Retinal Vasculitis - Signs, Symptoms, Diagnosis, and Management

What Is Retinal Vasculitis?

Retinal vasculitis is a fatal condition that causes swelling of blood vessels of the retina. Retinal vasculitis is either idiopathic or can result from systemic disease and infection. The research suggests that almost 33 percent of retinal vasculitis cases have unknown etiology. On the other hand, systemic diseases include multiple sclerosis, systemic lupus erythematosus, syphilis, or polyangiitis. The condition usually represents the symptoms from pain to visual acuity in patients. There are a plethora of differential diagnoses associated with this condition, and after ruling them out with various diagnostic methods, the doctor can go for the management. Moreover, there are many ophthalmic imaging options available that can evaluate the extent and progress of the condition as well as the location and the complications associated with it.

What Are the Classifications of Retinal Vasculitis?

Retinal vasculitis is classified as below:

• According to the Location:

  • Retinal Vasculitis Affecting Near Macula: This type of condition causes critical symptoms such as affected visual acuity: This type of form is usually asymptomatic.

  • Retinal Vasculitis Affecting Peripheral Vessels: It is a systemic or inflammatory disorder affecting peripheral retinal vessels. The condition is caused by either viral or bacterial infections.

• According to the Occlusive Form:

  • Occlusive: It is a rare type of retinal vasculitis that can lead to neovascularization which leads to vitreous hemorrhage.

  • Non-occlusive: It is usually in the asymptomatic form.

What Are the Disorders Associated With Retinal Vasculitis?

There are the following disorders connected with retinal vasculitis:

• Bacterial Disorders:

  • Tuberculosis.

  • Syphilis.

  • Lyme disease.

  • Whipple’s disease.

  • Pots-streptococcal syndrome.

• Viral Disorders:

  • Human T lymphoma virus.

  • Herpes simplex virus.

  • Varicella zoster virus.

  • Acquired immunodeficiency virus.

  • West Nile virus.

  • Dengue fever virus.

• Parasitic disorders.

• Toxoplasmosis.

• Multiple sclerosis.

• Malignancy.

• Neurological disorders.

• Systemic inflammatory diseases.

• Churg-Strauss syndrome.

• Rheumatoid arthritis.

• Crohn’s disease.

• Ocular disorders.

What Are the Signs and Symptoms of Retinal Vasculitis?

The followings are the signs and symptoms associated with retinal vasculitis:

• Pain.

• Reduced vision.

• Panuveitis is also known as diffuse uveitis. During this condition, the inflammations are seen associated with all uveal components of the eyes.

• Hypopyon formation is usually seen in corneal ulcers where the neutrophils and fibrin are accumulated.

• Neovascularization is the condition where new blood vessels are formed and grow. It is usually seen in the retina and cornea, where due to this condition, the blood vessels might leak and cause vision problems.

• Epiretinal membrane is a fibrovascular tissue that is commonly found on the inner exterior of the retina.

• Intraretinal hemorrhage is a dark red and sharply outlined lesion seen on the deep capillary layer of the eye due to bleeding in the new vessels.

• Vascular sheathing condition is the accumulation of inflammatory cells around the affected vessels in the eye. It is usually caused by toxoplasmosis along with TB (tuberculosis).

• Intraocular inflammation is an eye inflammation caused by infection, injury, or trauma.

How to Diagnose Retinal Vasculitis?

The diagnostic method is based on the physician's differential diagnosis from the case history and physical examination. Moreover, if the patient's symptoms are suggestive of underlying diseases, the healthcare provider usually suggests other tests. For instance, fluorescein angiogram, complete blood count, erythrocyte sedimentation rate, VDRL (veneral disease research laboratory test), FTA-ABS (fluorescent treponemal antibody absorption), blood chemistry, urinalysis, tuberculin skin testing, HIV serology (human immunodeficiency virus), and chest radiograph are the next options for diagnosis.

There are the following diagnostic methods used for retinal vasculitis:

• Laboratory Tests:

  • Erythrocyte sedimentation rate.

  • C-Reactive protein.

  • Blood sugar.

  • Tuberculin skin testing.

  • Lyme disease serology.

  • Dengue virus serology.

• Imaging:

  • Ultra Wide-Field Fluorescein Angiography: This type of diagnostic method is used to evaluate the extent of the condition, along with the location of vascular leakage in the retina. Also, with this diagnostic method along with others, retinal infiltration, retinal vasculitis, and cystoid macular edema can be detected with this diagnostic option.

  • Optical Coherence Tomography and Fundus Photography: This diagnostic method is used to detect retinal and choroidal lesions. Also, complications such as cystoid macular edema or retinal neovascularization can be detected with this method.

  • Ultrasonography: This diagnostic method is usually evaluated if the patient is suffering from retinal detachment or other vascular abnormalities associated with the eyes.

  • Chest X-ray: This diagnostic method is used to check an abnormality or complication associated with the heart, lungs, or other arteries caused by retinal vasculitis.

  • Magnetic Resonance Imaging (MRI): MRI is a widely used non-invasive diagnostic procedure that can give any information about the body’s soft tissues, blood flow, or organ function.

  • Sacroiliac X-ray: This diagnostic method is used to evaluate the etiological factor behind the pain or swelling in the pelvic region of the body.

What Are the Differential Diagnosis of Retinal Vasculitis Based on Ophthalmoscopic Findings?

The following are the differential diagnosis based on ophthalmoscopic findings:

• Phlebitis:

  • Bachet’s disease.

  • Tuberculosis.

  • Human immunodeficiency virus infection.

• Arteritis:

  • Acute retinal necrosis.

  • Idiopathic retinal vasculitis.

  • Polyarteritis.

• Cotton-Wool Spots:

  • Systemic vasculitis.

  • Churg-Strauss syndrome.

• Internal Infiltrations:

  • Bachet’s disease.

  • Cat scratch disease.

• Necrotizing Retinitis:

  • Ocular toxoplasmosis.

  • Acute retinal necrosis.

• Frosted Branch Angiitis:

  • Crohn’s disease.

  • Epstein-Barr virus infection.

  • Herpes simplex virus.

  • Acquired immunodeficiency syndrome.

• Retinal Ischemic:

  • Tuberculosis.

  • Eale’s disease.

  • Multiple sclerosis.

How to Manage Retinal Vasculitis?

There are different factors affecting the management options suitable for the patient. For instance, etiology, severity, cause of vasculitis, and type there are the following management options available for retinal vasculitis:

• Systemic Immunosuppressant:

  • Corticosteroids and Cyclosporine A to manage inflammation.

  • IMS agents, such as Methotrexate, along with systemic corticosteroids, can reduce cotton wool spots or vasculopathy.

  • Azathioprine twice a day.

• Biologics:

  • In the cases of vision acuity, anti-TNF (Antitumor necrosis factor alpha) drugs are used. These are Infliximab and Adalimumab. In severe cases, these drugs can be used as the first line of IMS treatment too. These two drugs are usually an excellent choice in treating refractory cases of sarcoidosis along with retinal vasculitis.

• Plasma Exchange:

  • Although plasma exchange has not proven to be as beneficial as other treatment options, it is still useful for severe cases of cerebritis or alveolar hemorrhage.

  • In critical cases of systemic lupus erythematosus, Plasma exchange treatment along with Rituximab infusion has given stability in occlusive retinal vasculopathy.

• Other Drugs:

  • Catastrophic antiphospholipid syndrome is managed by drug therapy that includes anticoagulants, corticosteroids, intravenous immunoglobulins, and plasma exchange. Having said that, long-term anticoagulation therapy has not proven to be much beneficial in retinal vaso-occlusive events.

Conclusion

Retinal vasculitis can cause fatal damage along with many health-related complications. The condition has many different causes from genetics to systemic disease. If the patient with this condition is not treated on time, they might suffer from irreversible vision. Usually, with retinal vasculitis, patients are recommended the diagnostic option such as wide-field angiography which can evaluate retinal ischemia.