Uveitis

Uveitis is a serious inflammatory condition affecting the uvea, the middle layer of the eye that includes the iris, ciliary body, and choroid. This condition can cause significant vision problems and, if left untreated, may lead to permanent vision loss. Understanding the symptoms, recognizing the need for prompt medical attention, and knowing the available treatment options are crucial for preserving sight and maintaining eye health.

Overview

Uveitis refers to a group of inflammatory diseases that affect the uvea, the pigmented middle layer of the eye situated between the sclera (white outer layer) and the retina (inner layer). The uvea consists of three parts: the iris (colored part of the eye), the ciliary body (muscle that controls lens shape), and the choroid (blood vessel layer that nourishes the retina).

This condition can occur at any age but most commonly affects people between 20 and 50 years old. Uveitis accounts for approximately 5-15% of all cases of blindness in developed countries, making it a significant cause of visual impairment. The inflammation can be acute (sudden onset, lasting weeks to months) or chronic (persistent, lasting months to years), and may affect one or both eyes.

Uveitis is classified by location: anterior uveitis (affecting the iris and ciliary body), intermediate uveitis (affecting the vitreous and peripheral retina), posterior uveitis (affecting the choroid and retina), and panuveitis (affecting all parts of the uvea). Each type has distinct characteristics, causes, and treatment approaches, but all forms require prompt medical attention to prevent vision-threatening complications.

Symptoms

Uveitis symptoms can vary depending on the type and severity of inflammation. Some forms may develop gradually with subtle symptoms, while others can present suddenly with severe discomfort and vision changes.

Primary Visual Symptoms

  • Eye redness - Particularly around the iris (conjunctival injection)
  • Eye pain - Often described as deep, aching pain
  • Blurred vision - Decreased visual clarity and sharpness
  • Light sensitivity - Discomfort or pain when exposed to light
  • Floaters - Dark spots or specks that move across the field of vision
  • Vision loss - Partial or complete loss of sight in affected eye

Type-Specific Symptoms

Anterior Uveitis (Most Common)

  • Sudden onset: Symptoms develop rapidly over hours to days
  • Severe photophobia: Extreme sensitivity to light
  • Ciliary flush: Ring of redness around the cornea
  • Small pupil: Constricted pupil due to inflammation
  • Tearing: Excessive tear production
  • Brow ache: Deep pain above the eyebrow

Intermediate Uveitis

  • Gradual onset: Symptoms develop slowly over weeks
  • Floaters: Primary complaint in many patients
  • Minimal pain: Usually painless or mild discomfort
  • Mild redness: Less prominent than anterior uveitis
  • Peripheral vision loss: May affect side vision

Posterior Uveitis

  • Painless: Often no eye pain or discomfort
  • Vision changes: Blurred or distorted vision
  • Blind spots: Scotomas in visual field
  • Central vision loss: If macula is affected
  • Color vision changes: Difficulty distinguishing colors

Panuveitis

  • Combined symptoms: Features of all types of uveitis
  • Severe vision loss: Often more pronounced
  • Systemic symptoms: May have fever, malaise
  • Both eyes affected: Bilateral involvement common

Associated Symptoms

  • Headache: Often accompanies eye pain
  • Nausea: From severe pain or increased eye pressure
  • Decreased night vision: Difficulty seeing in low light
  • Halos around lights: Especially with corneal edema
  • Eye fatigue: Tiredness with visual tasks

Complications Signs

  • Glaucoma symptoms: Severe eye pain, rainbow halos, nausea
  • Cataract symptoms: Progressive vision clouding
  • Macular edema: Central vision distortion or loss
  • Retinal detachment: Sudden onset of flashing lights, curtain-like vision loss

Causes

Uveitis can result from various causes, including autoimmune diseases, infections, trauma, and sometimes occurs without an identifiable cause (idiopathic).

Autoimmune and Inflammatory Causes

  • Ankylosing spondylitis: Inflammatory arthritis affecting the spine
  • Behçet's disease: Multisystem inflammatory disorder
  • Sarcoidosis: Granulomatous inflammatory disease
  • Inflammatory bowel disease: Crohn's disease and ulcerative colitis
  • Vogt-Koyanagi-Harada disease: Autoimmune condition affecting eyes and skin
  • Sympathetic ophthalmia: Autoimmune reaction following eye trauma
  • Juvenile idiopathic arthritis: Childhood arthritis with eye involvement
  • Psoriatic arthritis: Arthritis associated with psoriasis

Infectious Causes

Viral Infections

  • Herpes simplex virus: Can cause anterior or posterior uveitis
  • Varicella-zoster virus: Associated with shingles
  • Cytomegalovirus: Especially in immunocompromised patients
  • Epstein-Barr virus: Less common cause

Bacterial Infections

  • Tuberculosis: Can cause granulomatous uveitis
  • Syphilis: Both primary and secondary syphilis
  • Lyme disease: Tick-borne bacterial infection
  • Bartonella: Cat scratch disease

Fungal Infections

  • Histoplasmosis: Fungal infection common in certain geographic areas
  • Candida: Yeast infection, often in immunocompromised patients
  • Aspergillus: Mold infection

Parasitic Infections

  • Toxoplasmosis: Most common infectious cause of posterior uveitis
  • Toxocariasis: Roundworm infection
  • Onchocerciasis: River blindness

Other Causes

  • Trauma: Penetrating or blunt eye injury
  • Surgery: Complication of eye surgery
  • Medications: Drug-induced uveitis (bisphosphonates, antibiotics)
  • Malignancy: Lymphoma or leukemia affecting the eye
  • Masquerade syndromes: Conditions mimicking uveitis

Idiopathic Uveitis

In approximately 50% of cases, no specific cause can be identified despite thorough investigation. These cases are classified as idiopathic uveitis and are treated based on the pattern and severity of inflammation.

Risk Factors

Several factors can increase the likelihood of developing uveitis:

Demographics

  • Age: Most common between ages 20-50
  • Gender: Some types more common in women (Vogt-Koyanagi-Harada)
  • Race/ethnicity: Certain conditions more prevalent in specific populations
  • Geographic location: Some infectious causes are region-specific

Medical Conditions

  • Autoimmune diseases: Rheumatoid arthritis, lupus, multiple sclerosis
  • Inflammatory conditions: Inflammatory bowel disease, psoriasis
  • HLA-B27 positive: Genetic marker associated with anterior uveitis
  • Immunodeficiency: HIV/AIDS, cancer treatment
  • Previous uveitis: History of eye inflammation

Environmental and Lifestyle Factors

  • Infections: Recent bacterial, viral, or parasitic infections
  • Trauma history: Previous eye injury or surgery
  • Occupational exposures: Certain chemicals or infectious agents
  • Travel history: Exposure to endemic infections
  • Animal contact: Pets, livestock, or wild animals

Genetic Factors

  • HLA associations: Specific genetic markers linked to uveitis risk
  • Family history: Genetic predisposition to autoimmune diseases
  • Ethnic background: Some conditions more common in certain ethnicities

Medication-Related Risks

  • Immunosuppressive drugs: Increase infection risk
  • Bisphosphonates: Can trigger inflammatory uveitis
  • Antibiotics: Rare cause of drug-induced uveitis
  • Vaccines: Very rare trigger for uveitis

Diagnosis

Diagnosing uveitis requires a comprehensive eye examination and often extensive testing to determine the underlying cause and guide treatment.

Clinical Examination

Ophthalmologic Evaluation

  • Visual acuity testing: Measure sharpness of vision
  • Slit-lamp examination: Detailed examination of anterior eye structures
  • Pupil examination: Check pupillary responses and shape
  • Intraocular pressure: Measure eye pressure
  • Dilated fundoscopy: Examination of retina and vitreous
  • Gonioscopy: Examine drainage angle of the eye

Inflammation Assessment

  • Cell and flare grading: Quantify inflammation in aqueous humor
  • Keratic precipitates: White blood cell deposits on cornea
  • Posterior synechiae: Adhesions between iris and lens
  • Vitreous haze: Cloudiness in vitreous cavity
  • Vitreous cells: Inflammatory cells in vitreous

Imaging Studies

  • Fluorescein angiography: Evaluate retinal blood vessels and leakage
  • Optical coherence tomography (OCT): High-resolution retinal imaging
  • Ultrasound: When media opacity prevents direct visualization
  • Fundus photography: Document retinal changes
  • ICG angiography: Evaluate choroidal circulation

Laboratory Testing

Initial Screening

  • Complete blood count: Check for systemic infection or inflammation
  • ESR and CRP: Markers of systemic inflammation
  • ANA (antinuclear antibody): Screen for autoimmune diseases
  • HLA-B27: Genetic marker for certain types of uveitis
  • Chest X-ray: Screen for sarcoidosis or tuberculosis

Specific Testing

Based on clinical presentation and initial results:

  • VDRL/RPR: Syphilis screening
  • Toxoplasma antibodies: If posterior uveitis suspected
  • Tuberculin skin test or IGRA: Tuberculosis screening
  • ACE level: If sarcoidosis suspected
  • Lyme titers: In endemic areas

Advanced Testing

  • Aqueous or vitreous tap: Direct sampling for PCR or culture
  • Tissue biopsy: Rarely needed for diagnosis
  • Genetic testing: For specific hereditary conditions
  • Imaging studies: CT or MRI if systemic disease suspected

Classification

  • Anatomical location: Anterior, intermediate, posterior, or panuveitis
  • Course: Acute, recurrent, or chronic
  • Laterality: Unilateral or bilateral
  • Onset: Sudden or insidious
  • Granulomatous vs. non-granulomatous: Based on inflammatory cell type

Treatment Options

Treatment of uveitis aims to control inflammation, preserve vision, and address underlying causes. The approach varies based on the type, severity, and suspected etiology of the uveitis.

Corticosteroid Therapy

Topical Corticosteroids

  • First-line for anterior uveitis: Prednisolone acetate 1% or dexamethasone
  • Dosing: Initially every 1-2 hours, then taper based on response
  • Duration: Usually 6-8 weeks with gradual taper
  • Monitoring: Regular IOP checks due to steroid-induced glaucoma risk

Periocular Injections

  • Sub-Tenon's injection: Triamcinolone acetonide for posterior segment inflammation
  • Retrobulbar injection: For more severe cases
  • Advantages: Higher drug concentration in eye, fewer systemic effects
  • Frequency: Every 3-6 months as needed

Intravitreal Injections

  • Direct delivery: Triamcinolone or dexamethasone implants
  • Sustained release: Ozurdex (dexamethasone) implant
  • Long-acting: Iluvien (fluocinolone acetonide) implant
  • Indications: Macular edema, posterior uveitis

Systemic Corticosteroids

  • Severe uveitis: When topical therapy insufficient
  • Bilateral disease: Both eyes affected
  • Sight-threatening complications: Rapid intervention needed
  • Dosing: Prednisone 1-2 mg/kg/day initially, then taper
  • Monitoring: Blood sugar, blood pressure, bone density

Immunosuppressive Therapy

Conventional Immunosuppressants

  • Methotrexate: First-line steroid-sparing agent
  • Azathioprine: Alternative first-line option
  • Mycophenolate mofetil: Effective for posterior uveitis
  • Cyclosporine: Calcineurin inhibitor
  • Tacrolimus: Alternative calcineurin inhibitor

Biologic Agents

  • Adalimumab (Humira): FDA-approved for non-infectious uveitis
  • Infliximab: TNF-alpha inhibitor for severe cases
  • Rituximab: B-cell depleting agent
  • Tocilizumab: IL-6 receptor antagonist
  • Interferon-alpha: For Behçet's disease-related uveitis

Specific Treatments

Infectious Uveitis

  • Antiviral agents: Acyclovir, valacyclovir for viral causes
  • Antibiotics: Specific therapy based on organism
  • Antifungal agents: For fungal infections
  • Antiparasitic drugs: For toxoplasmosis or other parasites
  • Combination therapy: Antimicrobials plus anti-inflammatory agents

Adjunctive Treatments

  • Cycloplegics: Atropine or cyclopentolate to prevent synechiae
  • IOP-lowering agents: For steroid-induced or inflammatory glaucoma
  • Anti-VEGF injections: For macular edema (bevacizumab, ranibizumab)
  • Vitrectomy: For severe vitreous inflammation or complications

Monitoring and Follow-up

  • Regular eye exams: Monitor inflammation and treatment response
  • IOP monitoring: Watch for steroid-induced glaucoma
  • Systemic monitoring: Blood counts, liver function with immunosuppressants
  • Imaging follow-up: OCT for macular edema, fundus photos
  • Visual field testing: Monitor for glaucomatous damage

Treatment Goals

  • Control inflammation: Achieve zero or trace inflammation
  • Preserve vision: Maintain best possible visual acuity
  • Prevent complications: Glaucoma, cataracts, retinal damage
  • Minimize side effects: Balance efficacy with medication risks
  • Quality of life: Return to normal activities

Prevention

While many cases of uveitis cannot be prevented due to autoimmune or genetic factors, certain strategies may help reduce risk or prevent recurrence.

Primary Prevention

  • Infection prevention: Good hygiene, safe food handling
  • Vaccination: Stay up-to-date with recommended vaccines
  • Eye protection: Wear safety glasses during high-risk activities
  • Pet care: Proper handling of cats (toxoplasmosis prevention)
  • Travel precautions: Prophylaxis in endemic areas

Secondary Prevention

For Known Risk Factors

  • Autoimmune disease management: Optimal control of underlying conditions
  • Regular monitoring: Screening for patients with high-risk conditions
  • Medication compliance: Adherence to prescribed treatments
  • Lifestyle modifications: Stress reduction, healthy diet

Recurrence Prevention

  • Maintenance therapy: Long-term immunosuppression when indicated
  • Regular follow-up: Early detection of recurrent inflammation
  • Trigger avoidance: Identify and avoid personal triggers
  • Stress management: Techniques to reduce psychological stress

Complication Prevention

  • IOP monitoring: Regular pressure checks to prevent glaucoma
  • Cataract surveillance: Monitor for lens changes
  • Retinal monitoring: Watch for macular edema or detachment
  • Medication monitoring: Regular labs for immunosuppressant users

General Health Measures

  • Healthy lifestyle: Regular exercise, balanced diet
  • Smoking cessation: Reduces inflammatory burden
  • Adequate sleep: Supports immune system function
  • Stress reduction: Meditation, yoga, counseling
  • UV protection: Sunglasses to protect eyes

When to See a Doctor

Uveitis requires prompt medical attention to prevent vision-threatening complications. Certain symptoms warrant immediate emergency care.

Seek Immediate Medical Attention If:

Schedule Urgent Ophthalmologic Evaluation For:

  • New onset of eye redness with pain and light sensitivity
  • Gradual vision changes with eye discomfort
  • Persistent floaters or vision disturbances
  • Eye symptoms in patients with autoimmune diseases
  • Recurrent episodes of eye redness and pain
  • Any vision changes that concern you

High-Risk Patients Requiring Regular Monitoring

  • Children with juvenile idiopathic arthritis
  • Patients with ankylosing spondylitis
  • Individuals with inflammatory bowel disease
  • Those with history of uveitis
  • Patients on long-term immunosuppressive therapy

Follow-up Care

If diagnosed with uveitis, maintain regular follow-up:

  • Frequent monitoring during active inflammation
  • Regular IOP checks while on steroid therapy
  • Annual comprehensive eye exams minimum
  • Blood work monitoring if on systemic medications
  • Immediate contact for any symptom worsening

Red Flags

Call your eye doctor immediately if you experience:

  • Worsening symptoms despite treatment
  • New symptoms while on treatment
  • Side effects from medications
  • Any sudden vision changes
  • Signs of infection (discharge, fever)

Medical Disclaimer: This information is for educational purposes only and should not replace professional medical advice. Uveitis requires immediate ophthalmologic evaluation and treatment.

References

  1. Jabs DA, et al. (2023). Guidelines for the Treatment of Uveitis. Ophthalmology.
  2. American Academy of Ophthalmology. (2024). Preferred Practice Pattern: Uveitis.
  3. Dick AD, et al. (2023). Guidance on Noncorticosteroid Systemic Immunomodulatory Therapy in Noninfectious Uveitis. Ophthalmology.
  4. International Uveitis Study Group. (2024). Classification and Terminology of Uveitis. Am J Ophthalmol.
  5. Levy-Clarke G, et al. (2023). Expert Panel Recommendations for the Use of Anti-TNF Biologic Agents in Patients with Ocular Inflammatory Disorders. Ophthalmology.