Systemic disease and the eye - International Council of Ophthalmology

Systemic disease and Systemic disease and the eyethe eyeDeric De WitDeric De WitAldrinAldrin KhanKhan

Professor LightmanProfessor Lightman

Common systemic diseases Common systemic diseases affecting the eyeaffecting the eye

NonNon--infectiousinfectiousEndocrine Endocrine –– diabetes, diabetes, thyroidthyroidConnective tissue Connective tissue disease disease ––RA/SLE/RA/SLE/WegenersWegeners/PAN//PAN/Systemic sclerosisSystemic sclerosisVasculitidesVasculitides (GCA)(GCA)SarcoidosisSarcoidosisBehcetBehcet’’ss DiseaseDiseaseVogt Vogt KoyanagiKoyanagi Harada Harada syndromesyndromePhakomatoses

InfectiousInfectiousToxoplasmosisToxoplasmosisToxocariasisToxocariasisTBTBSyphilisSyphilisLeprosyLeprosyHIVHIVCMVCMV

Phakomatoses

DIABETIC RETINOPATHY

1. Adverse risk factors2. Pathogenesis3. Background diabetic retinopathy

5. Clinically significant macular oedema6. Preproliferative diabetic retinopathy

4. Diabetic maculopathies• Focal• Diffuse• Ischaemic

7. Proliferative diabetic retinopathy

Adverse Risk Factors1. Long duration of diabetes

2. Poor metabolic control

3. Pregnancy

4. Hypertension

5. Renal disease

• Obesity• Hyperlipidaemia

6. Other

• Smoking• Anaemia

Location of lesions in background diabetic retinopathy

Signs of background diabetic retinopathy

Microaneurysms usually temporal to fovea

Intraretinal dot and blot haemorrhages

Hard exudates frequentlyarranged in clumps or rings

Retinal oedema seen asthickening on biomicroscopy

Preproliferative diabetic retinopathy

• Cotton-wool spots• Venous irregularities

• Dark blot haemorrhages• Intraretinal microvascular

abnormalities (IRMA)

Signs

Treatment - not required but watch for proliferative disease

Proliferative diabetic retinopathy

• Flat or elevated• Severity determined by comparing with area of disc

Neovascularization

• Affects 5-10% of diabetics• IDD at increased risk (60% after 30 years)

Neovascularization of disc = NVDNeovascularization elsewhere = NVE

Laser panretinal photocoagulation

• Spot size (200-500 µm) dependson 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

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)

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

THYROID EYE DISEASE

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

2. Eyelid retraction3. Proptosis4. Optic neuropathy5. Restrictive myopathy

Soft tissue involvementPeriorbital and lid swelling

Chemosis

Conjunctival hyperaemia

Superior limbic keratoconjunctivitis

Signs of eyelid retractionOccurs in about 50%

• Bilateral lid retraction • No associated proptosis

• Bilateral lid retraction • Bilateral proptosis

• Lid lag in downgaze• Unilateral lid retraction • Unilateral proptosis

Proptosis• Occurs in about 50% • Uninfluenced by treatment of hyperthyroidism

Treatment optionsAxial and permanent in about 70% May be associated with choroidal folds

• Systemic steroids • Radiotherapy • Surgical decompression

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

• Occurs in about 40% • Due to fibrotic contracture

Restrictive myopathy

Elevation defect - most common Abduction defect - less common

Depression defect - uncommon Adduction defect - rare

SARCOIDOSISSARCOIDOSIS

Idiopathic multisystem disorderIdiopathic multisystem disorderCharacterised by nonCharacterised by non--caseating caseating granulomatagranulomataMore common in women 20More common in women 20--50 yrs50 yrsMore common in blacks and AsiansMore common in blacks and Asians? Related to mycobacteria? Related to mycobacteria

SARCOIDOSISSARCOIDOSISSystemic InvolvementSystemic Involvement

Lung lesions Lung lesions –– 95%95%Thoracic lymph nodes Thoracic lymph nodes –– 50%50%Skin lesions Skin lesions –– 30% 30% →→Eyes Eyes –– 30%30%

SARCOIDOSISSARCOIDOSISOcular InvolvementOcular Involvement

Anterior segment Anterior segment lesions (30%)lesions (30%)

Conjunctival granulomaConjunctival granulomaLacrimal gland Lacrimal gland involvement/dry eyeinvolvement/dry eyeAcute or chronic uveitis Acute or chronic uveitis →→KPs described as KPs described as ‘‘mutton fatmutton fat’’ because they because they are large and greasyare large and greasy


Posterior segment Posterior segment lesions (20%)lesions (20%)

Patchy venous sheathingPatchy venous sheathingCellular infiltrate around Cellular infiltrate around vesselsvesselsChorioretinalChorioretinalgranulonmasgranulonmasVasculitis including Vasculitis including occlusive causing:occlusive causing:--NeovascularisationNeovascularisationInfiltrate in vitreous Infiltrate in vitreous (vitritis) including cell (vitritis) including cell clumps (snowballs)clumps (snowballs)


Sheathing of the Sheathing of the retinal veinsretinal veins

Fluorescein Fluorescein angiography showing angiography showing leakage and staining leakage and staining at sites of sheathingat sites of sheathing

SARCOIDOSISSARCOIDOSISGranuloma in FundusGranuloma in Fundus

Retinal and preRetinal and pre--retinalretinal

Choroidal Choroidal

SARCOIDOSISSARCOIDOSISGranuloma in FundusGranuloma in Fundus

Optic nerve head Optic nerve head granulomagranuloma

Normal optic nerve Normal optic nerve headhead

SARCOIDOSISSARCOIDOSISSystemic SignsSystemic Signs

Lupus pernio affecting Lupus pernio affecting the nose the nose –– a chronic a chronic progressive progressive cutaneous sarcoid cutaneous sarcoid that most commonly that most commonly affects face and earsaffects face and ears

SARCOIDOSISSARCOIDOSISSystemic signsSystemic signs

Facial palsyFacial palsy

Salivary gland Salivary gland enlargementenlargement

SARCOIDOSISSARCOIDOSISSystemic signsSystemic signs

Hilar adenopathy on Hilar adenopathy on chest xchest x--rayrayLung infiltrateLung infiltrate

Erythema nodosumErythema nodosum

ArthritisArthritis

SARCOIDOSISSARCOIDOSISInvestigations (1)Investigations (1)

CXR CXR –– to detect to detect pulmonary signspulmonary signsBilateral hilar lymphBilateral hilar lymph--adenopathyadenopathy

Pulmonary mottlingPulmonary mottling


Serum angiotensinSerum angiotensin--converting enzyme converting enzyme (ACE) (ACE) –– elevated in active sarcoidosiselevated in active sarcoidosisMantoux test Mantoux test –– caution in patients who caution in patients who have had BCG vaccination. Test may be have had BCG vaccination. Test may be negativenegativeLung function testsLung function tests


Gallium scan showing Gallium scan showing increased uptake in increased uptake in the lacrimal and the lacrimal and parotid glands and parotid glands and pulmonary regions in pulmonary regions in a patient with active a patient with active sarcoidosissarcoidosis

SARCOIDOSISSARCOIDOSISTreatmentTreatment

Systemic steroids may be necessary in Systemic steroids may be necessary in patients with posterior segment disease patients with posterior segment disease where vision is threatened, especially if where vision is threatened, especially if

optic nerve is involvedoptic nerve is involved

PHACOMATOSES

1. Neurofibromatosis• Type I (NF-1) - von Recklinghausen disease

• Type II (NF-2) - bilateral acoustic neuromas

2. Tuberous sclerosis (Bourneville disease)

3. von-Hippel-Lindau syndrome

4. Sturge-Weber syndrome

Neurofibromatosis type-1 - (NF-1)• Most common phacomatosis• Affects 1:4000 individuals• Presents in childhood• Gene localized to chromosome 17q11

Appear during first year of life

Café-au-lait spots

Increase in size and number throughout childhood

Fibroma molluscum in NF-1

• Appear at puberty• Pedunculated, flabby nodules consisting of neurofibromas or schwannomas

• Increase in number throughout life

• Frequently widely distributed

Plexiform neurofibroma in NF-1

• May be associated with overgrowth of overlying skin

• Appear during childhood• Large and ill-defined

Skeletal defects in NF-1

• Mild head enlargement - uncommon• Other - scoliosis, short stature, thinning of

long bones

• Facial hemiatrophy

Orbital lesions in NF-1Spheno-orbital encephaloceleOptic nerve glioma in about 15%

• Sagittal MRI scan of optic nerve gliomainvading 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

Eyelid neurofibromas in NF-1

Nodular Plexiform

May cause mechanical ptosis May be associated with glaucoma

Intraocular lesions in NF-1Lisch nodules

Very common - eventually presentin 95% of cases

Congenital ectropion uveae

Uncommon - may be associatedwith glaucoma

Retinal astrocytomas

Rare - identical to those seen intuberous sclerosis

Choroidal naevi

Common - may be multifocaland bilateral

Ocular features of NF-2

Common - combined hamartomas of RPEand retina

Very common -presenile cataract

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)

Systemic hamartomas in tuberous sclerosisAstrocytic 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

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 plaqueAdvanced

Systemic features of v-H-L syndromeAutosomal dominant

• Tumours - renal carcinoma and phaeochromocytoma

• Cysts - kidneys, liver,pancreas, epididymis, ovary and lungs

• Polycythaemia

CNS Haemangioblastoma

MRI of spinal cord tumour

Angiogram of cerebellartumour

Visceral tumours

Retinal capillary haemangiomain 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

Systemic features of Sturge-Weber syndrome

• Congenital, does not blanche with pressure

• Associated with ipsilateralglaucoma in 30% of cases

Naevus flammeus

• CT scan showing left parietal haemangioma

• Complications - mental handicap, epilepsy and hemiparesis

Meningeal haemangioma

Ocular features of Sturge-Weber syndrome

Normal eye

Buphthalmos in 60% May be associated with episcleral haemangioma

Affected eye

Diffuse choroidal haemangioma

Glaucoma

Peripheral corneal involvement in rheumatoid arthritis

• Chronic and asymptomatic• Circumferential thinning with intact

epithelium (‘contact lens cornea’)

• Acute and painful• Circumferential ulceration and

infiltration

Without inflammation With inflammation

Treatment - systemic steroids and/or cytotoxic drugs

Peripheral corneal involvement in Wegener granulomatosis and polyarteritis nodos

Circumferential and centralulceration similar to Mooren ulcer

Unlike Mooren ulcer sclera may alsobecome involved

Treatment - systemic steroids and cyclophosphamide

GIANT CELL ARTERITISGIANT CELL ARTERITIS(Temporal or Cranial Arteritis)(Temporal or Cranial Arteritis)Idiopathic vasculitisIdiopathic vasculitisSame disease spectrum as polymyalgia Same disease spectrum as polymyalgia rheumaticarheumaticaMainly women 65Mainly women 65--80 years old80 years oldMedium and large arteries in head & neck Medium and large arteries in head & neck involvedinvolved

GIANT CELL ARTERITISGIANT CELL ARTERITISPresentationPresentation

HeadacheHeadacheScalp tendernessScalp tendernessThickened temporal Thickened temporal arteriesarteriesJaw claudicationJaw claudicationAcute visual lossAcute visual lossWeight loss, anorexia, Weight loss, anorexia, fever, night sweats, fever, night sweats, malaise & depressionmalaise & depression

GIANT CELL ARTERITISGIANT CELL ARTERITISOcular ComplicationsOcular Complications

Transient monocular Transient monocular visual loss (amaurosis visual loss (amaurosis fugax)fugax)Visual loss due toVisual loss due to

Central retinal artery Central retinal artery occlusion (CRAO) or occlusion (CRAO) or Anterior ischaemic Anterior ischaemic optic neuropathy optic neuropathy (AION)(AION)

Visual field defectsVisual field defects

GIANT CELL ARTERITISGIANT CELL ARTERITISManagementManagement

ESR if suspectedESR if suspectedStart high dose steroids immediately to Start high dose steroids immediately to prevent stroke or second eye involvementprevent stroke or second eye involvementTemporal artery biopsy within a week of Temporal artery biopsy within a week of starting steroidsstarting steroids

GIANT CELL ARTERITIS GIANT CELL ARTERITIS Temporal Artery BiopsyTemporal Artery Biopsy

Arteries have skip Arteries have skip lesionslesionsultrasound/Doppler may ultrasound/Doppler may help identify involved help identify involved areasareasIf positive, confirms If positive, confirms diagnosis diagnosis –– helpful in helpful in management of future management of future diseasediseaseIf negative, doesnIf negative, doesn’’t t exclude diagnosis, but exclude diagnosis, but need to think about an need to think about an alternative diagnosisalternative diagnosis

GIANT CELL ARTERITISGIANT CELL ARTERITISHistopathologyHistopathology

Granulomatous cell Granulomatous cell infiltrationinfiltrationGiant cellsGiant cellsDisruption of internal Disruption of internal elastic laminaelastic laminaProliferation of intimaProliferation of intimaOcclusion of lumenOcclusion of lumen

GIANT CELL ARTERITISGIANT CELL ARTERITISTreatmentTreatment

Intravenous and oral steroids Intravenous and oral steroids –– prolonged prolonged course of steroids often necessarycourse of steroids often necessary

Ocular manifestations of Ocular manifestations of HIV infectionHIV infection

IntroductionIntroduction

AIDS is an infectious disease caused by the gradual AIDS is an infectious disease caused by the gradual decrease in decrease in CD4+ T lymphocytesCD4+ T lymphocytes causing causing subsequent opportunistic infections and subsequent opportunistic infections and neoplasianeoplasia. It . It is a blood borne and sexually transmitted infection is a blood borne and sexually transmitted infection caused by the HIV (Human Immunodeficiency Virus)caused by the HIV (Human Immunodeficiency Virus)Approximately 36 million persons around the world Approximately 36 million persons around the world are infected. are infected. Up to 70% of patients infected with HIV Up to 70% of patients infected with HIV will develop some form of ocular involvement, will develop some form of ocular involvement, ieie: : direct infection by HIV,opportunistic infections and direct infection by HIV,opportunistic infections and neoplasianeoplasia..HIV infection progresses though HIV infection progresses though different phasesdifferent phases

Ophthalmic Manifestations of HIV InfectionOphthalmic Manifestations of HIV Infection

AROUND THE EYEAROUND THE EYEMolluscumMolluscum ContagiosumContagiosumHerpes Zoster Herpes Zoster OphthalmicusOphthalmicusKaposiKaposi’’s Sarcomas SarcomaConjunctivalConjunctival SquamousSquamousCell CarcinomaCell CarcinomaTrichomegalyTrichomegaly

FRONT OF THE EYEFRONT OF THE EYEDry EyeDry EyeAnterior Uveitis

BACK OF THE EYEBACK OF THE EYERetinal Retinal MicrovasculopathyMicrovasculopathyCMV RetinitisCMV RetinitisAcute Retinal NecrosisAcute Retinal NecrosisProgressive Outer Retinal Progressive Outer Retinal NecrosisNecrosisToxoplasmosis Toxoplasmosis RetinochoroiditisRetinochoroiditisSyphilis RetinitisSyphilis RetinitisCandida Candida albicansalbicansendophthalmitisendophthalmitis

NEURONEURO--OPHTHALMICAnterior Uveitis

OPHTHALMIC

Molluscum ContagiosumMolluscum Contagiosum

MolluscumMolluscum contagiosumcontagiosum is a is a viral infection of the skin.viral infection of the skin.Affects up to 20% of Affects up to 20% of symptomaticsymptomatic HIV infected HIV infected patients.patients.Clinically appears like painless, Clinically appears like painless, small, small, umbilicatedumbilicated nodules, nodules, which produce a waxy which produce a waxy discharge when pressured.discharge when pressured.Treatment consists on excision Treatment consists on excision of the lesion, curettage or of the lesion, curettage or cryotherapycryotherapy

Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus

Due to the reactivation of a latent infection by Due to the reactivation of a latent infection by VaricellaVaricellaZoster Virus in the dorsal root of trigeminal nerve Zoster Virus in the dorsal root of trigeminal nerve ganglion.ganglion.It manifests with a It manifests with a maculomaculo--papulopapulo--vesicularvesicular rashrash which which often is preceded by pain. Usually involves the upper lid often is preceded by pain. Usually involves the upper lid and does not cross the midline and does not cross the midline Treatment consists on oral Treatment consists on oral AciclovirAciclovir 800mg 5 times 800mg 5 times /day. In /day. In immunocompromisedimmunocompromised patients patients AciclovirAciclovir is given is given intravenously for two weeks. Ocular manifestations intravenously for two weeks. Ocular manifestations such as anterior uveitis, are treated with topical steroids such as anterior uveitis, are treated with topical steroids and and mydriaticsmydriatics..

KaposiKaposi’’s Sarcomas Sarcoma

KaposiKaposi’’s sarcoma is a vascular neoplasm which is almost s sarcoma is a vascular neoplasm which is almost exclusively seen in patients with AIDS.exclusively seen in patients with AIDS.KS is the commonest KS is the commonest anterior segment lesionanterior segment lesion seen in AIDS; seen in AIDS; appears as a appears as a violaceousviolaceous nonnon--tender noduletender nodule on the eyelid or on the eyelid or conjunctiva.conjunctiva.Typically KS involves only the skin but when there is a Typically KS involves only the skin but when there is a reduced CD4 count it can progress rapidly to other sites reduced CD4 count it can progress rapidly to other sites such as the gastrointestinal tract and CNSsuch as the gastrointestinal tract and CNSTreatment of ocular Treatment of ocular adnexaladnexal KS may be necessary for KS may be necessary for cosmesiscosmesis and to relieve functional difficulties. The mainstay and to relieve functional difficulties. The mainstay of treatment is radiotherapy. Other options include of treatment is radiotherapy. Other options include cryotherapycryotherapy or chemotherapy.or chemotherapy.

ConjunctivalConjunctival SquamousSquamous Cell CarcinomaCell Carcinoma

SquamousSquamous cell carcinoma (SCC) is the third most cell carcinoma (SCC) is the third most common neoplasm associated to HIV infection. This may common neoplasm associated to HIV infection. This may be due to an interaction between HIV, sunlight and be due to an interaction between HIV, sunlight and Human Human PapillomaPapilloma Virus infection.Virus infection.SCC appears as a SCC appears as a pink, gelatinous growth, usually in the pink, gelatinous growth, usually in the interpalpebralinterpalpebral areaarea. Often an engorged. Often an engorged blood vessel blood vessel feeding the tumour is seen. It may extend onto the feeding the tumour is seen. It may extend onto the cornea, but deep invasion and metastasis are rare. cornea, but deep invasion and metastasis are rare. The treatment of choice is local excision and The treatment of choice is local excision and cryotherapycryotherapybut the presence of orbital invasion is an indication of but the presence of orbital invasion is an indication of exenterationexenteration

TrichomegalyTrichomegaly

TrichomegalyTrichomegaly or or hypertrichosishypertrichosis is an is an exaggerated growth of exaggerated growth of the eye lashes found in the eye lashes found in the later stages of the the later stages of the diseasediseaseThe cause is not knownThe cause is not knownWhen symptomatic or for When symptomatic or for cosmetic reasons the cosmetic reasons the eyelashes can be eyelashes can be trimmed or pluckedtrimmed or plucked

Dry EyeDry EyeSiccaSicca syndrome is syndrome is frequent among frequent among patients with HIV patients with HIV infectioninfectionPatients complain of Patients complain of burning uncomfortable burning uncomfortable red eyes.red eyes.There are several There are several causes of dry eye in causes of dry eye in HIV infection from HIV infection from blepharitisblepharitis to to destruction of the destruction of the lacrimallacrimal glands.glands.Treatment is with tear Treatment is with tear supplements supplements

Anterior UveitisAnterior UveitisHIV related anteriorHIV related anterior uveitis can uveitis can be:be:

Direct manifestation of the Direct manifestation of the human immunodeficiency human immunodeficiency virus infectionvirus infectionautoimmnuneautoimmnune in originin origindrug induced drug induced ieie: : rifabutinrifabutin, , secondary to direct toxic secondary to direct toxic effect upon the noneffect upon the non--pigmented epithelium of the pigmented epithelium of the ciliaryciliary body body Any of the different infections Any of the different infections associated with AIDS, associated with AIDS, ieie: : Herpes Zoster Virus, Herpes Herpes Zoster Virus, Herpes Simplex Virus, Simplex Virus,

RifabutinRifabutin induced anterior uveitisinduced anterior uveitis

Retinal Retinal microvasculitismicrovasculitis

Retinal Retinal microvasculopathymicrovasculopathy occurs in more than half of the occurs in more than half of the patients with HIVpatients with HIVIt is seen as transient It is seen as transient cotton wool spots cotton wool spots ((CWSCWS), intra), intra--retinal retinal haemorrhages and haemorrhages and microaneurysmmicroaneurysm, which occurs in 50, which occurs in 50--70% of 70% of patients. It is usually asymptomatic.patients. It is usually asymptomatic.It has an unclear pathogenesis, but it is thought to be HIV It has an unclear pathogenesis, but it is thought to be HIV infection of retinal vascular cells.infection of retinal vascular cells.In an otherwise healthy individual the presence of CWS, should In an otherwise healthy individual the presence of CWS, should be differentiated from other forms of retinopathy, such as be differentiated from other forms of retinopathy, such as diabetic or hypertensive retinopathy. Serological test for HIV wdiabetic or hypertensive retinopathy. Serological test for HIV will ill confirm the diagnosisconfirm the diagnosisTreatment is based in delaying the progression of the disease Treatment is based in delaying the progression of the disease associated with HIVassociated with HIV

Cotton Wool SpotsCotton Wool Spots

CMV RetinitisCMV Retinitis

IntroductionIntroductionCMV Retinitis is the commonest intraocular ocular opportunistic CMV Retinitis is the commonest intraocular ocular opportunistic infection infection seen in patients with AIDSseen in patients with AIDSAntibodies are found in almost 95% of adults, causing a trivial Antibodies are found in almost 95% of adults, causing a trivial illness in illness in immunocompetentimmunocompetent adults, however severe adults, however severe immunosuppressionimmunosuppression causes causes viral reactivation and tissue invasive diseaseviral reactivation and tissue invasive disease

PathogenesisPathogenesisReactivation from Reactivation from extraocularextraocular sites leads to seeding in other sites such sites leads to seeding in other sites such as the retinaas the retina

EpidemiologyEpidemiologyThe number of newly diagnosed cases of CMVR has decreased since The number of newly diagnosed cases of CMVR has decreased since the introduction of the the introduction of the HAARTHAART

Highly Active Antiretroviral TherapyHighly Active Antiretroviral Therapy


Clinical manifestationsClinical manifestationsPatients may complain of minor visual symptoms such as floatersPatients may complain of minor visual symptoms such as floaters, , flashing lights or mild blurred vision, or be totally asymptomatflashing lights or mild blurred vision, or be totally asymptomatic.ic.It presents with a wide range of It presents with a wide range of clinical appearancesclinical appearances. From cotton wool . From cotton wool spots which may look like HIV Retinopathy to confluent areas of spots which may look like HIV Retinopathy to confluent areas of full full thickness retinal necrosis and thickness retinal necrosis and vasculitisvasculitis. CMVR can progress in a . CMVR can progress in a ““brushfirebrushfire”” pattern from the active edge of an active lesion. The retinal pattern from the active edge of an active lesion. The retinal vessels in an affected area show attenuation, becoming ghost vesvessels in an affected area show attenuation, becoming ghost vessels sels eventually.eventually.

TreatmentTreatmentThe treatment of CMVR in patients with AIDS requires the use of The treatment of CMVR in patients with AIDS requires the use of specific specific antiviral agents, antiviral agents, ganciclovirganciclovir, , foscarnetfoscarnet or or cidovircidovir in conjunction with in conjunction with HAART.HAART.These treatments can be administered orally, intravenously or These treatments can be administered orally, intravenously or

intravitreallyintravitreally. Systemic treatment has the advantage of treating infection . Systemic treatment has the advantage of treating infection elsewhere in the body as well as the other eye but has the elsewhere in the body as well as the other eye but has the disadvantages of systemic side effects.disadvantages of systemic side effects.I i l i l l h d i h i d hi i


Acute Retinal NecrosisAcute Retinal Necrosis

ARN is a ARN is a confluent peripheral whitening of the retina with confluent peripheral whitening of the retina with marked marked vitritisvitritis and blood vessel closureand blood vessel closure. Optic neuritis . Optic neuritis and retinal detachment are frequent complications.and retinal detachment are frequent complications.ARN is usually due to ARN is usually due to VaricellaVaricella--Zoster infection, but it can Zoster infection, but it can also be caused by Herpes Simplex virus or also be caused by Herpes Simplex virus or Cytomegalovirus.Cytomegalovirus.Initially described in the Initially described in the immunocompetentimmunocompetent, it has also , it has also been described in the been described in the immunosuppressedimmunosuppressed..The diagnosis is mainly clinical and is confirmed by The diagnosis is mainly clinical and is confirmed by PCR PCR assaysassays on vitreous samples.on vitreous samples.Patients are treated with high doses of intravenous Patients are treated with high doses of intravenous acicloviraciclovir or or famciclovirfamciclovir, combined with laser treatment to , combined with laser treatment to prevent retinal detachment.prevent retinal detachment.

Acute Retinal NecrosisAcute Retinal Necrosis

Progressive Outer Retinal Progressive Outer Retinal NecrosisNecrosis

((VaricellaVaricella--Zoster Retinitis)Zoster Retinitis)PORN is a devastating viral retinitis caused by PORN is a devastating viral retinitis caused by VaricellaVaricella--Zoster Zoster virus, without virus, without vitritisvitritis or retinal or retinal vasculitisvasculitis..The retinitis can be located anywhere but it is common for the The retinitis can be located anywhere but it is common for the lesions to coalesce and spread lesions to coalesce and spread posteriorlyposteriorly in a rapid fashion.in a rapid fashion.The main symptom is rapid loss of vision.The retina shows The main symptom is rapid loss of vision.The retina shows typically a typically a white lesion with no haemorrhages or exudateswhite lesion with no haemorrhages or exudates..Treatment is often unsatisfactory and usually requires Treatment is often unsatisfactory and usually requires combination of combination of GanciclovirGanciclovir and and AciclovirAciclovir. The prognosis is very . The prognosis is very poor and retinal detachment is common. Resolution may leave poor and retinal detachment is common. Resolution may leave a white plaque with the appearance of a white plaque with the appearance of ““cracked mudcracked mud””..

ToxoplasmaToxoplasma RetinochoroiditisRetinochoroiditis

Toxoplasmosis Toxoplasmosis retinochoroiditisretinochoroiditis is an uncommon is an uncommon infection of the eye in AIDS. infection of the eye in AIDS. Ocular toxoplasmosis in HIV Ocular toxoplasmosis in HIV positive patients is different in appearance from positive patients is different in appearance from immunocompetentimmunocompetent patientspatients. Unlike in . Unlike in immunocompetentimmunocompetentpatients, HIV infected patients often have bilateral and patients, HIV infected patients often have bilateral and multifocalmultifocal disease associated with anterior uveitis and disease associated with anterior uveitis and vitritisvitritis but unlike but unlike immunocompetentimmunocompetent patients, in HIV patients, in HIV infected patients often have with no pigmented scars infected patients often have with no pigmented scars adjacent to the areas of retinal necrosis. Toxoplasmosis adjacent to the areas of retinal necrosis. Toxoplasmosis in in immunocompromisedimmunocompromised patients is not selfpatients is not self--limiting as it limiting as it is in is in imunocompetentimunocompetent patients.patients.

ToxoplasmaToxoplasma RetinochoroiditisRetinochoroiditis

When testing patients for antibodies to toxoplasmosis When testing patients for antibodies to toxoplasmosis both both IgGIgG and and IgMIgM levels may be raised, but in levels may be raised, but in immunocompromisedimmunocompromised patients these tests may be patients these tests may be negative. negative. Treatment in Treatment in immunocompromisedimmunocompromised patients consists in patients consists in the association of the association of sulphadiazinesulphadiazine or or clindamycinclindamycin, , pyrimethaminepyrimethamine and and folinicfolinic acid (triple therapy).acid (triple therapy).Long term maintenance treatment may be needed in Long term maintenance treatment may be needed in order to prevent relapses.order to prevent relapses.Often associated with Often associated with toxoplasmatoxoplasma lesions in the lesions in the Central Central Nervous System.Nervous System.

One week later, the lesion showing ring enhancement

MRI T1 showing an uniformly enhancing lesion in the midbrain

ImmunocompetentImmunocompetent ImmunocompromisedImmunocompromised

Syphilis RetinitisSyphilis Retinitis

There is a strong association between syphilis and There is a strong association between syphilis and HIV infection.HIV infection.It can manifest as a It can manifest as a retinitis with dense retinitis with dense vitritisvitritis, , retinal retinal vasculitisvasculitis, serous retinal detachment or , serous retinal detachment or neuroretinitisneuroretinitis, as well as other types of ocular , as well as other types of ocular involvement such as, conjunctivitis, anterior uveitis, involvement such as, conjunctivitis, anterior uveitis, cranial nerve palsies and optic neuritis.cranial nerve palsies and optic neuritis.Treatment consists in high dose of intravenous Treatment consists in high dose of intravenous Penicillin for 2 weeks.Penicillin for 2 weeks.

Candida Candida albicansalbicansendophthalmitisendophthalmitis

Infection with Infection with candidacandida albicansalbicans is rare. Candida is rare. Candida albicansalbicansis the commonest cause of fungal endophthalmitisis the commonest cause of fungal endophthalmitisAffected patients usually have a history of drug abuse Affected patients usually have a history of drug abuse or indwelling central linesor indwelling central linesIn the initial stages, floaters are the main symptom. As In the initial stages, floaters are the main symptom. As the condition progresses, the condition progresses, whitish whitish ““puffpuff--ballsballs”” and and vitreous strands develop. Later, similar infiltrates appear vitreous strands develop. Later, similar infiltrates appear in the in the choroidchoroid and retinaand retinaThe treatment depends on the severity of the ocular The treatment depends on the severity of the ocular involvement and systemic disease. The original foci involvement and systemic disease. The original foci should be removed. The drugs of choice are should be removed. The drugs of choice are AmphotericineAmphotericine B and B and FluconazolFluconazol

Candida Candida albicansalbicansendophthalmitisendophthalmitis

GlossaryGlossary

CD4CD4: Director of the immune response. When activated it : Director of the immune response. When activated it releases releases cytokines which in turn will activate the immune cytokines which in turn will activate the immune systemsystemCotton Wool SpotsCotton Wool Spots: Light: Light--coloured deposits in the retina coloured deposits in the retina secondary secondary to infarcts of the nerve fibre layer to infarcts of the nerve fibre layer HAARTHAART: Highly Active Antiretroviral Therapy: Highly Active Antiretroviral TherapyImmunoblogulinImmunoblogulin: Protein in charge of fighting foreign : Protein in charge of fighting foreign substances in substances in our body. our body. IgGIgG is the commonest type of is the commonest type of

immunoglobulin and immunoglobulin and IgMIgM is the earliest class is the earliest class of immunoglobulin.of immunoglobulin.PCRPCR: Polymerase Chain Reaction is a technique used to make : Polymerase Chain Reaction is a technique used to make

numerous copies of an specific portion of DNAnumerous copies of an specific portion of DNAVDRLVDRL: Venereal Disease Research Laboratory. The test : Venereal Disease Research Laboratory. The test becomes becomes negative after successful treatment of the disease. negative after successful treatment of the disease.

Documents

Systemic disease and the eye - International Council of Ophthalmology