see a medical practitioner first, and simply contacts the hospital uveitis clinic to arrange a follow-up visit within a couple of weeks. I developed this strategy because this patient cohort often experiences a delay in

MUltIple-cHOIce qUeStIOnS take part at opticianonline.net

1 2 3

Which of the following is NOT associated with acute anterior uveitis? A Ciliary flush B Aqueous cells and flare c Mydriatic pupil D Photophobia Which of the following are characteristic of Fuchs uveitis syndrome? A Photophobia B Posterior synechiae c Redness D Stellate keratic precipitates Which of the following systemic diseases is not associated with uveitis? A Behcets disease B Sickle cell anaemia c Crohns disease D Reiters syndrome

4 5 6

Which of the following is the most likely cause of mutton fat KPs? A Chronic granulomatous anterior uveitis B Pars planitis c Acute anterior uveitis D Fuchs uveitis syndrome Which of the following is an immediate complication of acute anterior uveitis? A Phthisis B Hypotony c Iris bomb D Lens opacification What is the average number of recurrences expected by a patient with recurrent acute anterior uveitis during their lifetime? A One B Three c Six D 10

Successful participation in this module counts as one credit towards the GOC CET scheme administered by Vantage and one towards the Association of Optometrists Irelands scheme. The deadline for responses is November 19 2009

Figure 10 Established synechiae

starting medication. They struggle to get an immediate GP appointment and many GPs are reluctant to prescribe appropriate topical steroids for fear of exacerbating undiagnosed conditions like herpes simplex keratitis. The medical insurance organisations regularly publish reports of GPs being sued for treating patients with topical steroids, but they never publish vignettes of the countless patients who have developed complications of iritis because of the delay in commencing appropriate treatment. The most useful thing the optometrist can do when dealing with an acute presentation of iritis is to aggressively

dilate the pupil. The pain and photophobia are largely due to iris spasm, so dilating the pupil relieves the pain and photophobia, breaks any synechiae which may be forming and permits examination of the fundus which is an important part of the evaluation of every uveitis patient. Teifi James runs a regional uveitis
clinic at Calderdale Royal Hospital in Halifax, West Yorkshire. Declaration of interest: Mr James has a financial interest in The EyeBag Company which sells warm compresses which are used in conjunction with mydriatic drops to dilate the pupil in the presence of posterior synechiae
23.10.09 | Optician | 21

opticianonline.net

18-21-uveitis-cet 21

26/10/09 11:40:52