Ocular Manifestations of Systemic Disease

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Ocular Manifestations of Systemic Disease
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• Introduction

OCULAR MANIFESTATIONS
SYSTEMIC DISEASES
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CATEGORIES OF SYSTEMIC DISEASE

• Introduction

• Congenital
• Traumatic
• Vascular
• Neoplastic
• Autoimmune
• Idiopathic
• Infectious
• Metabolic/Endocrine
• Drugs/Toxins

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EYE EXAMINATION

• Introduction

• Vision
• External
• Pupils
• Relative afferent pupillary defect
• Motility
• Anterior segment exam
• Dilated ophthalmoscopy
• Visual fields

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CONGENITAL DISORDERS

• Congenital Disorders

• Down syndrome
• Marfan syndrome
• Myotonic dystrophy
• Tuberous sclerosis
• Neurofibromatosis
• Congenital metabolic disorders
• Lysosomal storage
• Carbohydrate metabolism

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NEUROFIBROMATOSIS NF1

• Congenital Disorders

• Café-au-lait spots

• Iris Lisch nodules

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• Traumatic Disorders

• Shaken baby syndrome multiple retinal hemorrhages

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SYSTEMIC HYPERTENSION FACTORS AFFECTING RETINAL
ARTERIOLES

• Vascular Disorders

• Severity
• Duration

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• Vascular Disorders

• Malignant hypertension

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Embolic Disease

• Vascular Disorders

• Sites of emboli to the
• ophthalmic circulation

• Cholesterol embolus
• (Hollenhurst plaque)

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• Vascular Disorders

• Left eye with superotemporal branch retinal
  artery occlusion

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CRAO

• Vascular Disorders

• Cherry red spot in CRAO

• Normal fundus

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AMAUROSIS FUGAX"Fleeting Blindness"

• Vascular Disorders

• Monocular dimming of vision
• Temporary arterial obstruction
• Sudden, transient, painless visual loss

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AMAUROSIS FUGAX A MULTIDISCIPLINARY EVALUATION

• Vascular Disorders

• Cardiovascular
• Cerebrovascular
• Ophthalmologic

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• Vascular Disorders

• Talc embol in IV drug abuser

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• Vascular Disorders

• Central retinal vein occlusion (CRVO)

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MIGRAINEVISUAL SYMPTOMS

• Vascular Disorders

• Scintillations
• Transient obscuration of vision
• Transient homonymous hemifield loss

Scintillations
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MIGRAINE TYPES

• Vascular Disorders

• With headache
• Classic
• Common
• Complicated
• Without headache
• Acephalgic

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MIGRAINE TREATMENT

• Vascular Disorders

• Prevent frequent attacks
• Examine for visual loss
• Consider discontinuing oral contraceptives

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BLOOD DYSCRASIASWITH OCULAR MANIFESTATIONS

• Vascular Disorders

• Hyperviscosity syndromes
• Thrombocytopenia
• Anemia, including sickle cell anemia

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HYPERVISCOSITY SYNDROMESOCULAR SYMPTOMS

• Vascular Disorders

• Amaurosis fugax
• Permanent visual loss

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• Vascular Disorders

• Hyperviscosity dilated retinal veins, retinal
  hemorrhages, and disc edema

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• Vascular Disorders

• Leukemia

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• Vascular Disorders

• Leukemic infiltration of the optic nerve

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SICKLE CELL RETINOPATHY

• Vascular Disorders

• HbSC disease (most common form)
• HbSS disease
• Sickle thalassemia

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SICKLE CELL ANEMIA

• Vascular Disorders

• Retinal arterial occlusions

• Sea fan neovascularization

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MALIGNANT NEOPLASMSINVOLVING THE EYE

• Neoplastic Disorders

• Primary ocular melanoma
• Large cell lymphoma
• Metastatic carcinoma

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METASTATIC CARCINOMA

• Neoplastic Disorders

• Most common intraocular malignancy in adults
• May be asymptomatic
• May produce decreased or distorted vision

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• Neoplastic Disorders

• Iris metastasis from lung carcinoma

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• Neoplastic Disorders

• Choroidal metastasis from breast cancer

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Ophthalmologists shouldmonitor ocular
metastasesat regular intervals.

• Neoplastic Disorders

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AUTOIMMUNE DISORDERS

• Autoimmune Disorders

• Connective tissue diseases
• Thyroid eye disease
• Myasthenia gravis

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CONNECTIVE TISSUE DISORDERS

• Autoimmune Disorders

• Dry eyes are the most common manifestation
  (keratoconjunctivitis sicca)
• Symptoms
• Burning
• Foreign-body sensation
• Photophobia

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SJÖGRENS SYNDROME

• Autoimmune Disorders

• Dry eyes
• Dry mouth
• ? Connective tissue disorder
• Specific antibodies

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DRY EYES TREATMENT

• Autoimmune Disorders

• Artificial tears
• Lubricating ointment at night
• Punctual occlusion
• Environmental modification
• Topical anti-inflammatory agents

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• Autoimmune Disorders

• Severe tear deficiency corneal ulceration and
  perforation

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ANKYLOSING SPONDYLITIS OCULAR SYMPTOMS

• Autoimmune Disorders

• Photophobia
• Redness
• Decreased vision

• If iritis, refer for evaluation and treatment.

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Topical corticosteroids may lead to serious
ocular complications.

• Autoimmune Disorders

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RHEUMATOID ARTHRITIS OCULAR MANIFESTATIONS

• Autoimmune Disorders

• Dry eyes
• Episcleritis
• Scleritis
• Corneal ulcers
• Uveitis

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• Autoimmune Disorders

• Episcleritis

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• Autoimmune Disorders

• Scleritis

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• Autoimmune Disorders

• Scleritis
  Necrotizing scleritis

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• Autoimmune Disorders

• Scleromalacia perforans

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• Autoimmune Disorders

• Peripheral corneal ulceration

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• Autoimmune Disorders

• Juvenile rheumatoid arthritis band keratopathy

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IRITIS IN JUVENILERHEUMATOID ARTHRITIS

• Autoimmune Disorders

• Few symptoms or signs
• Usually chronic
• Secondary cataract and glaucoma
• Refer for periodic ophthalmic screening.

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SYSTEMIC LUPUS ERYTHEMATOSUS OCULAR
MANIFESTATIONS

• Autoimmune Disorders

• Dry eyes
• Scleritis
• Peripheral corneal ulcers
• Retinopathy and optic neuropathy

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• Autoimmune Disorders

• Systemic lupus erythematosis Cotton wool spots
  and hypertensive retinopathy

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• Autoimmune Disorders

• Systemic lupus erythematosis optic nerve
  involvement

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POLYARTERITIS NODOSAOCULAR MANIFESTATIONS

• Autoimmune Disorders

• Dry eyes
• Corneal ulcers
• Scleritis
• Hypertensive retinopathy
• Retinal vasculitis

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SARCOIDOSIS

• Autoimmune Disorders

• More commonly affects African-Americans and
  Hispanics
• Ocular involvement in about 25 of patients

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SARCOIDOSIS

• Autoimmune Disorders

• Keratic precipitates in granulomatous uveitis

• Candlewax drippings in choroiditis

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Refer patients with sarcoidosis if ocular
involvement is suspected.Treatment may include
corticosteroids.

• Autoimmune Disorders

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GIANT CELL ARTERITIS SYMPTOMS

• Autoimmune Disorders

• Jaw claudication
• Headache
• Scalp tenderness
• Fever
• Weight loss
• Fatigue
• Myalgias
• Acute visual loss

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• Autoimmune Disorders

• Giant cell arteritis ischemic optic neuropathy

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• Autoimmune Disorders

• Giant cell arteritis third cranial nerve
  involvement

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GIANT CELL ARTERITIS DIAGNOSIS

• Autoimmune Disorders

• Clinical history
• STAT ESR and CRP
• Temporal artery biopsy
• Fluorescein angiogram

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GIANT CELL ARTERITIS MANAGEMENT

• Autoimmune Disorders

• Begin treatment immediately with high-dose
  corticosteroids daily
• Do not wait for results of temporal artery biopsy
• Ophthalmologist, PCP, and rheumatologist manage
  jointly

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THYROID OPHTHALMOPATHY

• Autoimmune Disorders

• Not always correlated with serum thyroid levels
• Can progress after thyroid function is normal

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SPECTRUM OF THYROID OPHTHALMOPATHY

• Autoimmune Disorders

• Eyelid retraction
• Exophthalmos (proptosis)
• Extraocular muscle dysfunction
• Corneal exposure
• Conjunctival erythema
• Optic nerve dysfunction

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THYROID OPHTHALMOPATHY OCULAR INVOLVEMENT

• Autoimmune Disorders

• Widened palpebral fissure

• Soft tissue involvement

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THYROID OPHTHALMOPATHY ADVANCED

• Autoimmune Disorders

• Proptosis

• Inferior rectus muscle restriction

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THYROID OPHTHALMOPATHY SEVERE

• Autoimmune Disorders

• Corneal damage

• Swollen optic disc

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THYROID OPHTHALMOPATHY COMPUTED TOMOGRAPHY OF
THE ORBIT

• Autoimmune Disorders

• Proptosis

• Enlarged extraocular muscles

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THYROID OPHTHALMOPATHY TREATMENT OF CONGESTIVE
PHASE

• Autoimmune Disorders

• Tear substitutes
• Corticosteroids
• Orbital irradiation or surgical decompression

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THYROID OPHTHALMOPATHY TREATMENT OF CICATRICIAL
PHASE

• Autoimmune Disorders

• Lid surgery
• Muscle surgery
• Orbital surgery

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MYASTHENIA GRAVIS SYSTEMIC INVOLVEMENT

• Autoimmune Disorders

• Refer suspects for neurologic evaluation.

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• Idiopathic Disorders

• Papilledema

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INTRACRANIAL HYPERTENSION CAUSES

• Idiopathic Disorders

• Brain tumor
• Meningitis
• Venous sinus thrombosis
• Hydrocephalus
• Idiopathic intracranial hypertension (pseudotumor
  cerebri)

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OPTIC NEURITIS

• Idiopathic Disorders

• May be initial manifestation of MS
• Acute, painful vision loss in one or both eyes
• Treat initial episode with intravenous
  methylprednisolone
• Interferon beta-1a may be of benefit

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AIDS OCULAR MANIFESTATIONS

• Infectious Disorders

• Dry eye
• Retinal cotton-wool spots
• CMV retinitis
• Kaposis sarcoma (eyelid or conjunctiva)

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• Infectious Disorders

• AIDS Cotton wool patches

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• Infectious Disorders

• AIDS cytomegalovirus (CMV) retinitis

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CMV RETINITIS THERAPY

• Infectious Disorders

• HAART (Highly Active Anti-Retroviral) Therapy
• IV ganciclovir
• IV foscarnet
• Intravitreal ganciclovir
• Implantable pellets of sustained-release
  ganciclovir into the vitreous

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CD4 COUNTS

• Infectious Disorders

• lt 100 cells/ml Opportunistic infections (eg,
  CMV retinitis)
• gt 100 cells/ml Other ocular infections (eg,
  syphilis)

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• Metabolic/Endocrine Disorders

• Diabetic retinopathy

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SYSTEMIC MEDICATIONS WITH SIGNIFICANT OCULAR
EFFECTS

• Drugs/Toxins

• Toxic Retinopathy
• Thioridazine
• Chloroquine
• Hydroxychloroquine
• Tamoxifen
• Toxic Optic Neuropathy
• Ethambutol
• Isoniazid
• Fluoroquinolones

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• Conclusion

OCULAR MANIFESTATIONS
SYSTEMIC DISEASES