uveitis Flashcards
waht is the uvea
iris, ciliary body and choroid.
what are the two structures of the iris
stroma and posterior pigmented layer
what are the zones of the stroma of the iris
pupillary zone extends from the pupillary edge to the collarette (thickest part of the iris that separates the two zones).
sphincter pupillae is located here.
what is the collarette
thickest part of the iris that separates pupillary and ciliary zone
role of sphincter pupilae made of what and supplied by
smooth muscle responsible for pupillary constriction with parasympathetic innervation (CNIII) via short ciliary nerves.
origin of ciliary zone and role
extends from the collarette to the origin of the iris at the ciliary body
with dilator pupillar
dilator pupilae made of and innervation
smooth muscle sympathetic innervation via long ciliar nerves
what is the posterior pigmented layer
A pigmented epithelial layer found at the posterior iris surface that is continuous with the neurosensory retina posteriorly.
what are the layers of the ciliary bpdy
stroma - CT where the vascular supply is found
muscle - PNS via CNIII
epithelium
blood supply to the ciliary body
anterior and long posterior ciliary arteries
parts of the ciliary body and its role
2) pars plana
- avascular
- lies between the ora serrata and ciliary processes of the pars plicata.
- Functionless,
- site for intravitreal injections
- vitreous removal (pars plana vitrectomy).
1) pars plicata
- highly vascularised
- forms attachments for the lens zonules
functions
- aqeous humour formation
lens accomodation
aqueous drainage via uveoscleral outflow
what is the choroid and location
posterior component of the uveal tract
between the sclera externally and RPE
consists of bruchs membrane and a vascular layer
role of the choroid
provide nutrients to the outer third of the retina
what is the bruchs membrane and its function
innermost layer before the RPE
has five layer and measures less than 4 micrometres
function - fluid transport from the choroid to the retina and is permeable to small moleules
what is the vascular layer of the choroid
has three layers
- choriocapillaries which are external to the Bruch membrane
- a medium-sized vessel layer
- a large vessel layer.
blood supply of the choroid
two long posterior ciliary arteries, the short posterior ciliary artery and anterior ciliary artery.
what is anterior uveitis
inflammation of the iris +/- pars plicata (anterior ciliary body)
what are keratic precipitates
WBCs on the posterior endothelial layer of the cornea
granulomatous v non-granulomatous precipitataes how do u differentiate in anterior uveitis
Granulomatous is defined by mutton fat KPs whereas nongranulomatous is defined with stellate KPs.
aetiology of anterior uveitis
● Idiopathic (50%).
● HLA-B27-associated conditions (psoriasis, reactive arthritis, inflammatory
bowel disease and ankylosing spondylitis).
● Inflammatory: Sarcoidosis, Behçet syndrome, systemic lupus erythematosus,
juvenile idiopathic arthritis, multiple sclerosis (MS), Fuchs’ heterochromic
cyclitis, tubulointerstitial nephritis, Posner-Schlossman syndrome.
● Infectious: Tuberculosis (TB), Lyme disease, syphilis, varicella zoster virus
(VZV).
Features of anterior uveitis
Painful red eye
blurred vision
photophobia
examination of anterior uveitis
complications
● Irregular miosed pupil: Due to inflammation of the sphincter pupillae of the iris. May predispose to posterior synechiae (adhesion between the iris and lens capsule).
● Anterior chamber cells: Due to inflammation of the ciliary body which produces aqueous humour.
● KP: Stellate or mutton fat depending on aetiology.
● Hypopyon: White cells in the anterior chamber.
● Iris atrophy and nodules: More common in chronic cases.
● Complications: Cystoid macular oedema (CMO) and cataracts.
Mx of anterior uveitis
● Investigate as appropriate based on clinical findings. For example, measuring calcium and serum ACE if sarcoidosis is suspected.
● Potent topical steroid and cyclopentolate to dilate the pupil.
what is intermediate uveitis
Inflammation of the posterior ciliary body (pars plana) and vitreous. Synonym: pars planitis.
aetiology of intermediate uveitis
● Idiopathic.
● Inflammatory: Sarcoidosis, MS, inflammatory bowel disease (ulcerative
colitis and Crohn disease).
● Infectious: Lyme disease.
features of intermediate uveitis
Patients often present with blurred vision and floaters (as vitreous is infected). There is no pain or red eye.
signs of intermediate uveitis
● Snowballs: White focal inflammatory cells and exudates. Most commonly in the inferior vitreous.
● Snowbanking: Whitish exudates in the ora serrata, typically inferiorly, that may extend into the pars plana. It can be viewed via an indirect ophthalmoscope with scleral depression.
● Peripheral periphlebitis (especially in MS).
complications of intermediate uveitis
● CMO ● Optic disc swelling ● Band keratopathy ● Glaucoma ● Cataracts: From steroid use
what is posterior uveitis
inflammation of the choroid +/- retina
chorioretinitis
causes of posterior uveitis
● Idiopathic.
● Inflammatory: Sympathetic ophthalmia, Vogt-Koyanagi-Harada (VKH)
syndrome, birdshot choroidopathy, sarcoidosis, Behçet syndrome.
● Infectious: Toxoplasmosis, onchocerciasis, toxocariasis, syphilis, TB, VZV,
herpes simplex virus (HSV), cytomegalovirus (CMV).
what is sarcoidosis
Inflammatory multisystem disorder characterized by noncaseating granulomas.
- Associated with HLA-DRB1
- Previousinfectionswith B.burgforferi, M.tuberucosis, P.acnes
histopathology of sarcoidosis
multinucleated giant cells of Langerhans type and Schaumann bodies
in who is sarcoidosis seen commonly and who is it worse in
common in caucasian
severe in african-carribean patients
extraocular features of sarcoidosis
● Bilateral hilar lymphadenopathy -> lung fibrosis
● Erythema nodosum
● Bilateral CNVII palsy
● Bilateral parotid enlargement
ocular features of sarcoidosis
anterior
intermediate
psoterior
Anterior, intermediate, posterior or pan-uveitis. Unilateral or bilateral.
● Anterior uveitis: Mutton fat KPs
- koeppe and Busacca iris nodules.
● Intermediate uveitis with snowbanking and snowballs.
● Posterior uveitis
CMO
Candle wax: Patchy periphlebitis (granulomas along venules)
occlusive vascular disease (BRVO or CRVO) - retinal ischaemia neovascularisation
Choroiditis Periretinal granuloma (Lander sign): Specific to sarcoidosis only and not seen in TB
Non-ocular features
• Lung=themostfrequentsite(90%ofcases)
• Bilateral hilar lymphadenopathy lung fibrosis
• Also skin, GI, liver, joints, CVS, CNS
Granuloma = giant epithelioid cells
• Produces activated vitamin D à hypercalcaemia and hyperuriaemia
(à renal stones)
• Produces angiotensinogen converting enzyme (ACE) à ↑[ACE]
• Reduced response to Mantoux test
DIagnosis of sarcoidosis
high serum ACE
Produces activated vitamin D -> hypercalcaemia and hyperuriaemia
(à renal stones)high calcium level
chest xray and spirometry
cause of TB
More common in
test for TB
Mycobacterium tuberculosis (acid-fast bacilli seen on Ziehl-Neelsen stain) causing caseating granulomas in the body. More common in patients from India. Multiple organs can be affected.
extraocular features of TB
● Night sweats
● Weight loss
● Fevers and rigors
● Haemoptysis
ocular features of TB
● Conjunctivitis, scleritis or keratitis.
● Anterior uveitis: Mutton fat KPs and iris nodules.
● Posterior uveitis
— Vitritis
— Retinal vasculitis with vascular occlusions can occur
— Choroiditis and choroidal granuloma
— CMO
● Lacrimal gland: Dacryoadenitis.
what is sympathetic ophthalmia and ass
bilateral T-cell mediated granulomatous pan-uveitis which days or years following ocular surgery or trauma. It is associated with HLA-DR4 and HLA-A11
features of sympathetic ophthalmia
● Pain: Due to anterior uveitis and/or optic neuritis.
● Granulomatous anterior uveitis.
● Vitritis.
● Choroidal infiltrates.
● Dalen-Fuchs’ nodules: Pigmented epithelioid cells between RPE and Bruch membrane.
• Multifocalchoroiditis
• Exudative retinal detachment
Mx of sympathetic ophthalmia
● Steroids.
● Enoculation: Prevents fellow eye from developing sympathetic ophthalmia –
best option for eyes that are blind.
what is vogt-koyanagi-harada syndrome and pathophysiology and associations
Bilateral granulomatous pan-uveitis associated with multisystem inflammation.
HLA-DR4 and HLA-B22.
Associated with HLA-DR4 and HLA-DRB1
May be due to T-cell response against melanocytes, melanin and the RPE.
This condition is distinct from sympathetic ophthalmia in that there is a lack of trauma and there are extraocular signs.
more common in japanese population
features of vogt-koyanagi-harada syndrome and systemic features
● Bilateral pan-uveitis.
● Dalen-Fuchs’ nodules.
● Depigmented limbus (Sugiura sign) and depigmented fundus (‘sunset glow’
appearance).
● Exudative retinal detachment.
● Systemic:
skin - hypopigmented lesions + Alopecia, vitiligo
auditory - sensorineural hearing loss and tinnitus
CNS - meningitis and N&V
Mx of vogt-koyanagi-harada syndrome
systemic steroids
recognised stages of vogt-koyanagi-harada syndrome
- Prodromal (3-5 days)
- Uveitic (several weeks)
- Convalescent/Chronic
- Recurrent
features of prodromal VKH
Viral-like illness
uveitic features of VKH
Posterior uveitis à anterior uveitis: - Dalen-Fuchs nodules - Thickening of choroid - Optic disc oedema - Well-demarcated macular oedema à exudative retinal detachment
Convalescent features of VKH
Vitiligo and poliosis Depigmentation of retina: - Resolved Dalen-Fuchs nodules
- ’Sunset fundus’ Resolution of RD
recurrent features of VKH
Recurrent episodes of anterior uveitis
what is birdshot choroidopathy + ass + predominantly affects
Chronic and bilateral posterior uveitis with characteristic hypopigmented fundus lesions.
Strong association with HLA-A29.
Predominantly affects middle-aged females.
features of birdshot choroidopathy including signs
Insidious impairment of central vision with flashes, floaters and nyctalopia.
‘Birdshot’ lesions:
• Cream oval shaped lesions at the level of choroid and RPE
• Optic disc à periphery along underlying choroidal vessels
• Other features:
• Retinal vasculitis
• Cystoid macular oedema
• Optic disc inflammation
Signs include:
● Moderate vitritis (no snowbanking/snowballs).
● Bilateral oval cream-coloured ill-defined lesions in posterior pole and
mid-periphery.
Ix of birshot choroidopathy
● Fluorescence Angiography: Early hypofluorescence with late hyperfluorescence.
● ERG: Prolonged 30 Hz flicker times.
what is fuchs’ heterochromic cyclitis
Nongranulomatous unilateral disorder causing a chronic anterior uveitis, posterior subcapsular cataract and a chronic open-angle glaucoma.
features of fuchs’ heterochromic cyclitis
● Chronic anterior uveitis: White eye, stellate KPs, anterior chamber flare.
● Iris atrophy and heterochromia (affected iris is hypochromic).
● Posterior subcapsular cataract: dVa and glare.
● Glaucoma.
what is tubulointerstitial nephritis and uveitis
Bilateral granulomatous anterior uveitis that occurs in patients with tubulointerstitial nephritis (TIN).
Commonly presents in female teenagers.
associated with flubriprofen
features of tubulointerstitial nephritis and uveitis
renal disease BEFORE ocular disease
fever malaise flank pain proteinuria anaemia
AV - painful red eye
photophobia
miosed pupil with AC flare and stellate KPs
in urine increased beta-2 microglobinuria in the urine is seen
what is juvenile idiopathic arthritis
The most common rheumatological disease in children
most common cause of anterior uveitis in children.
The aetiology is unknown; however, there is an association with HLA-DR5.
This condition is more common in females.
features of juvenile idiopathic arthritis
● Arthritis: Most common type that is seen is oligoarthritis (≤4 joints affected). Must be present for 6 weeks.
● Ophthalmic: Chronic and nongranulomatous anterior uveitis, cataract and band keratopathy.
● Spiking fevers (lasting 2 weeks) and rashes are also commonly seen.
Mx of juvenile idiopathic arthritis
● ANA is usually positive. Steroids are the mainstay treatment.
what is reiter syndrome
Infectious cause of arthritis and conjunctivitis with an immunological component (HLA-B27).
aetiology of retier syndrome
Non-gonococcal infections:
● GU: Chlamydia
● GI: Shigella, Salmonella, Yersinia
features of reiter syndrome
● Triad: Conjunctivitis, urethritis, arthritis
● Less commonly: Iritis, keratitis, prostatitis, keratoderma blennorrhagica
behcet disease what is it
Rare multisystem vasculitis that occurs more commonly in those of Turkish descent. Associated with HLA-B51.
More common along the Old Silk Road (Mediterranean à East Asia)
features of behcet disease
● Recurrent oral and genital ulcers
● Erythema nodosum
● Positive pathergy skin test: Skin lesions in response to minor trauma
● Pulmonary artery aneurysm (pathogenomic)
Mx of behcet disease
● Steroid therapy and anti-TNF agents
ocular features of behcet disease
● Anterior uveitis with pathogenomic mobile hypopyon
• Non-granulomatous necrotising obliterative vasculitis à panuveitis
• Anterior segment:
- Mobile hypopyon seen in 25%
- Complications are common: posterior synechiae, closed angle glaucoma
Posterior segment:
• Retinal vasculitis à retinal ischaemia à retinal and iris neovascularisation
• Vasculitis of larger vessels à BRVO and BRAO
• Vitritis and CMO
• Optic neuritis à optic atrophy
what is kawasaki disease
medium vessel vasculitis
features of kawasaki disease
Suspect in any patient (usually children) with a fever lasting longer than 5 days that is not responsive to typical antipyretics such as paracetamol.
● Eyes - Bilateral conjunctival injection sparing the limbus - Bilateral anterior uveitis ● Mouth: Strawberry tongue ● Skin: Shedding rash of the extremities
Mx of kawasaki disease
aspirin only agent to reduce the fever
IVIG
complications of kawasaki disease
● Coronary artery aneurysm: All patients require an echocardiogram to exclude this complication.
what is onchocerciasis
Caused by Onchocerca volvulus
transmitted - Simulium black fly being the vector. Endemic in Africa and South America. It is the second leading cause of infectious blindness in the world behind trachoma.
Mx of onchocerciasis
annual oral ivermectin
features of onchocerciasis
A maculopapular rash is often the first sign seen and precedes ocular features, which include:
● dVA.
● Sclerosing keratitis: Subepithelial punctate lesions (snowflake opacities).
● Anterior uveitis: Painful red eye with photophobia. Pearly shaped pupil (due
to posterior synechiae; attachment of the iris to the lens, may predispose to
glaucoma and increase IOP).
● Bilateral chorioretinitis with RPE atrophy.
● Optic neuritis and atrophy.
● Visualization of the nematode on slit lamp retroillumination.
what is toxocariasis
Commonly caused by Toxocara cani, with dogs being the definitive hosts.
features of toxocariasis
Unilateral endophthalmitis in children aged 2–6:
● Progressive vitritis, grey-white exudates.
● Leukocoria, strabismus, visual loss.
● Anterior uveitis: Pain photophobia, etc.
● Pars planitis: Snowballs.
Ix for toxocariasis
● Absence of calcification on CT scan.
● Differentiates from retinoblastoma, another cause of leukocoria in that age
group.
Mx of toxocariasis
● Corticosteroids play an important role.
● Surgery may be indicated.
● Limited role for antihelmintics such as thiabendazole.
AIDS related conditions
Toxoplasmosis
presumed ocular histoplasmosis syndrome (POHS)
CMV retinitis
progressive outer retinal necrosis (PORN)
what is toxoplasmosis
Caused by the parasite Toxoplasma gondii, from cats. This condition occurs in patients with AIDS, specifically with CD4+ counts <200.
features of congenital toxoplasmosis
Triad: Hydrocephalus, intraretinal calcification and chorioretinitis. Sabin
Hydrocephalus
• Intracranialcalcification • Chorioretinitis (late
complication)
Ix for congenital toxoplasmosis
Fieldman test positive.
Mx for congenital toxoplasmosis
Pyrimethamine (folic acid antagonist).
features of acquired toxoplasmosis
White lesion of retinitis with an associated pigmented scar
Focal retinitis with overlying vitritis (‘headlight in the fog’).
Perivasculitis:
• Diffusevenoussheathing
• Segmented arterial sheathing (Kyrielieis arteriolitis)
‘Spillover’ anterior uveitis: Due to vitreous spilling in anterior chamber.
Mx of toxoplasmosis
Pyrimethamine + sulfadiazine + corticosteroid.
usually 6 weeks
Alternatives: azithromycin; co-trimoxazole OR clindamycin/sulfadiazine PLUS prednisolone
indications for ABx
- Lesions threatening fovea or optic disc
- Moderate vitritis or vasculitis
- ↓VA
- Large singular or multiple active lesions
- Progressive or prolonged course
- Immunocompromised patients (e.g. HIV +ve)
what is presumed ocular histoplasmosis syndrome
ass w
This clinical presentation occurs due to Histoplasma capsulatum (dimorphic fungi). Associated with HLA-B7 and HLA-DR2. Famously occurs in Ohio, Mississippi, and St Lawrence River valleys. More common in patients with AIDS.
features of presumed ocualr histoplasmosis syndrome
● Patient usually presents with loss of vision.
● Examination findings (triad)
Multiple white atrophic punched-out chorioretinal scars Peripapillary atrophy
Absent vitritis
complications of presumed ocular histoplasmosis syndrome
● Choroidal neovascularization – late manifestation.
● Exudative macular detachment.
what is cytomegalovirus retinitis
A weak opportunistic infection that only occurs in AIDS patients when the CD4+ count is less than 50. This infection can present in the eye and cause full-thickness retinal inflammation with necrosis, retinal tears and detachments.
Mx of cytomegalovirus retinitis
IV ganciclovir
features of cytomegalovirus retinitis
● Rapid visual loss.
● Mainly posterior pole affected.
● Pizza pie/cottage cheese-with-ketchup appearance indicating prominent
haemorrhages (Figure 13.2).
● Periphlebitis, frosted branch angiitis (vein and artery involvement).
● Mild-moderate vitritis.
what is progressive outer retinal necrosis
This is a devastating necrotizing retinitis caused by VZV in AIDS patients with a CD4+ count less than 50
Mx of progressive outer retinal necrosis
IV ganciclovir
features of progressive outer retinal necrosis
● Very rapid visual loss.
● Multifocal yellow/white retinal infiltrates.
● Rapidly conjugating white areas of peripheral retinal necrosis.
● Minimal vasculitis, absent vitritis.
what is acute retinal necrosis adn Mx
Devastating pan-uveitis not associated with AIDS. Treatment is with IV acyclovir.
aetiology of acute retinal necrosis
● VZV: Older patients
● HSV: Children
features of acute retinal necrosis
● Rapid loss of visual acuity; floaters, discomfort.
● Predominantly a peripheral disease of occlusive arteritis.
● Full-thickness peripheral retinal necrotising retinitis.
● Marked vitritis.
what is tuberculous uveitis
myscobacterium TB
- acid fast bacilli on ziehl-neelsen stain
ocular features of TB uveitis
conjunctivitis scleritis keratitis anterior uveitis: mutton fat KPs psoterior uveitis dacryoadenitis
what is cat scratch neuroretinitis
inflammation of optic nerve and neural retina causin oedema from the nerve head to the macula
pathogen
systemic features
ocular features
fundoscopy sign
Mx
of cat scratch neuroretinitsi
bartonella henselae gram -ve
fever and lymphadenopathy
unilateral
painless central decrease in VA
macular star sign
may need ABx and steroids
what is CMV retinitis
opportunistic infetion caused by CMV
- occurs in AIDS pts when CD4+ count <50
full thickness retinal infection that can lead to necrosis adn retinal breaks and detachment
Sx
Ix
Mx
for CMV retinitis
can be asymptomatic rapid visual loss floaters flashing lights blind spots
often clinical
aqueous or vitreous tap
pizza pie/ cottage cheese with ketchup appearnace
IV ganciclovir
Sx of toxoplasmosis
floaters
blurred vision
can developp anterior uveitis
secondary to vitreous inflammtion
diagnosed w PCR
white focal retinitis w overlying vitreous inflammation ‘headlight in the fog’ w accompanying pigmented scar
Mx
primethamine
sulfadiazine
steroid
what is the HLA number
birdshot Behcet's HLA Anterior uveitis Sympathetic opthalmia panuveitis intermediate uveitis chronic iridocyclitis (IJA)
birdshot HLA-A29 Behcet's HLA B51 Anterior uveitis HLA B27 Sympathetic opthalmia HLA DR4 panuveitis HLA DR4 intermediate uveitis HLA DR3 chronic iridocyclitis (IJA) HLA DR5
what is MS
epidemiology adn RFs
Autoimmune reaction to MOG and MBP à discrete demyelination CNS plaques separated in space and time
- Youngfemale
- Averageageofonset:28-31 • Female: male ratio = 3:1
- EarlylifeexposuretoEBV
- Temperate climates
- Vitamin D and sunlight are protective
poor prognostic factors of MS
- Older males
- Motorsignsofonset
- Many relapses early on
- Many MRI lesions
- Axonal loss
ocular manifestations of MS
- Optic neuritis
- Uhthoff’sphenomenon
- Intermediate uveitis
infectious ass of intermediate uvieitis
non infectious
Infectious Non-infectious - TB - Leprosy - Lyme disease - Syphilis - Whipple’s disease - Toxocariasis
Sarcoidosis
- Sjögren’s disease - IBD
- MS
- Lymphoma
- TINU
pathogens involved in reactive arthritis
Symptoms triggered by a recent infection:
• GU: Chlamydia trachimatis, Ureaplasma,
Mycoplasma genitalium, BCG
• GI: E.coli, Salmonella, Shigella, Yersenia,
Campylobacter, C. difficile
clinical features of reactive arthritis
- Classic triad: uveitis, urethritis and arthritis à ‘can’t see, can’t see, can’t climb a tree’
- Arthritis: asymmetrical oligo or polyarthritis (wrists, elbows, knees and ankles)
- Ocularfeatures:
- Conjunctivitis = common
- Acute non-granulomatous AU = 10-20%
- Cutaneousmanifestations:
- Keratoderma blennorrhagicum
- Circinate balanitis
