Eales Disease - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Eales disease is also called Eales retinopathy and idiopathic peripheral peri phlebitis. It is a rare disorder characterized by inflammation of the eyes and the formation of a white haze around the overcoat of the veins in the retina. It was first described by Henry Eales in 1880, hence called Eales disease. It has male predilection and is seen bilaterally. There is a sudden blurring of vision because of the formation of a clear jelly around and behind the eye's lens. At the onset of the disease, there is encapsulation around the outer layers of the retina. With the progression of the disease, the inflammation extends behind the lens.

Eales disease is also associated with peripheral retinal neovascularization, which is the formation of new blood vessels around the retina. In advanced cases, retinopathy and extensive bleeding are seen in the retina. In addition, clouding of the lens (cataract) may develop with the progression of the disease. The prognosis is good if it is treated early.

What Are the Causes of Eales Disease?

The exact etiology is unknown, and this disorder can occur spontaneously.

• History of tuberculosis may cause Eales disease, and hypersensitivity to tuberculoprotein may lead to Eales disease.

• Multiple sclerosis.

• Oxidative stress decreases levels of glutathione, vitamin C, and vitamin E.

• Autoimmune diseases.

• Free radical-mediated damage.

• Biochemical studies show raised globulin and decreased albumin levels in serum samples are seen in Eales disease.

What Is the Epidemiology of Eales Disease?

It is primarily reported in Asia, the Indian subcontinent, and the Middle East. It is more commonly seen in males than females around the second decade of life. It primarily occurs in young adults. Visual compromise is seen in patients with vitreous hemorrhage.

How does Eales Disease Develop?

The pathophysiology includes retinal periphlebitis (vasculitis that affects the peripheral retina), retinal ischemia, and neovascularization. In addition, Eales disease is associated with tuberculosis, multiple sclerosis, and individuals sensitive to tuberculoprotein.

What Is the Classification of Eales Disease?

According to Charmis, Eales disease is classified as follows:

Stage 1 - Mild peri phlebitis detected by ophthalmoscopy.

Stage 2 - Large veins are affected, and perivasculitis is seen in the retina.

Stage 3 - Neovascularization is seen with vitreous hemorrhages.

Stage 4 - Repeated vitreous hemorrhages and end-stage disease.

What Are the Symptoms Associated With Eales Disease?

• Unilateral or bilateral decreased vision.

• Photopsias (flickering lights).

• Vitreous hemorrhages.

• Eye floaters (small spots are formed in the vision region).

How Is Eales Disease Diagnosed?

• Clinical Features: Diagnosis is based on clinical features, signs, and symptoms and on the principle of exclusion from other systemic or ocular conditions that may have similar presentations.

• Mycobacterium Tuberculosis Genome: The presence of the Mycobacterium tuberculosis genome and disease can also aid in the diagnosis, as it is a known etiology and other autoimmune causes.

• Fluorescein Angiography (FA): If there is leakage of fluorescent dye around the veins, it shows active inflammation, and if there is no leakage, it indicates sclerosis of vessels.

• Wide-Field FA: It is done to assess peripheral areas of the retina with better quality.

• Ultrasonography: Ultrasonography helps in the detection of retinal detachment.

• Optical Coherence Tomography (OCT): OCT helps detect the involvement in the peripheral retina or macular degeneration.

• OCT-Angiography (OCT-A): OCT-A helps detect neovascularization depending on the location of involvement.

• Laboratory Tests: A distinct protein with an isoelectric point of 5.9 and weight of 23 kDa is associated with Eales disease; also, there is an elevation of alpha globulins, alpha-1 acid glycoproteins, MMP-9, and reduction of serum albumin.

What Is the Management of Eales Disease?

The treatment of the disease is symptomatic. The main aim of the treatment is to reduce perivasculitis, vitritis, and vitreous hemorrhage.

1. Corticosteroids - They remain the mainstay of treatment in the inflammation stage and help reduce vasculitis. Oral and periocular corticosteroids are given to treat vasculitis, such as Prednisolone and intravitreal Triamcinolone.

2. Antitubercular Treatment (ATT) - ATT is given for nine months. It is given to patients with acute phlebitis with nodule formation and complete vein obliteration.

3. Anti-Vascular Endothelial Growth Factor Therapy - This is a definitive therapy in the management of Eales disease. Intravitreal Bevacizumab is used in managing vitreous hemorrhage and helps in the regression of new blood vessels.

4. Photocoagulation - Photocoagulation is done in the proliferative phase of Eales disease. Retinal lasers are used to cure the disease, replacing the xenon photocoagulation and anterior retinal cryopexy. Patients undergoing photocoagulation show a better prognosis. Fluorescein angiography is performed to monitor the treatment.

5. Vitrectomy - Vitrectomy is an eye surgery to treat various problems with the retina and vitreous. Indication of vitrectomy in recurring vitreous hemorrhages and retinal detachments. Additional procedures are also done, such as belt buckling, a surgical procedure done to produce functional closure of the retinal breaks responsible for the retinal detachment, and lensectomy can also be performed to reduce the opacities and improve the visualization. It surgically removes the crystalline lens through an incision in which vitreous gel is partially or entirely removed. Surgical complications include early cataracts, glaucoma, neovascularization, and retinal detachment.

How to Prevent Eales Disease?

Following are some steps one can take to maintain good eye health and reduce the risk of developing eye conditions, including Eales disease. These include the following:

• Having regular eye exams.

• Eating a healthy diet.

• Protecting the eyes from UV radiation.

• Avoid smoking.

• Regular eye check-ups play a pivotal role in preventing the progression of Eales disease. Routine visits to an ophthalmologist can help detect any early signs of retinal inflammation or vision changes. Individuals with a family history of retinal disorders or those in the at-risk age group should be particularly vigilant about scheduling annual eye examinations. Early detection allows timely intervention and reduces the risk of severe vision complications.

• A diet rich in antioxidants, vitamins (particularly vitamin A), and omega-3 fatty acids can support retinal health. Foods like leafy greens, fish, citrus fruits, and nuts are beneficial for maintaining optimal eye function.

• Staying adequately hydrated is essential for overall health, including eye health. Proper hydration supports the function of the tear film and the maintenance of clear vision.

• Individuals engaging in activities that pose a risk of eye injury, such as sports or certain professions, should use appropriate protective eyewear. Eye injuries can trigger inflammation and potentially exacerbate underlying conditions like Eales disease.

Conclusion

Eales disease is especially prevalent in India. There is a strong association between occlusive vasculitis and tuberculosis. Eales disease is treated with antitubercular drugs and corticosteroids. Photocoagulation is performed in patients with retinal ischemia. Recurrent vitreous hemorrhage is a hallmark of the disease, so regular follow-up is required for early intervention. With a proper diagnosis, the prognosis of the disease is good. The primary cause of vision loss is recurrent vitreous hemorrhage. Visual outcomes depend on the disease stage, treatment, and regular follow-ups.