Eye Manifestation in systemic disease พญ. พัชรินทร์ แซ่ลิ่ว

Common systemic disease Systemic hypertension Diabetic retinopathy AIDS Thyroid SJS Ocular toxicity from systemic drug Systemic steriod Antimalarial ; chloroquine Antituberculosis druds

Common systemic diseases affecting the eye Infectious Toxoplasmosis Toxocariasis TB Syphilis Leprosy HIV CMV Non-infectious Endocrine – diabetes, HT, thyroid Connective tissue disease – RA/ SLE Vasculitides (GCA) Sarcoidosis Behcet’s Disease Vogt Koyanagi Harada syndrome

Hypertensive retinopathy

Hypertensive Retinopathy Elevation of systemic BP -> focal and generalized constriction of retinal arterioles. Chronic effect of HT -> arteriolosclerotic changes; vascular thickening, arteriolar narrowing (reduction of A/V ratio).

Prolonged duration of high BP -> breakdown of inner blood-retinal barrier ; retinal hemorrhage, CWS, exudate. Acute/accelerate HT -> choriocapillaris infarction -> breakdown of outer blood-retinal barrier. Severe HT -> optic nerve involvemet.

Modified Scheie Classification of Hypertensive Retinopathy Stage 0 ; no visible retinal vascular abnormality. Stage 1 ; diffuse arteriolar narrowing, no focal constriction. Stage 2 ; obvious arteriolar narrowing with focal irregularity. Stage 3 ; arteriolar narrowing with retinal hemorrhage/exudate. Stage 4 ; previous abnormality with retinal edema, optic disc edema.

Grading of arteriolosclerotic changes Grade 1 ; broadening of light reflex from arteriole, minimal/no arteriovenous compression. Grade 2 ; broadening of light reflex and deflection of vein at arteriovenous crossing. Grade 3 ; copper wire appearance of arterioles, more arteriovenous compression. Grade 4 ; silver wire appearance of arterioles, severe arteriovenous crossing.

THYROID EYE DISEASE 1. Soft tissue involvement 2. Eyelid retraction 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 Conjunctival hyperaemia Superior limbic keratoconjunctivitis Chemosis

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

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 Abduction defect - less common Depression defect - uncommon Adduction defect - rare

Vit A deficiency

Connective tissue disease

Peripheral corneal involvement in rheumatoid arthritis Without inflammation With inflammation 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

Ocular manifestations of HIV infection

Ophthalmic Manifestations of HIV Infection AROUND THE EYE Molluscum Contagiosum Herpes Zoster Ophthalmicus Kaposi’s Sarcoma Conjunctival Squamous Cell Carcinoma Trichomegaly FRONT OF THE EYE Dry Eye Anterior Uveitis BACK OF THE EYE Retinal Microvasculopathy CMV Retinitis Acute Retinal Necrosis Progressive Outer Retinal Necrosis Toxoplasmosis Retinochoroiditis Syphilis Retinitis Candida albicans endophthalmitis NEURO-OPHTHALMIC

Ophthalmic manifestation in AIDS 70–80% of adult AIDS patients experienced an ocular complication Ophthalmic complications is lower in the pediatric age group All patients with HIV disease should undergo routine ophthalmologic examinations CD4+T Lymphocyte proved to be a reliable predictor of ocular complications

HIV retinopathy

CMV RETINITIS CMV is the most common cause of retinitis in those with AIDS Generally is a late manifestation, small percentage of patients, it may be the manifesting sign of AIDS. CD4 is reliable risk factor , almost cases CD4 < 50 cells/mm3

CMV Retinitis Introduction CMV Retinitis is the commonest intraocular ocular opportunistic infection seen in patients with AIDS Antibodies are found in almost 95% of adults, causing a trivial illness in immunocompetent adults, however severe immunosuppression causes viral reactivation and tissue invasive disease Pathogenesis Reactivation from extraocular sites leads to seeding in other sites such as the retina Epidemiology The number of newly diagnosed cases of CMVR has decreased since the introduction of the HAART Highly Active Antiretroviral Therapy

CMV Retinitis Clinical manifestations Patients may complain of minor visual symptoms such as floaters, flashing lights or mild blurred vision, or be totally asymptomatic. It presents with a wide range of clinical appearances. From cotton wool 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. Treatment The treatment of CMVR in patients with AIDS requires the use of specific antiviral agents, ganciclovir, foscarnet or cidovir in conjunction with 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. Intravitreal implants release the drug over a six-month period, achieving prolonged high intravitreal drug.

CMV RETINITIS three patterns of CMV retinitis hemorrhagic brush fire granular All of these may simultaneously occur in the same eye.

FIGURE 6  CMV retinitis exhibiting a brush-fire pattern in a patient with AIDS

Herpesvirus Common affect AIDS patients Ophthalmic finding include keratitis, panuveitis, vitritis, retinal arteritis, disc edema, necrotizing retinitis (ARN or PORN) Neuro-ophthalmic findings are uncommon Encephalitis is most common in CNS involvement

Acute retinal necrosis Progressive outer retinal necrosis viruses of the herpes family may also cause retinal infections in the patient with HIV, ARN consisting of severe vitreitis and a necrotizing retinitis seen in patients with or without immunosuppression. caused by both VZV and herpes simplex PORN limited to immunosuppressed patients more rapidly progressive retinitis minimal to no anterior chamber or vitreous reaction. causative agent  VZV

ARN/PORN

Drugs toxicity

Chloroquine toxicity Use for Tx of collagen-vas dis, malaria prophylaxis Toxic relate to duration, total dose, pt age Symptom: blurr vision, color vision loss, dark-adaptation deficit Macular change most typically: bull’s eye, paracentral scotoma Toxic occur: total accu dose > 300g (safe maintain < 250 mg daily) Early detection: important but difficult

Chloroquine toxicity Pt should: baseline ophthalmoscopy, fundus photo, VA, color, central VF (red) F/U q 6-12mo Recommend: cessation of drug at first sign of toxic Sometimes progress despite discont

CQ maculopathy Risk factors Dose Obesity Old age>60 yrs Retinal dz

2) Hydroxychloroquine(HCQ) -also used for Rx RA &SLE -can produce retinopathy identical to CQ but rare -VA ,paracentral scotoma &bull’s eye maculopathy -received above recommended daily dosage of 400 mg/d (or 6.5 MKD)