This document discusses common systemic diseases that can affect the eye, including infectious diseases like toxoplasmosis and tuberculosis, and non-infectious diseases like diabetes, connective tissue diseases, and vasculitides. It provides details on diabetic retinopathy, including risk factors, stages of progression from background retinopathy to proliferative retinopathy, and treatment with laser panretinal photocoagulation. It also summarizes ocular features and systemic signs of sarcoidosis, phakomatoses including neurofibromatosis, tuberous sclerosis, von Hippel-Lindau syndrome, and Sturge-Weber syndrome. Giant cell arteritis is also briefly discussed.

1. Mohammad Sazzad Hossen
B.Optom, M.Optom
Consultant Optometrist
Ad-din Medical College Hospital
Visiting Faculty: UniSA, Dhaka
Systemic disease and the eye

2. Common systemic diseasesCommon systemic diseases
affecting the eyeaffecting the eye
 InfectiousInfectious
 ToxoplasmosisToxoplasmosis
 ToxocariasisToxocariasis
 TBTB
 SyphilisSyphilis
 LeprosyLeprosy
 HIVHIV
 CMVCMV
 Non-infectiousNon-infectious
 Endocrine – diabetes,Endocrine – diabetes,
thyroidthyroid
 Connective tissueConnective tissue
disease –disease –
RA/SLE/Wegeners/PARA/SLE/Wegeners/PA
N/Systemic sclerosisN/Systemic sclerosis
 Vasculitides (GCA)Vasculitides (GCA)
 SarcoidosisSarcoidosis
 Behcet’s DiseaseBehcet’s Disease
 Vogt Koyanagi HaradaVogt Koyanagi Harada
syndromesyndrome
 PhakomatosesPhakomatoses

3. DIABETIC RETINOPATHY
1. Adverse risk factors
2. Pathogenesis
5. Clinically significant macular oedema
6. Preproliferative diabetic retinopathy
3. Background diabetic retinopathy
4. Diabetic maculopathies
• Focal
• Diffuse
• Ischaemic
7. Proliferative diabetic retinopathy

4. Adverse Risk Factors
1. Long duration of diabetes
• Obesity
• Hyperlipidaemia
2. Poor metabolic control
3. Pregnancy
4. Hypertension
5. Renal disease
6. Other
• Smoking
• Anaemia

5. Location of lesions in background
diabetic retinopathy

6. Signs of background diabetic retinopathy
Microaneurysms usually
temporal to fovea
Intraretinal dot and
blot haemorrhages
Hard exudates
frequently
arranged in clumps or
rings
Retinal oedema seen as
thickening on biomicroscopy

7. Preproliferative diabetic retinopathy
Treatment - not required but watch for proliferative disease
• Cotton-wool spots
• Venous irregularities
• Dark blot haemorrhages
• Intraretinal microvascular
abnormalities (IRMA)
Signs

8. Proliferative diabetic retinopathy
• Flat or elevated
• Severity determined by comparing with area of disc
Neovascularization
Neovascularization of disc = NVD
• Affects 5-10% of diabetics
• IDD at increased risk (60% after 30 years)
Neovascularization elsewhere = NVE

9. • Spot size (200-500 µm) depends
on contact lens magnification
• Gentle intensity burn (0.10-0.05 sec)
• Follow-up 4 to 8 weeks
• Area covered by complete PRP• Initial treatment is 2000-3000 burns
Laser panretinal photocoagulation

10. Retinal Vein OcclusionRetinal Vein Occlusion
Second most common cause of vascular-related visual loss.
Risk factors: hypertension, age, blood dyscrasias (OCP,HRT) and
vasculitis (Behcets,sarcoidosis,AIDS,SLE)

11. Retinal Artery OcclusionRetinal Artery Occlusion
Risk factors: Carotid artery atherosclerosis (CRAO), carotid emboli
(BRAO), vasculitis (GCA,SLE,PAN), coagulopathy.
OCULAR EMERGENCY - Immediate referral to ophthalmologist

12. 1. Soft tissue involvement
• Periorbital and lid swelling
• Conjunctival hyperaemia
• Chemosis
• Superior limbic keratoconjunctivitis
2. Eyelid retraction
3. Proptosis
4. Optic neuropathy
5. Restrictive myopathy
THYROID EYE DISEASE

13. Soft tissue involvement
Periorbital and lid swelling
Chemosis
Conjunctival hyperaemia
Superior limbic
keratoconjunctivitis

14. Signs of eyelid retraction
Occurs in about 50%
• Bilateral lid retraction
• No associated proptosis
• Bilateral lid retraction
• Bilateral proptosis
• Lid lag in downgaze• Unilateral lid retraction
• Unilateral proptosis

15. Proptosis
Treatment options
• Systemic steroids
• Radiotherapy
• Surgical decompression
• Occurs in about 50%
• Uninfluenced by treatment of hyperthyroidism
Axial and permanent in about 70% May be associated with choroidal folds

16. Optic neuropathy
• Occurs in about 5%
• Early defective colour vision
• Usually normal disc appearance
Caused by optic nerve
compression at
orbital apex by enlarged recti
Often occurs in absence of significant
proptosis

17. • Occurs in about 40%
• Due to fibrotic contracture
Restrictive myopathy
Elevation defect - most common Abduction defect - less common
Depression defect - uncommon Adduction defect - rare

18. SARCOIDOSISSARCOIDOSIS
 Idiopathic multisystem disorderIdiopathic multisystem disorder
 Characterised by non-caseatingCharacterised by non-caseating
granulomatagranulomata
 More common in women 20-50 yrsMore common in women 20-50 yrs
 More common in blacks and AsiansMore common in blacks and Asians
 ? Related to mycobacteria? Related to mycobacteria

19. SARCOIDOSISSARCOIDOSIS
Systemic InvolvementSystemic Involvement
 Lung lesions –Lung lesions –
95%95%
 Thoracic lymphThoracic lymph
nodes – 50%nodes – 50%
 Skin lesions – 30%Skin lesions – 30%
→→
 Eyes – 30%Eyes – 30%

20. SARCOIDOSISSARCOIDOSIS
Ocular InvolvementOcular Involvement
 Anterior segmentAnterior segment
lesions (30%)lesions (30%)
– ConjunctivalConjunctival
granulomagranuloma
– Lacrimal glandLacrimal gland
involvement/dry eyeinvolvement/dry eye
– Acute or chronicAcute or chronic
uveitisuveitis →→
– KPs described asKPs described as
‘mutton fat’ because‘mutton fat’ because
they are large andthey are large and
greasygreasy

21. SARCOIDOSISSARCOIDOSIS
Ocular InvolvementOcular Involvement
 Posterior segmentPosterior segment
lesions (20%)lesions (20%)
– Patchy venousPatchy venous
sheathingsheathing
– Cellular infiltrateCellular infiltrate
around vesselsaround vessels
– ChorioretinalChorioretinal
granulonmasgranulonmas
– Vasculitis includingVasculitis including
occlusive causing:-occlusive causing:-
– NeovascularisationNeovascularisation
– Infiltrate in vitreousInfiltrate in vitreous
(vitritis) including(vitritis) including
cell clumpscell clumps
(snowballs)(snowballs)

22. SARCOIDOSISSARCOIDOSIS
Ocular InvolvementOcular Involvement
 Sheathing of theSheathing of the
retinal veinsretinal veins
 FluoresceinFluorescein
angiographyangiography
showing leakageshowing leakage
and staining atand staining at
sites of sheathingsites of sheathing

23. SARCOIDOSISSARCOIDOSIS
Granuloma in FundusGranuloma in Fundus
 Retinal and pre-Retinal and pre-
retinalretinal
 ChoroidalChoroidal

24. SARCOIDOSISSARCOIDOSIS
Granuloma in FundusGranuloma in Fundus
 Optic nerve headOptic nerve head
granulomagranuloma
 Normal optic nerveNormal optic nerve
headhead

25. SARCOIDOSISSARCOIDOSIS
Systemic SignsSystemic Signs
Lupus pernioLupus pernio
affecting the noseaffecting the nose
– a chronic– a chronic
progressiveprogressive
cutaneous sarcoidcutaneous sarcoid
that mostthat most
commonly affectscommonly affects
face and earsface and ears

26. SARCOIDOSISSARCOIDOSIS
Systemic signsSystemic signs
 Facial palsyFacial palsy
 Salivary glandSalivary gland
enlargementenlargement

27. SARCOIDOSISSARCOIDOSIS
Systemic signsSystemic signs
 Hilar adenopathyHilar adenopathy
on chest x-rayon chest x-ray
 Lung infiltrateLung infiltrate
 Erythema nodosumErythema nodosum
 ArthritisArthritis

28. SARCOIDOSISSARCOIDOSIS
Investigations (1)Investigations (1)
CXR – to detectCXR – to detect
pulmonary signspulmonary signs
 Bilateral hilarBilateral hilar
lymph-adenopathylymph-adenopathy
 Pulmonary mottlingPulmonary mottling

29. SARCOIDOSISSARCOIDOSIS
Investigations (2)Investigations (2)
 Serum angiotensin-convertingSerum angiotensin-converting
enzyme (ACE) – elevated in activeenzyme (ACE) – elevated in active
sarcoidosissarcoidosis
 Mantoux test – caution in patientsMantoux test – caution in patients
who have had BCG vaccination. Testwho have had BCG vaccination. Test
may be negativemay be negative
 Lung function testsLung function tests

30. SARCOIDOSISSARCOIDOSIS
Investigations (3)Investigations (3)
Gallium scanGallium scan
showing increasedshowing increased
uptake in theuptake in the
lacrimal andlacrimal and
parotid glands andparotid glands and
pulmonary regionspulmonary regions
in a patient within a patient with
active sarcoidosisactive sarcoidosis

31. SARCOIDOSISSARCOIDOSIS
TreatmentTreatment
Systemic steroids may be necessarySystemic steroids may be necessary
in patients with posterior segmentin patients with posterior segment
disease where vision is threatened,disease where vision is threatened,
especially if optic nerve is involvedespecially if optic nerve is involved

32. PHACOMATOSES
1. Neurofibromatosis
2. Tuberous sclerosis (Bourneville disease)
3. von-Hippel-Lindau syndrome
4. Sturge-Weber syndrome
• Type I (NF-1) - von Recklinghausen disease
• Type II (NF-2) - bilateral acoustic neuromas

33. Neurofibromatosis type-1 - (NF-1)
Appear during first year of life
Café-au-lait spots
• Most common phacomatosis
Increase in size and number throughout
childhood
• Affects 1:4000 individuals
• Presents in childhood
• Gene localized to chromosome 17q11

34. Fibroma molluscum in NF-1
• Appear at puberty
• Pedunculated, flabby nodules consisting of
neurofibromas or schwannomas
• Increase in number
throughout life
• Frequently widely distributed

35. Plexiform neurofibroma in NF-1
• May be associated with
overgrowth of overlying skin
• Appear during childhood
• Large and ill-defined

36. Skeletal defects in NF-1
• Mild head enlargement - uncommon
• Other - scoliosis, short stature, thinning of
long bones
• Facial hemiatrophy

37. Orbital lesions in NF-1
Spheno-orbital encephaloceleOptic nerve glioma in about 15%
• Sagittal MRI scan of optic nerve glioma
invading hypothalamus
• Glioma may be unilateral or bilateral
• Axial CT scan of congenital absence of
left greater wing of sphenoid bone
• Causes pulsating proptosis without bruit

38. Eyelid neurofibromas in NF-1
Nodular Plexiform
May cause mechanical ptosis May be associated with glaucoma

39. Intraocular lesions in NF-1
Lisch nodules
Very common - eventually present
in 95% of cases
Congenital ectropion uveae
Uncommon - may be associated
with glaucoma
Retinal astrocytomas
Rare - identical to those seen in
tuberous sclerosis
Choroidal naevi
Common - may be multifocal
and bilateral

40. Ocular features of NF-2
Common - combined hamartomas of RPE
and retina
Very common -presenile cataract

41. Tuberous sclerosis (Bourneville disease)
• Diffuse thickening over
lumbar region
• Present in 40%
Shagreen patches
• Autosomal dominant
• Triad - mental handicap, epilepsy, adenoma sebaceum
Adenoma sebaceum
• Around nose and
cheeks
• Appear after age 1
and slowly enlarge
Ash leaf spots
• Hypopigmented skin patches
• In infants best detected using
ultraviolet light (Wood’s lamp)

42. Systemic hamartomas in tuberous sclerosis
Astrocytic cerebral hamartomas
• Slow-growing periventricular tumours
• May cause hydrocephalus, epilepsy and
mental retardation
• Usually asymptomatic and
innocuous
• Kidneys (angiomyolipoma), heart
(rhabdomyoma)
Visceral and subungual hamartomas

43. Retinal astrocytomas in tuberous scleritis
Dense white tumour Mulberry-like tumour
Early
• Innocuous tumour present in 50% of patients
• May be multiple and bilateral
Semitranslucent nodule White plaque
Advanced

44. Systemic features of v-H-L syndrome
Autosomal dominant
• Tumours - renal
carcinoma and
phaeochromocytoma
• Cysts - kidneys, liver,
pancreas, epididymis,
ovary and lungs
• Polycythaemia
CNS Haemangioblastoma
MRI of spinal cord tumour
Angiogram of cerebellar
tumour
Visceral tumours

45. Retinal capillary haemangioma
in v-H-L syndrome
Round orange-red mass
Early
• Vision-threatening tumour present in 50% of patients
• May be multiple and bilateral
Tiny lesion between
arteriole and venuole
Small red nodule
Associated dilatation and
tortuosity of feeder vessels
Advanced

46. Systemic features of Sturge-Weber syndrome
• Congenital, does not blanche
with pressure
• Associated with ipsilateral
glaucoma in 30% of cases
Naevus flammeus
• CT scan showing left
parietal haemangioma
• Complications - mental handicap,
epilepsy and hemiparesis
Meningeal haemangioma

47. Ocular features of Sturge-Weber syndrome
Normal eye
Buphthalmos in 60%May be associated with
episcleral haemangioma
Affected eye
Diffuse choroidal haemangioma
Glaucoma

48. Peripheral corneal involvement in
rheumatoid arthritis
• Chronic and asymptomatic
• Circumferential thinning with intact
epithelium (‘contact lens cornea’)
• Acute and painful
• Circumferential ulceration and
infiltration
Treatment - systemic steroids and/or cytotoxic drugs
Without inflammation With inflammation

49. Peripheral corneal involvement in
Wegener granulomatosis and polyarteritis nodos
Circumferential and central
ulceration similar to Mooren ulcer
Unlike Mooren ulcer sclera may also
become involved
Treatment - systemic steroids and cyclophosphamide

50. GIANT CELL ARTERITISGIANT CELL ARTERITIS
(Temporal or Cranial Arteritis)(Temporal or Cranial Arteritis)
 Idiopathic vasculitisIdiopathic vasculitis
 Same disease spectrum asSame disease spectrum as
polymyalgia rheumaticapolymyalgia rheumatica
 Mainly women 65-80 years oldMainly women 65-80 years old
 Medium and large arteries in head &Medium and large arteries in head &
neck involvedneck involved

51. GIANT CELL ARTERITISGIANT CELL ARTERITIS
PresentationPresentation
 HeadacheHeadache
 Scalp tendernessScalp tenderness
 ThickenedThickened
temporal arteriestemporal arteries
 Jaw claudicationJaw claudication
 Acute visual lossAcute visual loss
 Weight loss,Weight loss,
anorexia, fever,anorexia, fever,
night sweats,night sweats,
malaise &malaise &
depressiondepression

52. GIANT CELL ARTERITISGIANT CELL ARTERITIS
Ocular ComplicationsOcular Complications
 TransientTransient
monocular visualmonocular visual
loss (amaurosisloss (amaurosis
fugax)fugax)
 Visual loss due toVisual loss due to
– Central retinalCentral retinal
artery occlusionartery occlusion
(CRAO) or(CRAO) or
– AnteriorAnterior
ischaemic opticischaemic optic
neuropathyneuropathy
(AION)(AION)
 Visual field defectsVisual field defects

53. GIANT CELL ARTERITISGIANT CELL ARTERITIS
ManagementManagement
 ESR if suspectedESR if suspected
 Start high dose steroids immediatelyStart high dose steroids immediately
to prevent stroke or second eyeto prevent stroke or second eye
involvementinvolvement
 Temporal artery biopsy within aTemporal artery biopsy within a
week of starting steroidsweek of starting steroids

54. GIANT CELL ARTERITISGIANT CELL ARTERITIS
Temporal Artery BiopsyTemporal Artery Biopsy
 Arteries have skipArteries have skip
lesionslesions
 ultrasound/Dopplerultrasound/Doppler
may help identifymay help identify
involved areasinvolved areas
 If positive, confirmsIf positive, confirms
diagnosis – helpfuldiagnosis – helpful
in management ofin management of
future diseasefuture disease
 If negative, doesn’tIf negative, doesn’t
exclude diagnosis,exclude diagnosis,
but need to thinkbut need to think
about an alternativeabout an alternative
diagnosisdiagnosis

55. GIANT CELL ARTERITISGIANT CELL ARTERITIS
HistopathologyHistopathology
 Granulomatous cellGranulomatous cell
infiltrationinfiltration
 Giant cellsGiant cells
 Disruption ofDisruption of
internal elasticinternal elastic
laminalamina
 Proliferation ofProliferation of
intimaintima
 Occlusion of lumenOcclusion of lumen

56. GIANT CELL ARTERITISGIANT CELL ARTERITIS
TreatmentTreatment
 Intravenous and oral steroids –Intravenous and oral steroids –
prolonged course of steroids oftenprolonged course of steroids often
necessarynecessary

57. Ocular manifestationsOcular manifestations
of HIV infectionof HIV infection

58. IntroductionIntroduction
 AIDS is an infectious disease caused by theAIDS is an infectious disease caused by the
gradual decrease ingradual decrease in CD4+ T lymphocytesCD4+ T lymphocytes
causing subsequent opportunistic infectionscausing subsequent opportunistic infections
and neoplasia. It is a blood borne and sexuallyand neoplasia. It is a blood borne and sexually
transmitted infection caused by the HIVtransmitted infection caused by the HIV
(Human Immunodeficiency Virus)(Human Immunodeficiency Virus)
 Approximately 36 million persons around theApproximately 36 million persons around the
world are infected. Up to 70% of patientsworld are infected. Up to 70% of patients
infected with HIV will develop some form ofinfected with HIV will develop some form of
ocular involvement, ie: direct infection byocular involvement, ie: direct infection by
HIV,opportunistic infections and neoplasia.HIV,opportunistic infections and neoplasia.
 HIV infection progresses thoughHIV infection progresses though
different phasesdifferent phases

60. Ophthalmic Manifestations of HIV InfectionOphthalmic Manifestations of HIV Infection
 AROUND THE EYEAROUND THE EYE
– Molluscum ContagiosumMolluscum Contagiosum
– Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus
– Kaposi’s SarcomaKaposi’s Sarcoma
– Conjunctival Squamous Cell CarcinomaConjunctival Squamous Cell Carcinoma
– TrichomegalyTrichomegaly
 FRONT OF THE EYEFRONT OF THE EYE
– Dry EyeDry Eye
– Anterior UveitisAnterior Uveitis
 BACK OF THE EYEBACK OF THE EYE
– Retinal MicrovasculopathyRetinal Microvasculopathy
– CMV RetinitisCMV Retinitis
– Acute Retinal NecrosisAcute Retinal Necrosis
– Progressive Outer Retinal NeProgressive Outer Retinal Ne
– Toxoplasmosis RetinochoroidToxoplasmosis Retinochoroid
– Syphilis RetinitisSyphilis Retinitis
– Candida albicans endophthalCandida albicans endophthal
 NEURO-OPHTHALMICNEURO-OPHTHALMIC

61. Molluscum ContagiosumMolluscum Contagiosum
 Molluscum contagiosum isMolluscum contagiosum is
a viral infection of the skin.a viral infection of the skin.
 Affects up to 20% ofAffects up to 20% of
symptomatic HIV infectedsymptomatic HIV infected
patients.patients.
 Clinically appears likeClinically appears like
painless, small, umbilicatedpainless, small, umbilicated
nodules, which produce anodules, which produce a
waxy discharge whenwaxy discharge when
pressured.pressured.
 Treatment consists onTreatment consists on
excision of the lesion,excision of the lesion,
curettage or cryotherapycurettage or cryotherapy

62. Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus
 Due to the reactivation of a latent infection byDue to the reactivation of a latent infection by
Varicella Zoster Virus in the dorsal root ofVaricella Zoster Virus in the dorsal root of
trigeminal nerve ganglion.trigeminal nerve ganglion.
 It manifests with a maculo-papulo-vesicular rashIt manifests with a maculo-papulo-vesicular rash
which often is preceded by pain. Usually involveswhich often is preceded by pain. Usually involves
the upper lid and does not cross the midlinethe upper lid and does not cross the midline
 Treatment consists on oral Aciclovir 800mg 5Treatment consists on oral Aciclovir 800mg 5
times /day. In immunocompromised patientstimes /day. In immunocompromised patients
Aciclovir is given intravenously for two weeks.Aciclovir is given intravenously for two weeks.
Ocular manifestations such as anterior uveitis,Ocular manifestations such as anterior uveitis,
are treated with topical steroids and mydriatics.are treated with topical steroids and mydriatics.

64. Kaposi’s SarcomaKaposi’s Sarcoma
 Kaposi’s sarcoma is a vascular neoplasm which isKaposi’s sarcoma is a vascular neoplasm which is
almost exclusively seen in patients with AIDS.almost exclusively seen in patients with AIDS.
 KS is the commonest anterior segment lesion seen inKS is the commonest anterior segment lesion seen in
AIDS; appears as a violaceous non-tender nodule onAIDS; appears as a violaceous non-tender nodule on
the eyelid or conjunctiva.the eyelid or conjunctiva.
 Typically KS involves only the skin but when there isTypically KS involves only the skin but when there is
a reduced CD4 count it can progress rapidly to othera reduced CD4 count it can progress rapidly to other
sites such as the gastrointestinal tract and CNSsites such as the gastrointestinal tract and CNS
 Treatment of ocular adnexal KS may be necessaryTreatment of ocular adnexal KS may be necessary
for cosmesis and to relieve functional difficulties. Thefor cosmesis and to relieve functional difficulties. The
mainstay of treatment is radiotherapy. Other optionsmainstay of treatment is radiotherapy. Other options
include cryotherapy or chemotherapy.include cryotherapy or chemotherapy.

66. Conjunctival Squamous Cell CarcinomaConjunctival Squamous Cell Carcinoma
 Squamous cell carcinoma (SCC) is the third mostSquamous cell carcinoma (SCC) is the third most
common neoplasm associated to HIV infection.common neoplasm associated to HIV infection.
This may be due to an interaction between HIV,This may be due to an interaction between HIV,
sunlight and Human Papilloma Virus infection.sunlight and Human Papilloma Virus infection.
 SCC appears as a pink, gelatinous growth, usuallySCC appears as a pink, gelatinous growth, usually
in the interpalpebral area. Often an engorgedin the interpalpebral area. Often an engorged
blood vessel feeding the tumour is seen. It mayblood vessel feeding the tumour is seen. It may
extend onto the cornea, but deep invasion andextend onto the cornea, but deep invasion and
metastasis are rare.metastasis are rare.
 The treatment of choice is local excision andThe treatment of choice is local excision and
cryotherapy but the presence of orbital invasioncryotherapy but the presence of orbital invasion
is an indication of exenterationis an indication of exenteration

68. TrichomegalyTrichomegaly
 Trichomegaly orTrichomegaly or
hypertrichosis is anhypertrichosis is an
exaggerated growth ofexaggerated growth of
the eye lashes foundthe eye lashes found
in the later stages ofin the later stages of
the diseasethe disease
 The cause is notThe cause is not
knownknown
 When symptomatic orWhen symptomatic or
for cosmetic reasonsfor cosmetic reasons
the eyelashes can bethe eyelashes can be
trimmed or pluckedtrimmed or plucked

69. Dry EyeDry Eye
 Sicca syndrome isSicca syndrome is
frequent amongfrequent among
patients with HIVpatients with HIV
infectioninfection
 Patients complain ofPatients complain of
burningburning
uncomfortable reduncomfortable red
eyes.eyes.
 There are severalThere are several
causes of dry eye incauses of dry eye in
HIV infection fromHIV infection from
blepharitis toblepharitis to
destruction of thedestruction of the
lacrimal glands.lacrimal glands.
 Treatment is withTreatment is with

70. Anterior UveitisAnterior Uveitis
 HIV related anteriorHIV related anterior uveitisuveitis
can be:can be:
– Direct manifestation of theDirect manifestation of the
human immunodeficiencyhuman immunodeficiency
virus infectionvirus infection
– autoimmnune in originautoimmnune in origin
– drug induced ie: rifabutin,drug induced ie: rifabutin,
secondary to direct toxicsecondary to direct toxic
effect upon the non-effect upon the non-
pigmented epithelium ofpigmented epithelium of
the ciliary bodythe ciliary body
– Any of the differentAny of the different
infections associated withinfections associated with
AIDS, ie: Herpes ZosterAIDS, ie: Herpes Zoster
Virus, Herpes SimplexVirus, Herpes Simplex

71. Rifabutin induced anterior uveitisRifabutin induced anterior uveitis

72. Retinal microvasculitisRetinal microvasculitis
 Retinal microvasculopathy occurs in more than half ofRetinal microvasculopathy occurs in more than half of
the patients with HIVthe patients with HIV
 It is seen as transient cotton wool spots (CWS), intra-It is seen as transient cotton wool spots (CWS), intra-
retinal haemorrhages and microaneurysm, which occursretinal haemorrhages and microaneurysm, which occurs
in 50-70% of patients. It is usually asymptomatic.in 50-70% of patients. It is usually asymptomatic.
 It has an unclear pathogenesis, but it is thought to beIt has an unclear pathogenesis, but it is thought to be
HIV infection of retinal vascular cells.HIV infection of retinal vascular cells.
 In an otherwise healthy individual the presence ofIn an otherwise healthy individual the presence of
CWS, should be differentiated from other forms ofCWS, should be differentiated from other forms of
retinopathy, such as diabetic or hypertensiveretinopathy, such as diabetic or hypertensive
retinopathy. Serological test for HIV will confirm theretinopathy. Serological test for HIV will confirm the
diagnosisdiagnosis
 Treatment is based in delaying the progression of theTreatment is based in delaying the progression of the

73. Cotton Wool SpotsCotton Wool Spots

74. CMV RetinitisCMV Retinitis
 IntroductionIntroduction
– CMV Retinitis is the commonest intraocular ocularCMV Retinitis is the commonest intraocular ocular
opportunistic infection seen in patients with AIDSopportunistic infection seen in patients with AIDS
– Antibodies are found in almost 95% of adults,Antibodies are found in almost 95% of adults,
causing a trivial illness in immunocompetent adults,causing a trivial illness in immunocompetent adults,
however severe immunosuppression causes viralhowever severe immunosuppression causes viral
reactivation and tissue invasive diseasereactivation and tissue invasive disease
 PathogenesisPathogenesis
– Reactivation from extraocular sites leads to seedingReactivation from extraocular sites leads to seeding
in other sites such as the retinain other sites such as the retina
 EpidemiologyEpidemiology
– The number of newly diagnosed cases of CMVR hasThe number of newly diagnosed cases of CMVR has
decreased since the introduction of the HAARTdecreased since the introduction of the HAART
Highly Active Antiretroviral TherapyHighly Active Antiretroviral Therapy

75. CMV RetinitisCMV Retinitis
 Clinical manifestationsClinical manifestations
– Patients may complain of minor visual symptoms such asPatients may complain of minor visual symptoms such as
floaters, flashing lights or mild blurred vision, or be totallyfloaters, flashing lights or mild blurred vision, or be totally
asymptomatic.asymptomatic.
– It presents with a wide range of clinical appearances. FromIt presents with a wide range of clinical appearances. From
cotton wool spots which may look like HIV Retinopathy tocotton wool spots which may look like HIV Retinopathy to
confluent areas of full thickness retinal necrosis and vasculitis.confluent areas of full thickness retinal necrosis and vasculitis.
CMVR can progress in a “brushfire” pattern from the activeCMVR can progress in a “brushfire” pattern from the active
edge of an active lesion. The retinal vessels in an affected areaedge of an active lesion. The retinal vessels in an affected area
show attenuation, becoming ghost vessels eventually.show attenuation, becoming ghost vessels eventually.
 TreatmentTreatment
– The treatment of CMVR in patients with AIDS requires the useThe treatment of CMVR in patients with AIDS requires the use
of specific antiviral agents, ganciclovir, foscarnet or cidovir inof specific antiviral agents, ganciclovir, foscarnet or cidovir in
conjunction with HAART.conjunction with HAART.
– These treatments can be administered orally, intravenously orThese treatments can be administered orally, intravenously or
intravitreally. Systemic treatment has the advantage of treatingintravitreally. Systemic treatment has the advantage of treating
infection elsewhere in the body as well as the other eye but hasinfection elsewhere in the body as well as the other eye but has

76. CMV RetinitisCMV Retinitis

77. Acute Retinal NecrosisAcute Retinal Necrosis
 ARN is a confluent peripheral whitening of theARN is a confluent peripheral whitening of the
retina with marked vitritis and blood vesselretina with marked vitritis and blood vessel
closure. Optic neuritis and retinal detachment areclosure. Optic neuritis and retinal detachment are
frequent complications.frequent complications.
 ARN is usually due to Varicella-Zoster infection,ARN is usually due to Varicella-Zoster infection,
but it can also be caused by Herpes Simplex virusbut it can also be caused by Herpes Simplex virus
or Cytomegalovirus.or Cytomegalovirus.
 Initially described in the immunocompetent, it hasInitially described in the immunocompetent, it has
also been described in the immunosuppressed.also been described in the immunosuppressed.
 The diagnosis is mainly clinical and is confirmedThe diagnosis is mainly clinical and is confirmed
by PCR assays on vitreous samples.by PCR assays on vitreous samples.
 Patients are treated with high doses ofPatients are treated with high doses of
intravenous aciclovir or famciclovir, combined withintravenous aciclovir or famciclovir, combined with
laser treatment to prevent retinal detachment.laser treatment to prevent retinal detachment.

78. Acute Retinal NecrosisAcute Retinal Necrosis

79. Progressive Outer RetinalProgressive Outer Retinal
NecrosisNecrosis
(Varicella-Zoster Retinitis)(Varicella-Zoster Retinitis)
 PORN is a devastating viral retinitis caused by Varicella-PORN is a devastating viral retinitis caused by Varicella-
Zoster virus, without vitritis or retinal vasculitis.Zoster virus, without vitritis or retinal vasculitis.
 The retinitis can be located anywhere but it is commonThe retinitis can be located anywhere but it is common
for the lesions to coalesce and spread posteriorly in afor the lesions to coalesce and spread posteriorly in a
rapid fashion.rapid fashion.
 The main symptom is rapid loss of vision.The retinaThe main symptom is rapid loss of vision.The retina
shows typically a white lesion with no haemorrhages orshows typically a white lesion with no haemorrhages or
exudates.exudates.
 Treatment is often unsatisfactory and usually requiresTreatment is often unsatisfactory and usually requires
combination of Ganciclovir and Aciclovir. The prognosiscombination of Ganciclovir and Aciclovir. The prognosis
is very poor and retinal detachment is common.is very poor and retinal detachment is common.
Resolution may leave a white plaque with theResolution may leave a white plaque with the
appearance of “cracked mud”.appearance of “cracked mud”.

81. Toxoplasma RetinochoroiditisToxoplasma Retinochoroiditis
 Toxoplasmosis retinochoroiditis is an uncommonToxoplasmosis retinochoroiditis is an uncommon
infection of the eye in AIDS. Ocular toxoplasmosisinfection of the eye in AIDS. Ocular toxoplasmosis
in HIV positive patients is different in appearancein HIV positive patients is different in appearance
from immunocompetent patients. Unlike infrom immunocompetent patients. Unlike in
immunocompetent patients, HIV infected patientsimmunocompetent patients, HIV infected patients
often have bilateral and multifocal diseaseoften have bilateral and multifocal disease
associated with anterior uveitis and vitritis butassociated with anterior uveitis and vitritis but
unlike immunocompetent patients, in HIVunlike immunocompetent patients, in HIV
infected patients often have with no pigmentedinfected patients often have with no pigmented
scars adjacent to the areas of retinal necrosis.scars adjacent to the areas of retinal necrosis.
Toxoplasmosis in immunocompromised patientsToxoplasmosis in immunocompromised patients
is not self-limiting as it is in imunocompetentis not self-limiting as it is in imunocompetent
patients.patients.

82. Toxoplasma RetinochoroiditisToxoplasma Retinochoroiditis
 When testing patients for antibodies toWhen testing patients for antibodies to
toxoplasmosis both IgG and IgM levels may betoxoplasmosis both IgG and IgM levels may be
raised, but in immunocompromised patientsraised, but in immunocompromised patients
these tests may be negative.these tests may be negative.
 Treatment in immunocompromised patientsTreatment in immunocompromised patients
consists in the association of sulphadiazine orconsists in the association of sulphadiazine or
clindamycin, pyrimethamine and folinic acidclindamycin, pyrimethamine and folinic acid
(triple therapy).(triple therapy).
 Long term maintenance treatment may beLong term maintenance treatment may be
needed in order to prevent relapses.needed in order to prevent relapses.
 Often associated with toxoplasma lesions in theOften associated with toxoplasma lesions in the
Central Nervous System.Central Nervous System.

83. MRI T1 showing an uniformly
enhancing lesion in the
midbrain
One week later, the lesion
showing ring enhancement

84. ImmunocompetentImmunocompetent ImmunocompromisedImmunocompromised

85. Syphilis RetinitisSyphilis Retinitis
 There is a strong association betweenThere is a strong association between
syphilis and HIV infection.syphilis and HIV infection.
 It can manifest as a retinitis with denseIt can manifest as a retinitis with dense
vitritis, retinal vasculitis, serous retinalvitritis, retinal vasculitis, serous retinal
detachment or neuroretinitis, as well as otherdetachment or neuroretinitis, as well as other
types of ocular involvement such as,types of ocular involvement such as,
conjunctivitis, anterior uveitis, cranial nerveconjunctivitis, anterior uveitis, cranial nerve
palsies and optic neuritis.palsies and optic neuritis.
 Treatment consists in high dose ofTreatment consists in high dose of
intravenous Penicillin for 2 weeks.intravenous Penicillin for 2 weeks.

87. Candida albicansCandida albicans
endophthalmitisendophthalmitis
 Infection with candida albicans is rare. CandidaInfection with candida albicans is rare. Candida
albicans is the commonest cause of fungalalbicans is the commonest cause of fungal
endophthalmitisendophthalmitis
 Affected patients usually have a history of drugAffected patients usually have a history of drug
abuse or indwelling central linesabuse or indwelling central lines
 In the initial stages, floaters are the mainIn the initial stages, floaters are the main
symptom. As the condition progresses, whitishsymptom. As the condition progresses, whitish
“puff-balls” and vitreous strands develop. Later,“puff-balls” and vitreous strands develop. Later,
similar infiltrates appear in the choroid and retinasimilar infiltrates appear in the choroid and retina
 The treatment depends on the severity of theThe treatment depends on the severity of the
ocular involvement and systemic disease. Theocular involvement and systemic disease. The
original foci should be removed. The drugs oforiginal foci should be removed. The drugs of
choice are Amphotericine B and Fluconazolchoice are Amphotericine B and Fluconazol

88. Candida albicansCandida albicans
endophthalmitisendophthalmitis

89. GlossaryGlossary
 CD4CD4: Director of the immune response. When activated: Director of the immune response. When activated
it releasesit releases cytokines which in turn will activate thecytokines which in turn will activate the
immune systemimmune system
 Cotton Wool SpotsCotton Wool Spots: Light-coloured deposits in the: Light-coloured deposits in the
retina secondaryretina secondary to infarcts of the nerve fibre layerto infarcts of the nerve fibre layer
 HAARTHAART: Highly Active Antiretroviral Therapy: Highly Active Antiretroviral Therapy
 ImmunoblogulinImmunoblogulin: Protein in charge of fighting foreign: Protein in charge of fighting foreign
substances insubstances in our body.our body. IgGIgG is the commonest type ofis the commonest type of
immunoglobulin andimmunoglobulin and IgMIgM is theis the
earliest class of immunoglobulin.earliest class of immunoglobulin.
 PCRPCR: Polymerase Chain Reaction is a technique used to: Polymerase Chain Reaction is a technique used to
makemake numerous copies of an specific portionnumerous copies of an specific portion
of DNAof DNA
 VDRLVDRL: Venereal Disease Research Laboratory. The test: Venereal Disease Research Laboratory. The test

90. Questions?