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Unformatted text preview: Systemic disease and the eye the Deric De Wit Aldrin Khan Professor Lightman Common systemic diseases affecting the eye affecting Infectious Toxoplasmosis Toxocariasis TB Syphilis Leprosy HIV CMV Non-infectious Endocrine – diabetes, Endocrine thyroid thyroid Connective tissue Connective disease – RA/SLE/Wegeners/PAN/ RA/SLE/Wegeners/PAN/ Systemic sclerosis Vasculitides (GCA) Sarcoidosis Behcet’s Disease Vogt Koyanagi Harada Vogt syndrome syndrome Phakomatoses Phakomatoses DIABETIC RETINOPATHY 1. Adverse risk factors 2. Pathogenesis 3. Background diabetic retinopathy 4. Diabetic maculopathies • Focal • Diffuse • Ischaemic 5. Clinically significant macular oedema 6. Preproliferative diabetic retinopathy 7. Proliferative diabetic retinopathy Adverse Risk Factors 1. Long duration of diabetes 2. Poor metabolic control 3. Pregnancy 4. Hypertension 5. Renal disease 6. Other • • • • Obesity Hyperlipidaemia Smoking Anaemia Location of lesions in background diabetic retinopathy Signs of background diabetic retinopathy Microaneurysms usually temporal to fovea Hard exudates frequently arranged in clumps or rings Intraretinal dot and blot haemorrhages Retinal oedema seen as thickening on biomicroscopy Preproliferative diabetic retinopathy Signs • Cotton-wool spots • Venous irregularities • Dark blot haemorrhages • Intraretinal microvascular abnormalities (IRMA) Treatment - not required but watch for proliferative disease Proliferative diabetic retinopathy • • Affects 5-10% of diabetics IDD at increased risk (60% after 30 years) Neovascularization Flat or elevated Severity determined by comparing with area of disc • • Neovascularization of disc = NVD eovascularization elsewhere = NVE N Laser panretinal photocoagulation • Initial treatment is 2000-3000 burns • Area covered by complete PRP • • Spot size (200-500 µ m) depends on contact lens magnification Gentle intensity burn (0.10-0.05 sec) • Follow-up 4 to 8 weeks Retinal Vein Occlusion Retinal 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 Occlusion Retinal 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 retraction 3. Proptosis 4. Optic neuropathy 5. Restrictive myopathy Soft tissue involvement Periorbital and lid swelling Chemosis Conjunctival hyperaemia Superior limbic keratoconjunctivitis Signs of eyelid retraction Occurs in about 50% • Bilateral lid retraction • No associated proptosis • Bilateral lid retraction • Bilateral proptosis • Unilateral lid retraction • Unilateral proptosis • Lid lag in downgaze Proptosis • Occurs in about 50% • Uninfluenced by treatment of hyperthyroidism Axial and permanent in about 70% May be associated with choroidal folds Treatment options • 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 Restrictive myopathy • Occurs in about 40% • Due to fibrotic contracture Elevation defect - most common Depression defect - uncommon Abduction defect - less common Adduction defect - rare SARCOIDOSIS SARCOIDOSIS Idiopathic multisystem disorder Characterised by non-caseating Characterised granulomata granulomata More common in women 20-50 yrs More common in blacks and Asians ? Related to mycobacteria SARCOIDOSIS SARCOIDOSIS Systemic Involvement Lung lesions – 95% Thoracic lymph nodes Thoracic – 50% 50% Skin lesions – 30% → Skin Eyes – 30% SARCOIDOSIS SARCOIDOSIS Ocular Involvement Anterior segment Anterior lesions (30%) lesions Conjunctival granuloma Lacrimal gland Lacrimal involvement/dry eye involvement/dry Acute or chronic uveitis Acute → KPs described as KPs ‘mutton fat’ because they are large and greasy are SARCOIDOSIS SARCOIDOSIS Ocular Involvement Posterior segment Posterior lesions (20%) lesions Patchy venous sheathing Cellular infiltrate around Cellular vessels vessels Chorioretinal Chorioretinal granulonmas granulonmas Vasculitis including Vasculitis occlusive causing:occlusive Neovascularisation Infiltrate in vitreous Infiltrate (vitritis) including cell clumps (snowballs) clumps SARCOIDOSIS SARCOIDOSIS Ocular Involvement Sheathing of the Sheathing retinal veins retinal Fluorescein Fluorescein angiography showing leakage and staining at sites of sheathing at SARCOIDOSIS SARCOIDOSIS Granuloma in Fundus Retinal and pre-retinal Choroidal Choroidal SARCOIDOSIS SARCOIDOSIS Granuloma in Fundus Optic nerve head Optic granuloma granuloma Normal optic nerve Normal head head SARCOIDOSIS SARCOIDOSIS Systemic Signs Lupus pernio affecting the nose – a chronic progressive cutaneous sarcoid that most commonly affects face and ears affects SARCOIDOSIS SARCOIDOSIS Systemic signs Facial palsy Salivary gland Salivary enlargement enlargement SARCOIDOSIS SARCOIDOSIS Systemic signs Hilar adenopathy on Hilar chest x-ray chest Lung infiltrate Erythema nodosum Arthritis SARCOIDOSIS SARCOIDOSIS Investigations (1) CXR – to detect pulmonary signs pulmonary Bilateral hilar lymphadenopathy Pulmonary mottling SARCOIDOSIS SARCOIDOSIS Investigations (2) Serum angiotensin-converting enzyme Serum (ACE) – elevated in active sarcoidosis (ACE) Mantoux test – caution in patients who Mantoux have had BCG vaccination. Test may be negative negative Lung function tests SARCOIDOSIS SARCOIDOSIS Investigations (3) Gallium scan showing increased uptake in the lacrimal and parotid glands and pulmonary regions in a patient with active sarcoidosis sarcoidosis SARCOIDOSIS SARCOIDOSIS Treatment Systemic steroids may be necessary in patients with posterior segment disease where vision is threatened, especially if optic nerve is involved optic 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 • Café-au-lait spots Increase in size and number throughout Appear during first year of life 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 • • Appear during childhood Large and ill-defined • May be associated with overgrowth of overlying skin Skeletal defects in NF-1 • Facial hemiatrophy • • Mild head enlargement - uncommon Other - scoliosis, short stature, thinning of long bones Orbital lesions in NF-1 Optic nerve glioma in about 15% Spheno-orbital encephalocele • Sagittal MRI scan of optic nerve glioma xial CT scan of congenital absence of •A invading hypothalamus left greater wing of sphenoid bone • Glioma may be unilateral or bilateral Causes pulsating proptosis without bruit • Eyelid neurofibromas in NF-1 Nodular May cause mechanical ptosis Plexiform May be associated with glaucoma Intraocular lesions in NF-1 Lisch nodules Congenital ectropion uveae Very common - eventually present ncommon - may be associated U in 95% of cases with glaucoma Choroidal naevi Retinal astrocytomas Common - may be multifocal Rare - identical to those seen in tuberous sclerosis and bilateral Ocular features of NF-2 Very common presenile cataract - Common - combined hamartomas of RPE and retina Tuberous sclerosis (Bourneville disease) • • 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) Shagreen patches • • Diffuse thickening over lumbar region Present in 40% Systemic hamartomas in tuberous sclerosis Astrocytic cerebral hamartomas Visceral and subungual hamartomas • Slow-growing periventricular tumours • Usually asymptomatic and • May cause hydrocephalus, epilepsy and innocuous • Kidneys (angiomyolipoma), heart mental retardation (rhabdomyoma) Retinal astrocytomas in tuberous scleritis • • Innocuous tumour present in 50% of patients May be multiple and bilateral Early Semitranslucent nodule White plaque Advanced Dense white tumour Mulberry-like tumour Systemic features of v-H-L syndrome Autosomal dominant CNS Haemangioblastoma Visceral tumours MRI of spinal cord tumour • Tumours - renal carcinoma and phaeochromocytoma • Cysts - kidneys, liver, pancreas, epididymis, ovary and lungs Angiogram of cerebellar• Polycythaemia tumour Retinal capillary haemangioma in v-H-L syndrome • • Vision-threatening tumour present in 50% of patients May be multiple and bilateral Early Tiny lesion between Small red nodule arteriole and venuole Advanced Associated dilatation and Round orange-red massortuosity of feeder vessels t Systemic features of Sturge-Weber syndrome Naevus flammeus • • Congenital, does not blanche• with pressure Associated with ipsilateral • glaucoma in 30% of cases Meningeal haemangioma CT scan showing left parietal haemangioma Complications - mental handicap, epilepsy and hemiparesis Ocular features of Sturge-Weber syndrome Glaucoma Buphthalmos in 60% May be associated with episcleral haemangioma Diffuse choroidal haemangioma Normal eye Affected eye Peripheral corneal involvement in rheumatoid arthritis Without inflammation • Chronic and asymptomatic • Circumferential thinning with intact epithelium (‘contact lens cornea’) With inflammation • Acute and painful • Circumferential ulceration and infiltration Treatment - systemic steroids and/or cytotoxic drugs Peripheral corneal involvement in egener 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 GIANT CELL ARTERITIS GIANT (Temporal or Cranial Arteritis) Idiopathic vasculitis Same disease spectrum as polymyalgia Same rheumatica rheumatica Mainly women 65-80 years old Medium and large arteries in head & neck Medium involved involved GIANT CELL ARTERITIS GIANT Presentation Headache Scalp tenderness Thickened temporal Thickened arteries arteries Jaw claudication Acute visual loss Weight loss, anorexia, Weight fever, night sweats, malaise & depression malaise GIANT CELL ARTERITIS GIANT Ocular Complications Transient monocular Transient visual loss (amaurosis fugax) fugax) Visual loss due to Central retinal artery Central occlusion (CRAO) or Anterior ischaemic Anterior optic neuropathy (AION) (AION) Visual field defects GIANT CELL ARTERITIS GIANT Management ESR if suspected Start high dose steroids immediately to Start prevent stroke or second eye involvement prevent Temporal artery biopsy within a week of Temporal starting steroids starting GIANT CELL ARTERITIS Temporal Artery Biopsy Temporal Arteries have skip Arteries lesions lesions ultrasound/Doppler may ultrasound/Doppler help identify involved areas areas If positive, confirms If diagnosis – helpful in management of future disease disease If negative, doesn’t If exclude diagnosis, but need to think about an alternative diagnosis alternative GIANT CELL ARTERITIS GIANT Histopathology Granulomatous cell Granulomatous infiltration infiltration Giant cells Disruption of internal Disruption elastic lamina elastic Proliferation of intima Occlusion of lumen GIANT CELL ARTERITIS GIANT Treatment Intravenous and oral steroids – prolonged Intravenous course of steroids often necessary course Ocular manifestations of HIV infection HIV Introduction Introduction AIDS is an infectious disease caused by the gradual AIDS decrease in CD4+ T lymphocytes causing CD4+ subsequent opportunistic infections and neoplasia. It is a blood borne and sexually transmitted infection caused by the HIV (Human Immunodeficiency Virus) caused Approximately 36 million persons around the world Approximately are infected. Up to 70% of patients infected with HIV will develop some form of ocular involvement, ie: direct infection by HIV,opportunistic infections and neoplasia. neoplasia. HIV infection progresses though different phases HIV different Ophthalmic Manifestations of HIV Infection Ophthalmic AROUND THE EYE Molluscum Contagiosum Herpes Zoster Ophthalmicus Kaposi’s Sarcoma Conjunctival Squamous Squamous Cell Carcinoma Trichomegaly FRONT OF THE EYE Dry Eye Anterior Uveitis BACK OF THE EYE Retinal Microvasculopathy Retinal Microvasculopathy CMV Retinitis Acute Retinal Necrosis Progressive Outer Retinal Necro Toxoplasmosis Toxoplasmosis Retinochoroiditis Retinochoroiditis Syphilis Retinitis Candida albicans Candida albicans endophthalmitis NEURO-OPHTHALMIC Molluscum Contagiosum Molluscum Molluscum contagiosum is a Molluscum viral infection of the skin. viral Affects up to 20% of Affects symptomatic HIV infected patients. patients. Clinically appears like painless, Clinically small, umbilicated nodules, which produce a waxy discharge when pressured. discharge Treatment consists on excision Treatment of the lesion, curettage or cryotherapy cryotherapy Herpes Zoster Ophthalmicus Herpes Due to the reactivation of a latent infection by Varicella Due Zoster Virus in the dorsal root of trigeminal nerve ganglion. ganglion. It manifests with a maculo-papulo-vesicular rash which It often is preceded by pain. Usually involves the upper lid and does not cross the midline Treatment consists on oral Aciclovir 800mg 5 times /day. In immunocompromised patients Aciclovir is given intravenously for two weeks. Ocular manifestations such as anterior uveitis, are treated with topical steroids and mydriatics. and Kaposi’s Sarcoma Kaposi’s Kaposi’s sarcoma is a vascular neoplasm which is almost Kaposi’s exclusively seen in patients with AIDS. exclusively KS is the commonest anterior segment lesion seen in AIDS; KS appears as a violaceous non-tender nodule on the eyelid or conjunctiva. conjunctiva. Typically KS involves only the skin but when there is a Typically reduced CD4 count it can progress rapidly to other sites such as the gastrointestinal tract and CNS such Treatment of ocular adnexal KS may be necessary for Treatment cosmesis and to relieve functional difficulties. The mainstay of treatment is radiotherapy. Other options include cryotherapy or chemotherapy. cryotherapy Conjunctival Squamous Cell Carcinoma Conjunctival Squamous cell carcinoma (SCC) is the third most Squamous common neoplasm associated to HIV infection. This may be due to an interaction between HIV, sunlight and Human Papilloma Virus infection. Human SCC appears as a pink, gelatinous growth, usually in the SCC interpalpebral area. Often an engorged blood vessel interpalpebral feeding the tumour is seen. It may extend onto the cornea, but deep invasion and metastasis are rare. The treatment of choice is local excision and cryotherapy but the presence of orbital invasion is an indication of exenteration exenteration Trichomegaly Trichomegaly Trichomegaly or Trichomegaly hypertrichosis is an exaggerated growth of the eye lashes found in the later stages of the disease disease The cause is not known When symptomatic or for When cosmetic reasons the eyelashes can be trimmed or plucked trimmed Dry Eye Dry Sicca syndrome is Sicca frequent among patients with HIV infection infection Patients complain of Patients burning uncomfortable red eyes. red There are several There causes of dry eye in HIV infection from blepharitis to destruction of the lacrimal glands. lacrimal Treatment is with tear Treatment supplements Anterior Uveitis Anterior HIV related anterior uveitis can be: be: Direct manifestation of the Direct human immunodeficiency virus infection virus autoimmnune in origin drug induced ie: rifabutin, drug secondary to direct toxic effect upon the noneffect pigmented epithelium of the pigmented ciliary body Any of the different infections Any associated with AIDS, ie: Herpes Zoster Virus, Herpes Simplex Virus, Rifabutin induced anterior uveitis Rifabutin Retinal microvasculitis Retinal Retinal microvasculopathy occurs in more than half of the Retinal patients with HIV patients It is seen as transient cotton wool spots (CWS), intra-retinal It haemorrhages and microaneurysm, which occurs in 50-70% of patients. It is usually asymptomatic. patients. It has an unclear pathogenesis, but it is thought to be HIV It infection of retinal vascular cells. infection In an otherwise healthy individual the presence of CWS, should In be differentiated from other forms of retinopathy, such as diabetic or hypertensive retinopathy. Serological test for HIV will confirm the diagnosis confirm Treatment is based in delaying the progression of the disease Treatment associated with HIV associated Cotton Wool Spots Cotton CMV Retinitis CMV Introduction CMV Retinitis is the commonest intraocular ocular opportunistic infection CMV seen in patients with AIDS seen Antibodies are found in almost 95% of adults, causing a trivial illness in Antibodies immunocompetent adults, however severe immunosuppression causes viral reactivation and tissue invasive disease viral Pathogenesis Reactivation from extraocular sites leads to seeding in other sites such Reactivation as the retina as Epidemiology The number of newly diagnosed cases of CMVR has decreased since The the introduction of the HAART the Highly Active Antiretroviral Therapy Highly Active Antiretroviral Therapy CMV Retinitis CMV Clinical manifestations Patients may complain of minor visual symptoms such as floaters, Patients flashing lights or mild blurred vision, or be totally asymptomatic. flashing It presents with a wide range of clinical appearances. From cotton wool It spots which may look like HIV Retinopathy to confluent areas of full thickness retinal necrosis and vasculitis. CMVR can progress in a “brushfire” pattern from the active edge of an active lesion. The retinal vessels in an affected area show attenuation, becoming ghost vessels eventually. eventually. Treatment The treatment of CMVR in patients with AIDS requires the use of specific The antiviral agents, ganciclovir, foscarnet or cidovir in conjunction with HAART. HAART. These treatments can be administered orally, intravenously or intravitreally. Systemic treatment has the advantage of treating infection elsewhere in the body as well as the other eye but has the disadvantages of systemic side effects. disadvantages CMV Retinitis CMV Acute Retinal Necrosis Acute ARN is a confluent peripheral whitening of the retina with ARN marked vitritis and blood vessel closure. Optic neuritis and retinal detachment are frequent complications. and ARN is usually due to Varicella-Zoster infection, but it can ARN also be caused by Herpes Simplex virus or Cytomegalovirus. Cytomegalovirus. Initially described in the immunocompetent, it has also Initially been described in the immunosuppressed. been The diagnosis is mainly clinical and is confirmed by PCR The assays on vitreous samples. assays Patients are treated with high doses of intravenous Patients aciclovir or famciclovir, combined with laser treatment to prevent retinal detachment. prevent Acute Retinal Necrosis Acute Progressive Outer Retinal Necrosis Necrosis (Varicella-Zoster Retinitis) PORN is a devastating viral retinitis caused by Varicella-Zoster PORN virus, without vitritis or retinal vasculitis. virus, The retinitis can be located anywhere but it is common for the The lesions to coalesce and spread posteriorly in a rapid fashion. lesions The main symptom is rapid loss of vision.The retina shows The typically a white lesion with no haemorrhages or exudates. typically Treatment is often unsatisfactory and usually requires Treatment combination of Ganciclovir and Aciclovir. The prognosis is very poor and retinal detachment is common. Resolution may leave a white plaque with the appearance of “cracked mud”. white Toxoplasma Retinochoroiditis Toxoplasma Toxoplasmosis retinochoroiditis is an uncommon Toxoplasmosis infection of the eye in AIDS. Ocular toxoplasmosis in HIV positive patients is different in ap Ocular immunocompetent patients. Unlike in immunocompetent Unlike patients, HIV infected patients often have bilateral and multifocal disease associated with anterior uveitis and vitritis but unlike immunocompetent patients, in HIV infected patients often have with no pigmented scars adjacent to the areas of retinal necrosis. Toxoplasmosis in immunocompromised patients is not self-limiting as it is in imunocompetent patients. is Toxoplasma Retinochoroiditis Toxoplasma When testing patients for antibodies to toxoplasmosis When both IgG and IgM levels may be raised, but in immunocompromised patients these tests may be negative. Treatment in immunocompromised patients consists in the association of sulphadiazine or clindamycin, pyrimethamine and folinic acid (triple therapy). pyrimethamine Long term maintenance treatment may be needed in order to prevent relapses. order Often associated with toxoplasma lesions in the Central Often Nervous System. Nervous MRI T1 showing an uniformly enhancing lesion in the midbrain One week later, the lesion showing ring enhancement Immunocompetent Immunocompetent Immunocompromised Syphilis Retinitis Syphilis There is a strong association between syphilis and There HIV infection. HIV It can manifest as a retinitis with dense vitritis, It retinal vasculitis, serous retinal detachment or neuroretinitis, as well as other types of ocular involvement such as, conjunctivitis, anterior uveitis, cranial nerve palsies and optic neuritis. cranial Treatment consists in high dose of intravenous Treatment Penicillin for 2 weeks. Penicillin Candida albicans endophthalmitis endophthalmitis Infection with candida albicans is rare. Candida albicans Infection is the commonest cause of fungal endophthalmitis is Affected patients usually have a history of drug abuse or indwelling central lines or In the initial stages, floaters are the main symptom. As In the condition progresses, whitish “puff-balls” and vitreous strands develop. Later, similar infiltrates appear in the choroid and retina in The treatment depends on the severity of the ocular The involvement and systemic disease. The original foci should be removed. The drugs of choice are Amphotericine B and Fluconazol Amphotericine Candida albicans endophthalmitis endophthalmitis Glossary Glossary CD4: Director of the immune response. When activated it Director releases cytokines which in turn will activate the immune system system Cotton Wool Spots: Light-coloured deposits in the retina Light-coloured secondary to infarcts of the nerve fibre layer HAART: Highly Active Antiretroviral Therapy HAART Immunoblogulin: Protein in charge of fighting foreign Protein substances in our body. IgG is the commonest type of immunoglobulin and IgM is the earliest class IgM of immunoglobulin. of PCR: Polymerase Chain Reaction is a technique used to make Polymerase numerous copies of an specific portion of DNA numerous VDRL: Venereal Disease Research Laboratory. The test Venereal becomes negative after successful treatment of the disease. ...
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