Ocular Manifestations of Systemic Conditions

List of Conditions Covered

 

Hypertension

Systemic hypertension can directly affect the retina and optic nerve

 

Autoimmune Conditions

Many autoimmune conditions manifest in the eye. Proper identification of these signs can lead to a diagnosis

Diabetes

Diabetic retinopathy is amongst the leading causes of blindness worldwide

 

Thyroid Eye Disease

This is a potentially vision threatening autoimmune disease, with prominent exam findings

Sickle Cell Disease

Sickle Cell Retinopathy is a vision threatening complication of SCD

 

Pregnancy

Pregnancy can predispose individuals to specific eye diseases and worsen existing eye diseases

HIV/Immune Deficiency

Immune deficiency can lead to opportunistic infections in the eye

 

Neurologic Conditions

Giant cell arteritis, retinal artery/vein occlusions and increased intracranial pressure can be life threatening

Hypertension and the Eye

  • Prevalence: 4-7% of population have hypertensive retinopathy (HR)

  • Symptoms: Mostly asymptomatic, however, complications can cause loss of vision (see prognosis)

  • Diagnosis: Fundoscopic exam with characteristic findings (see video)

  • Treatment: Systemic blood pressure control

  • Screening guidelines: No consensus on standard guidelines

  • Prognosis:

    • Patients with HR 2-3x more likely to have a stroke compared to those who do not have HR

    • 70% 5 year survival if mild arteriolar narrowing

    • 6% 5 year survival if optic disc swelling

    • Loss of vision can occur secondary to aneurysm rupture or macular edema

Diabetes and the Eye

  • Prevalence: 28.5% of patients with diabetes have diabetic retinopathy (DR)

    • The leading cause of blindness in American adults

  • Symptoms: Initially asymptomatic until macular edema or hemorrhage can cause vision loss.

  • Diagnosis: Blood sugar levels, characteristic fundoscopic exam findings. Disease staging is essential for treatment and prognosis:

    • Non-proliferative DR: Various fundoscopic features such as cotton-wool spots and exudates

    • Proliferative DR: Characterized by neovascularization of the retinal vessels

    • Diabetic Macular Edema: Fluid leaks from new vessels into retina. Diagnosed with OCT imaging

  • Treatment: Blood sugar control, anti-VEGF injections (to control angiogenesis), laser photocoagulation (to reduce neovascularization), surgery to remove accumulated blood from hemorrhages

  • Screening guidelines: Dilated fundus exam performed by ophthalmologist at time of diabetes diagnosis and annually thereafter

  • Prognosis: Highly variable depending on blood sugar control, DR stage and treatment adherence. Irreversible blindness can occur if not appropriately managed

Sickle Cell Disease and the Eye

  • Prevalence: Highest in individuals with Hb SC or Hb S-thal genotype compared to patients with the more systemically involved Hb SS genotype (33% and 14% respectively versus 3%)

  • Symptoms: Eye pain, redness, decreased vision, floaters

  • Diagnosis: Evidence of sickle cell disease + characteristic conjunctival ‘comma vessel’ and retinal findings. Confirmatory studies may include retinal angiography and OCT

  • Treatment: Systemic management of disease and preventing neovascularization due to ischemia in the retina using anti-VEGF injections, lasers and/or surgical management of complications

  • Screening guidelines: Eye examination by an ophthalmologist twice a year

  • Prognosis: Permanent vision loss can occur from retinal detachments, hemorrhages and other complications; more likely in patients with advanced disease stages.

HIV and the Eye

  • Many of these pathologies can also manifest in patients that are profoundly immune-compromised due to other etiologies as well

  • 70% of patients with advanced AIDS experience a variety of ocular complications with declining CD4 counts. Following are some of the most important disease associations:

  • HIV Retinopathy: HIV virus is thought to directly damage the retina and cause microvasculopathy by an unknown mechanism. Treatment involves controlling the disease with HAART and ophthalmic observation

  • CMV Retinitis and Necrotizing Herpetic Retinopathies: Occurs in 15-40% of patients with AIDS and is characterized by retinal necrosis. Will see granular white dots with hemorrhages in retina. Treat with IV antiviral medications

  • Ocular Malignancies: AIDS can also predispose the eye to malignancies such as:

    • Kaposi Sarcoma

    • Squamous Cell Carcinoma

    • Non-Hodgkin’s Lymphoma

  • Neuro-ophthalmologic complications: Can include

    • Papilledema

    • Cranial nerve palsies

    • Visual Field Defects

    • Optic Neuritis

  • Opportunistic Infections: See list of common infections here

Autoimmune Conditions and the Eye

 

Systemic Lupus Erythematosus

  • Ocular involvement can precede systemic manifestation of SLE and predict disease severity

  • External Eye: Can have orbital involvement and keratoconjunctivitis sicca (dry, inflamed eye)

  • Anterior Segment: Episcleritis, scleritis, uveitis

  • Posterior Segment: Lupus retinopathy/choroidopathy

  • Neuro-ophthalmic: Optic Neuritis, visual field loss

  • SLE Treatment with hydroxychloroquine can cause retinal toxicity (see Medications section)

Rheumatoid Arthritis

  • Ocular involvement of RA may not be correlated with systemic disease severity and prompt identification is needed to prevent vision loss

  • External Eye: Keratoconjunctivitis sicca (dry, inflamed eye)

  • Anterior Segment: Episcleritis, scleritis, keratitis

 

Sarcoidosis

  • 50% of patients with sarcoidosis have ocular involvement

  • Common findings: Uveitis (30-60%) and conjunctival nodules (40%)

  • Other findings: Dry eye, episcleritis, scleritis, neuro-ophthalmic manifestations

  • Uveitis often precedes systemic findings

Thyroid Eye Disease

  • Prevalence: Is observed in up to 50% of patients with Graves Disease. Overall a rare disease however, with a prevalence of ~19 per 100,000

  • Symptoms: Exophthalmos (bulging eyes), restriction of eye movement, lid retraction, dry eyes with foreign sensation, lid edema, vision loss if severe due to compression of the optic nerve

  • Diagnosis: Clinical exam with characteristic signs (see video), Thyroid function tests and head CT/MRI imaging

  • Treatment:

    • Active inflammatory stage:

      • Reverse hyperthyroidism

      • Reduce ocular surface irritation (artificial tears, etc.)

      • Treat inflammation in orbit tissues (steroids, biologics, radiation, surgery)

      • Smoking cessation (improves inflammation and response to therapy)

    • Remission stage:

      • Correct any proptosis, eyelid or eye alignment issues that were caused by disease

  • Prognosis: Highly variable depending on disease severity. Risk of blindness due to optic nerve compression and damage to cornea due to exposure

Pregnancy and the Eye

  • The eye undergoes numerous physiologic changes during pregnancy, notably a transient change in refraction

  • Preeclampsia: 40-100% of patients have retinal vascular changes and can manifest with vision complaints.

    • Patients are predisposed to retinal detachments, especially if they had HELLP syndrome

  • Central Serous Chorioretinopathy: Is an eye disease characterized by fluid buildup in the retina, causing vision distortions. Occurs more commonly in late pregnancy

  • Diabetic Retinopathy: Pre-existing disease can worsen during pregnancy.

    • Screening guidelines: Pregnant women with background diabetic retinopathy should see their ophthalmologist at least once each trimester

Neurologic Conditions and the Eye

 

Giant Cell Arteritis

  • Epidemiology: More prevalent in women and in ages over 70

  • Symptoms:

    • Constitutional (fever, weight loss, night sweats),

    • Headache (unilateral, typically over temple)

    • Jaw claudication, jaw pain when chewing

    • Vision loss (unilateral)

    • Symptoms of polymyalgia rheumatica (50% association with GCA)

  • Diagnostics (ACR Criteria - need 3 for diagnosis):

    • Age >50

    • Headaches

    • Temporal artery abnormalities

    • ESR >50 mm/hr

    • Histopathological abnormalities

  • Treatment:

    • Urgent initiation of steroids

    • Urgent treatment can prevent permanent vision loss or other life threatening complications

Retinal Artery/Vein Occlusions

  • Epidemiology: Risk increases with age, and other systemic vasculopathic risk factors

  • Symptoms:

    • Sudden painless loss of vision in one eye -

      • Complete if central retinal artery or vein occlusion (CRAO and CRVO respectively) and RAPD present

      • Sudden field defect (scotomas) for branch retinal artery occlusion (BRAO)

      • Asymptomatic for branch retinal vein occlusion (BRVO)

  • Diagnostics:

    • Ophthalmic exam with characteristic findings (see here)

    • Need to evaluate cardiovascular/embolic/vasculitis risk factors

      • Carotid ultrasound

      • ECG

      • Inflammatory markers

  • Treatment:

    • Acutely, there is little evidence of specific treatment improving outcomes in RAO/RVO

    • Treatment geared at preventing future risk factors as well as managing ischemic complications in the eye

 

Increased Intracranial Pressure

  • Etiologies: Can be numerous, including

    • Idiopathic (pseudotumor cerebri)

    • Mass effect

    • Hydrocephalus

    • Inflammation

  • Ocular Symptoms:

    • Transient visual obscurations

    • Photopsias

    • Retrobulbar pain

    • Horizontal diplopia

    • Enlarged blind spots and visual field loss

  • Diagnostics: In addition to neurologic exam and imaging -

    • Papilledema on fundus exams

    • Absence of venous pulsations

    • 6th nerve palsies

    • Visual field defects

  • Treatment: Depends on correctly identifying the etiology

  • Complications: Untreated elevated ICP can lead to permanent visual field loss secondary to optic nerve compression and atrophy