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uveitis Flashcards

1
Q

waht is the uvea

A

iris, ciliary body and choroid.

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2
Q

what are the two structures of the iris

A

stroma and posterior pigmented layer

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3
Q

what are the zones of the stroma of the iris

A

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.

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4
Q

what is the collarette

A

thickest part of the iris that separates pupillary and ciliary zone

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5
Q

role of sphincter pupilae made of what and supplied by

A

smooth muscle responsible for pupillary constriction with parasympathetic innervation (CNIII) via short ciliary nerves.

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6
Q

origin of ciliary zone and role

A

extends from the collarette to the origin of the iris at the ciliary body

with dilator pupillar

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7
Q

dilator pupilae made of and innervation

A

smooth muscle sympathetic innervation via long ciliar nerves

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8
Q

what is the posterior pigmented layer

A

A pigmented epithelial layer found at the posterior iris surface that is continuous with the neurosensory retina posteriorly.

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9
Q

what are the layers of the ciliary bpdy

A

stroma - CT where the vascular supply is found

muscle - PNS via CNIII

epithelium

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10
Q

blood supply to the ciliary body

A

anterior and long posterior ciliary arteries

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11
Q

parts of the ciliary body and its role

A

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

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12
Q

what is the choroid and location

A

posterior component of the uveal tract

between the sclera externally and RPE

consists of bruchs membrane and a vascular layer

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13
Q

role of the choroid

A

provide nutrients to the outer third of the retina

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14
Q

what is the bruchs membrane and its function

A

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
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15
Q

what is the vascular layer of the choroid

A

has three layers

  • choriocapillaries which are external to the Bruch membrane
  • a medium-sized vessel layer
  • a large vessel layer.
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16
Q

blood supply of the choroid

A

two long posterior ciliary arteries, the short posterior ciliary artery and anterior ciliary artery.

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17
Q

what is anterior uveitis

A

inflammation of the iris +/- pars plicata (anterior ciliary body)

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18
Q

what are keratic precipitates

A

WBCs on the posterior endothelial layer of the cornea

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19
Q

granulomatous v non-granulomatous precipitataes how do u differentiate in anterior uveitis

A

Granulomatous is defined by mutton fat KPs whereas nongranulomatous is defined with stellate KPs.

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20
Q

aetiology of anterior uveitis

A

● 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).

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21
Q

Features of anterior uveitis

A

Painful red eye
blurred vision
photophobia

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22
Q

examination of anterior uveitis

complications

A

● 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.

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23
Q

Mx of anterior uveitis

A

● 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.

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24
Q

what is intermediate uveitis

A

Inflammation of the posterior ciliary body (pars plana) and vitreous. Synonym: pars planitis.

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25
Q

aetiology of intermediate uveitis

A

● Idiopathic.
● Inflammatory: Sarcoidosis, MS, inflammatory bowel disease (ulcerative
colitis and Crohn disease).
● Infectious: Lyme disease.

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26
Q

features of intermediate uveitis

A

Patients often present with blurred vision and floaters (as vitreous is infected). There is no pain or red eye.

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27
Q

signs of intermediate uveitis

A

● 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).

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28
Q

complications of intermediate uveitis

A 
● CMO
● Optic disc swelling
● Band keratopathy
● Glaucoma
● Cataracts: From steroid use
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29
Q

what is posterior uveitis

A

inflammation of the choroid +/- retina

chorioretinitis

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30
Q

causes of posterior uveitis

A

● 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).

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31
Q

what is sarcoidosis

A

Inflammatory multisystem disorder characterized by noncaseating granulomas.

  • Associated with HLA-DRB1
  • Previousinfectionswith B.burgforferi, M.tuberucosis, P.acnes
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32
Q

histopathology of sarcoidosis

A

multinucleated giant cells of Langerhans type and Schaumann bodies

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33
Q

in who is sarcoidosis seen commonly and who is it worse in

A

common in caucasian

severe in african-carribean patients

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34
Q

extraocular features of sarcoidosis

A

● Bilateral hilar lymphadenopathy -> lung fibrosis
● Erythema nodosum
● Bilateral CNVII palsy
● Bilateral parotid enlargement

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35
Q

ocular features of sarcoidosis
anterior
intermediate
psoterior

A

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

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36
Q

DIagnosis of sarcoidosis

A

high serum ACE
Produces activated vitamin D -> hypercalcaemia and hyperuriaemia
(à renal stones)high calcium level

chest xray and spirometry

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37
Q

cause of TB
More common in
test for TB

A

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.

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38
Q

extraocular features of TB

A

● Night sweats
● Weight loss
● Fevers and rigors
● Haemoptysis

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39
Q

ocular features of TB

A

● 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.

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40
Q

what is sympathetic ophthalmia and ass

A

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

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41
Q

features of sympathetic ophthalmia

A

● 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

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42
Q

Mx of sympathetic ophthalmia

A

● Steroids.
● Enoculation: Prevents fellow eye from developing sympathetic ophthalmia –
best option for eyes that are blind.

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43
Q

what is vogt-koyanagi-harada syndrome and pathophysiology and associations

A

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

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44
Q

features of vogt-koyanagi-harada syndrome and systemic features

A

● 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

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45
Q

Mx of vogt-koyanagi-harada syndrome

A

systemic steroids

46
Q

recognised stages of vogt-koyanagi-harada syndrome

A
  • Prodromal (3-5 days)
  • Uveitic (several weeks)
  • Convalescent/Chronic
  • Recurrent
47
Q

features of prodromal VKH

A

Viral-like illness

48
Q

uveitic features of VKH

A 
Posterior uveitis à anterior uveitis:
- Dalen-Fuchs nodules
- Thickening of choroid
- Optic disc oedema
- Well-demarcated macular
oedema à exudative retinal detachment
49
Q

Convalescent features of VKH

A

Vitiligo and poliosis Depigmentation of retina: - Resolved Dalen-Fuchs nodules
- ’Sunset fundus’ Resolution of RD

50
Q

recurrent features of VKH

A

Recurrent episodes of anterior uveitis

51
Q

what is birdshot choroidopathy + ass + predominantly affects

A

Chronic and bilateral posterior uveitis with characteristic hypopigmented fundus lesions.

Strong association with HLA-A29.

Predominantly affects middle-aged females.

52
Q

features of birdshot choroidopathy including signs

A

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.

53
Q

Ix of birshot choroidopathy

A

● Fluorescence Angiography: Early hypofluorescence with late hyperfluorescence.
● ERG: Prolonged 30 Hz flicker times.

54
Q

what is fuchs’ heterochromic cyclitis

A

Nongranulomatous unilateral disorder causing a chronic anterior uveitis, posterior subcapsular cataract and a chronic open-angle glaucoma.

55
Q

features of fuchs’ heterochromic cyclitis

A

● 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.

56
Q

what is tubulointerstitial nephritis and uveitis

A

Bilateral granulomatous anterior uveitis that occurs in patients with tubulointerstitial nephritis (TIN).
Commonly presents in female teenagers.

associated with flubriprofen

57
Q

features of tubulointerstitial nephritis and uveitis

A

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

58
Q

what is juvenile idiopathic arthritis

A

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.

59
Q

features of juvenile idiopathic arthritis

A

● 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.

60
Q

Mx of juvenile idiopathic arthritis

A

● ANA is usually positive. Steroids are the mainstay treatment.

61
Q

what is reiter syndrome

A

Infectious cause of arthritis and conjunctivitis with an immunological component (HLA-B27).

62
Q

aetiology of retier syndrome

A

Non-gonococcal infections:
● GU: Chlamydia
● GI: Shigella, Salmonella, Yersinia

63
Q

features of reiter syndrome

A

● Triad: Conjunctivitis, urethritis, arthritis

● Less commonly: Iritis, keratitis, prostatitis, keratoderma blennorrhagica

64
Q

behcet disease what is it

A

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)

65
Q

features of behcet disease

A

● Recurrent oral and genital ulcers
● Erythema nodosum

● Positive pathergy skin test: Skin lesions in response to minor trauma
● Pulmonary artery aneurysm (pathogenomic)

66
Q

Mx of behcet disease

A

● Steroid therapy and anti-TNF agents

67
Q

ocular features of behcet disease

A

● 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

68
Q

what is kawasaki disease

A

medium vessel vasculitis

69
Q

features of kawasaki disease

A

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
70
Q

Mx of kawasaki disease

A

aspirin only agent to reduce the fever

IVIG

71
Q

complications of kawasaki disease

A

● Coronary artery aneurysm: All patients require an echocardiogram to exclude this complication.

72
Q

what is onchocerciasis

A

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.

73
Q

Mx of onchocerciasis

A

annual oral ivermectin

74
Q

features of onchocerciasis

A

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.

75
Q

what is toxocariasis

A

Commonly caused by Toxocara cani, with dogs being the definitive hosts.

76
Q

features of toxocariasis

A

Unilateral endophthalmitis in children aged 2–6:
● Progressive vitritis, grey-white exudates.
● Leukocoria, strabismus, visual loss.

● Anterior uveitis: Pain photophobia, etc.
● Pars planitis: Snowballs.

77
Q

Ix for toxocariasis

A

● Absence of calcification on CT scan.
● Differentiates from retinoblastoma, another cause of leukocoria in that age
group.

78
Q

Mx of toxocariasis

A

● Corticosteroids play an important role.
● Surgery may be indicated.
● Limited role for antihelmintics such as thiabendazole.

79
Q

AIDS related conditions

A

Toxoplasmosis
presumed ocular histoplasmosis syndrome (POHS)
CMV retinitis
progressive outer retinal necrosis (PORN)

80
Q

what is toxoplasmosis

A

Caused by the parasite Toxoplasma gondii, from cats. This condition occurs in patients with AIDS, specifically with CD4+ counts <200.

81
Q

features of congenital toxoplasmosis

A

Triad: Hydrocephalus, intraretinal calcification and chorioretinitis. Sabin

Hydrocephalus
• Intracranialcalcification • Chorioretinitis (late
complication)

82
Q

Ix for congenital toxoplasmosis

A

Fieldman test positive.

83
Q

Mx for congenital toxoplasmosis

A

Pyrimethamine (folic acid antagonist).

84
Q

features of acquired toxoplasmosis

A

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.

85
Q

Mx of toxoplasmosis

A

Pyrimethamine + sulfadiazine + corticosteroid.

usually 6 weeks

Alternatives: azithromycin; co-trimoxazole OR clindamycin/sulfadiazine PLUS prednisolone

86
Q

indications for ABx

A
  • 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)
87
Q

what is presumed ocular histoplasmosis syndrome

ass w

A

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.

88
Q

features of presumed ocualr histoplasmosis syndrome

A

● Patient usually presents with loss of vision.
● Examination findings (triad)
Multiple white atrophic punched-out chorioretinal scars Peripapillary atrophy
Absent vitritis

89
Q

complications of presumed ocular histoplasmosis syndrome

A

● Choroidal neovascularization – late manifestation.

● Exudative macular detachment.

90
Q

what is cytomegalovirus retinitis

A

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.

91
Q

Mx of cytomegalovirus retinitis

A

IV ganciclovir

92
Q

features of cytomegalovirus retinitis

A

● 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.

93
Q

what is progressive outer retinal necrosis

A

This is a devastating necrotizing retinitis caused by VZV in AIDS patients with a CD4+ count less than 50

94
Q

Mx of progressive outer retinal necrosis

A

IV ganciclovir

95
Q

features of progressive outer retinal necrosis

A

● Very rapid visual loss.
● Multifocal yellow/white retinal infiltrates.
● Rapidly conjugating white areas of peripheral retinal necrosis.
● Minimal vasculitis, absent vitritis.

96
Q

what is acute retinal necrosis adn Mx

A

Devastating pan-uveitis not associated with AIDS. Treatment is with IV acyclovir.

97
Q

aetiology of acute retinal necrosis

A

● VZV: Older patients

● HSV: Children

98
Q

features of acute retinal necrosis

A

● Rapid loss of visual acuity; floaters, discomfort.
● Predominantly a peripheral disease of occlusive arteritis.
● Full-thickness peripheral retinal necrotising retinitis.
● Marked vitritis.

99
Q

what is tuberculous uveitis

A

myscobacterium TB

- acid fast bacilli on ziehl-neelsen stain

100
Q

ocular features of TB uveitis

A 
conjunctivitis
scleritis
keratitis
anterior uveitis: mutton fat KPs
psoterior uveitis
dacryoadenitis
101
Q

what is cat scratch neuroretinitis

A

inflammation of optic nerve and neural retina causin oedema from the nerve head to the macula

102
Q

pathogen

systemic features

ocular features

fundoscopy sign

Mx

of cat scratch neuroretinitsi

A

bartonella henselae gram -ve

fever and lymphadenopathy

unilateral
painless central decrease in VA

macular star sign

may need ABx and steroids

103
Q

what is CMV retinitis

A

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

104
Q

Sx

Ix

Mx
for CMV retinitis

A 
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

105
Q

Sx of toxoplasmosis

A

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

106
Q

what is the HLA number

birdshot 
Behcet's HLA
Anterior uveitis 
Sympathetic opthalmia
panuveitis
intermediate uveitis
chronic iridocyclitis (IJA)
A 
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
107
Q

what is MS

epidemiology adn RFs

A

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
108
Q

poor prognostic factors of MS

A
  • Older males
  • Motorsignsofonset
  • Many relapses early on
  • Many MRI lesions
  • Axonal loss
109
Q

ocular manifestations of MS

A
  • Optic neuritis
  • Uhthoff’sphenomenon
  • Intermediate uveitis
110
Q

infectious ass of intermediate uvieitis

non infectious

A 
Infectious
Non-infectious
- TB
- Leprosy
- Lyme disease
- Syphilis
- Whipple’s disease - Toxocariasis

Sarcoidosis

  • Sjögren’s disease - IBD
  • MS
  • Lymphoma
  • TINU
111
Q

pathogens involved in reactive arthritis

A

Symptoms triggered by a recent infection:
• GU: Chlamydia trachimatis, Ureaplasma,
Mycoplasma genitalium, BCG
• GI: E.coli, Salmonella, Shigella, Yersenia,
Campylobacter, C. difficile

112
Q

clinical features of reactive arthritis

A
  • 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

DEP (20 decks)

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