Uvea: Uveitis Flashcards
What is uveitis?
- Broad range of disorders
- Any inflammation of any part of uveal tract
- Wide range of causes and associations
What is the classification of location of uveitis?
o Anterior – iris
o Intermediate – ciliary body
o Posterior – choroid
o Panuveitis – all layers
What is the classification of cause of uveitis?
o Infectious – bacterial, viral, parasitic, fungal
o Non-infectious – inflammatory (most cases)
o Masquerade – neoplastic or non-neoplastic (uveitis is not predominant problem it could be a lymphoma for e.g.)
What is the classification of onset of uveitis?
o Acute
o Recurrent – quiet periods in between episodes
o Chronic – quiet period lasts <3 months
o Persistent – episode lasts >3 months
What is the anterior chamber grading for flare?
- Standardisation of Uveitis Nomenclature group (SUN)
- Not looking at cells but looking at general look of anterior chamber
GRADE DESCRIPTION
0 None
1+ Faint
2+ Moderate – iris & lens detail clear
3+ Marked – iris & lens details hazy
4+ Intense – fibrin or plastic aqueous
What is the vitreous grading of haze in uveitis?
HAZE SEVERITY GRADING
Good view of nerve fibre layer (NFL) 0
Clear disc & vessels but hazy NFL +1
Disc & vessels hazy +2
Only disc visible +3
Disc not visible +4
Things like cataract can affect this, AC changes can also impact this
What are the symptoms of uveitis?
- Red eye
- Pain
- Photophobia
- Watering
- Blurred vision
- Onset is usually rapid – within days but can be insidious
- None – particularly in children with Juvenile Idiopathic Uveitis
- Systemic symptoms – enquire about these
What are the signs of uveitis?
- Ciliary/ limbal injection – red eye – injection at the limbus
- Keratic precipitates – inflammatory aggregates that sit on the corneal endothelium
- AC Cells
- AC Flare
- Posterior synechiae – pupil margin gets stuck to lens
- Iris atrophy – so much inflammation that some pigmented tissue of iris is lost – can see red reflex through iris
- Cataract – can be due to inflammation itself or to prolonged steroid use
- High or low IOP
Describe anterior uveitis & its management?
- Commonest form of uveitis is acute anterior uveitis (AAU)
- 50% idiopathic
- Infectious – viral (commonest), TB, syphilis, Lyme disease
- Non-infectious – usually idiopathic – associated with systemic conditions
- Masquerade – neoplastic or non-neoplastic – things like lymphomas
- Management:
o Single episode AAU does not require investigation
o Recurrent AAU, bilateral severe AAU or chronic anterior uveitis (CAU) need investigation – investigation is to exclude infectious causes or any systemic causes & to look for any other things that may be causing the uveitis
o Uveitis screen:
Blood tests – looking for inflammatory markers & indicators of other conditions
Urine sample – a form of uveitis can be associated with kidney disease
Chest x-ray
o May consider “AC tap” – if uveitis v severe and concerned it is not settling down
Fine needle is inserted into limbus & small sample of aqueous taken and investigated – tested for viral/bacterial causes & cancerous causes
What are the systemic associations of acute anterior uveitis?
- Spondyloarthropathies – e.g. ankylosing spondylitis – associated with HLA-B27 – HLA is Human Leukocyte Antigen (cell surface proteins on human cells that are responsible for regulation of immune system) – variations/abnormalities of HLA types can affect regulation of immune system & can cause autoimmune conditions
- Sero-negative arthropathies – don’t have HLA +ve disease but can still have inflammatory autoimmune conditions
- Inflammatory bowel disease
- Sarcoidosis – largely respiratory condition but can also affect eyes and skin
- Behcet’s disease – affects skin, causes mouth ulcers, autoimmune condition
- Tubulointerstitial nephritis and uveitis (TINU) – uveitis w/ kidney disease
- Multiple sclerosis – typically causes optic neuritis but can also cause anterior uveitis or intermediate uveitis
- Systemic lupus erythematosus (SLE) – autoimmune condition that can be associated with uveitis
- Juvenile idiopathic arthritis
What is the treatment for acute anterior uveitis?
- Topical:
o Predforte (steroid)
o Maxidex (steroid)
o Cyclopentolate (cycloplegia – given as symptomatic relief)
Paralyses ciliary body and dilates pupil (breaks posterior synechiae – best to do this at the onset of the condition – important as once pupil margin is stuck down to lens it affects the aqueous flow)
o Atropine (cycloplegia – given as symptomatic relief)
Paralyses ciliary body – Limits ciliary spasm helping reduce symptoms of photophobia & pain - Periocular:
o Subconj dexamethasone (steroid)
o Subconj betnosal (steroid)
o Subconj mydricaine (combo of anaesthetic agent and cyclopentolate) - Intraocular:
o Ozurdex implant (dexamethasone) – used if no systemic associations or if condition affecting 1 eye only - Systemic:
o Oral steroids –> Oral second line immunosuppression (after trying the oral steroids 1st)
Describe Fuchs Hetereocromic Cyclitis (FHC)?
- Chronic non-granulomatous uveitis
- Often asymptomatic – low grade grumbling uveitis
- 90% unilateral
- Usually present with reduced vision – due to cataract – not likely to be symptoms of pain due to the low grade inflammation
- Signs:
o Iris heterochromia – lighter eye is affected eye
o Stellate KPs – only condition where KPs on corneal endothelium are affecting entire cornea
o Mild flare, occasional cells (0.5+)
o Iris atrophy
o Iris nodules
o Cataract
o Raised IOP - Treatment:
o Observation – if low grade inflammation
o Topical steroids for exacerbations – e.g. pain and other uveitis symptoms
o Topical treatment for glaucoma – may need laser/surgery if topical tx not effective
Glaucoma caused due to the inflammation being so insidious that it can affect the trab meshwork causing the glaucoma
o Cataract surgery – higher risk of complications due to anatomy/inflammation in these pxs
Describe juvenile idiopathic arthritis (JIA)?
- Commonest condition associated with childhood uveitis
- Asymptomatic therefore diagnosed with arthritis are screened
- Uveitis may be presenting feature of the disease
- ALL CHILDREN (<12 YRS OLD) W/ UVEITIS SHOULD BE REFERRED TO EYE CLINIC
- Signs:
o Band keratopathy
o Uveitis
o Posterior Synechiae (PS)
o Cataract
o Glaucoma - Treatment:
o Topical treatments for anterior uveitis – often insufficient
o Joint treatment with paediatricians & rheumatology
o Require immunosuppression – e.g. methotrexate
o Oral steroids not used in children as can slow down growth & cause other problems so often go straight to 2nd line immunosuppression
Describe intermediate uveitis (IU)?
- Involves ciliary body
- Primary site of inflammation is vitreous
- 50% are idiopathic
- Systemic associations:
o Sarcoidosis
o Multiple Sclerosis
o Behcet’s
o Infectious causes - ALL PATIENTS WITH SIGNS OF INTERMEDIATE UVEITIS MUST BE REFERRED URGENTLY TO EYE CASUALTY CLINIC – need treated to reduce risk of sight loss
- Symptoms:
o Blurred vision
o Floaters
o Pain/redness – can be variable
o Photophobia – can be variable - Signs:
o May have AC cells
o High or low IOP
o Vitreous Cells and vitreous haze
o Cataract
o Snowballs – cells in vitreous clump together and get yellowish deposits that form in inferior vitreous
o Snowbanking – large collections of inflammation – usually associated with pars planitis
o Cystoid Macular Oedema - Investigation:
o Uveitis screen (as for AAU)
o Fluorescein angiography
o AC or vitreous tap – if px has poor response to tx or if have worrying signs of infection then can perform this – use tiny needle, inject into AC or vitreous and sent away for testing
Describe the treatment & viral causes of intermediate uveitis?
- Treatment:
o Topical:
Predforte
Maxidex
Cyclopentolate
Atropine
o Antibiotic/antiviral/antifungal
o Intra-ocular steroid:
Ozurdex implant
o Systemic Steroid – tend to be used almost all time in intermediate uveitis
o 2nd line immunosuppression
o 3rd line immunosuppression - Viral Causes:
o Herpes Simplex Virus (HSV) – causes dendritic ulcers
o Varicella Zoster Virus (VZV) – causes chicken pox & shingles
o Cytomegalovirus (CMV) – more common in immunosuppressed pxs
o Epstein Barr Virus (EBV) – more common in immunosuppressed pxs
o More common in immunosuppressed patients
o When see these pxs ALWAYS dilate them to make sure no signs of vitritis or any posterial or fundal diseases
Describe cytomegalovirus (CMV) retinitis?
- Usually immunosuppressed patients
- Most commonly seen in HIV patients when CD4 count is less than 50
o CD4 count is count of how well pxs immune system is doing (<50 means not working well)
o Opportunistic infection – in healthy eye these viruses would not cause any infections but in someone with compromised immune system they will cause infections
Compromised immune system could be due to drug intake or HIV etc - Treatment is for the eye but also to prevent infection elsewhere
- HAART therapy in HIV pxs – immune boosting tx
What are the bacterial causes of intermediate uveitis?
- Tuberculosis
- Syphilis
- Lyme disease
- Cat-scratch disease
- Brucellosis
- Endogenous endophthalmitis – if have sepsis elsewhere in body – little bits of the infection can travel along the arterial blood flow and lodge in the eye and cause this – not had surgery or trauma but still ended up with infection in the eye – needs to be treated in same way a post-op endophthalmitis would be – aggressive tx
Describe Posterior Uveitis?
Encompasses clinical entities of Retinitis, choroiditis & retinal vasculitis. Some lesions may originate in retina or choroid but often there is involvement of both (retinochoroiditis & chorioretinitis)
* Presentation: varies according to location of inflammation & presence of vitritis. A px w/ peripheral lesion may complain of floaters but px w/ lesion involving macula will predominantly complain of impaired central vision
* Retinitis: may be focal (solitary), multifocal, geographic or diffuse. Active lesions are characterised by whitish retinal opacities w/ indistinct borders due to surrounding oedema. As lesion resolves, borders become more defined.
* Choroiditis: may be focal, multifocal, geographic or diffuse. Does not usually induce vitritis in absence of concomitant retinal involvement. Active choroiditis is characterised by a round, yellow nodule.
* Vasculitis: may occur as a primary condition or as secondary phenomenon adjacent to a focus of retinitis. Both arteries (periarteritis) & veins (perophlebitis) may be affected, although venous involvement is more common. Active vasculitis is characterised by yellowish or grey-white, pathy, perivascular cuffing sometimes associated with haemorrhage. Quiescent vasculitis may leave peripvascular scarring, which should not be mistaken for active disease
Describe Acute Retinal Necrosis (ARN) & the treatment?
- Herpes Simplex Virus (HSV)
- Varicella Zoster Virus (VZV)
- Necrosis (death) of retinal tissue – can affect vision but can also cause retinal holes, breaks tears and detachments
- Needs tx to preserve sight and also retina as a whole
- ARN usually starts out in peripherally and moves in towards disc
- ARN occurs in pxs who are able to amount an immune response – pxs who have a normal immune system – when they get a virus, if the virus develops in the eye, the eye’s response to that is to generate a lot of inflammation to try and get rid of that virus & so have lot of inflammation in eye . Degree of progression can be quite rapid w/ prominent vitritis
- Both of these (ARN & PORN) are really serious & both require URGENT tx – if concerned at all about anyone who has retinal changes that look anything like this then they need to be referred to eye clinic ASAP
- ARN Treatment:
o Intravitreal Foscarnet – antiviral agents
o IV Aciclovir
o Oral Valaciclovir – prodrug – broken down into acyclovir once in body – used more frequently nowadays
o Famciclovir
o Systemic corticosteroids – used once treated with antivirals
o Topical corticosteroids
o Barrier laser – 3rd line tx – used to stop the active areas progressing into healthier retina – results are variable – px can still end up with retinal detachments & variable vision
Describe Progressive Outer Retinal Necrosis (PORN) & the treatment?
- Herpes Simplex Virus (HSV)
- Varicella Zoster Virus (VZV)
- Necrosis (death) of retinal tissue – can affect vision but can also cause retinal holes, breaks tears and detachments
- Needs tx to preserve sight and also retina as a whole
- PORN usually starts centrally and moves outwards
- In PORN, tends to happen in pxs who are immunosuppressed – body is unable to react to virus – unable to generate inflammation – disease progresses v rapidly but don’t have much vitritis –> will have good view of the retina
- Both of these (ARN & PORN) are really serious & both require URGENT tx – if concerned at all about anyone who has retinal changes that look anything like this then they need to be referred to eye clinic ASAP
- PORN Treatment:
o Intravitreal Foscarnet – antiviral agents
o IV Ganciclovir
o HAART therapy if HIV +ve
