Wegener’s Granulomatosis, now more commonly known as Granulomatosis with Polyangiitis (GPA), is a rare autoimmune condition that primarily affects the small and medium-sized blood vessels. It is part of a group of disorders called vasculitis, where the body’s immune system attacks blood vessels, leading to inflammation. GPA typically impacts the respiratory system (lungs, nose, sinuses) and kidneys but can also affect the eyes, causing a variety of ocular manifestations that, if untreated, can lead to serious vision complications.

This article will focus on GPA’s impact on ocular health, discussing the disease’s pathophysiology, common eye-related symptoms, diagnostic approaches, and treatment options.

In GPA, the body’s immune system mistakenly targets the blood vessels, leading to the formation of granulomas (masses of immune cells) and polyangiitis (inflammation of multiple vessels). This combination of granuloma formation and vessel inflammation disrupts blood flow to various organs and tissues, including the eyes, potentially causing damage. The disease is strongly associated with the presence of antineutrophil cytoplasmic antibodies (ANCAs), which play a key role in its pathogenesis.

Ocular involvement occurs in approximately 50% of GPA patients, either as the initial symptom or as the disease progresses. GPA can affect virtually every structure of the eye, ranging from mild to severe complications.

1. Orbital Involvement

Orbital disease is one of the most common ocular manifestations of GPA and can cause significant discomfort and vision impairment. It includes:

• Orbital Pseudotumor: The formation of an inflammatory mass in the orbit, mimicking a tumor, causing proptosis (bulging of the eye), pain, and sometimes double vision.
• Orbital Inflammation: Inflammation of the tissues around the eye, leading to swelling, pain, and restricted eye movement. Orbital inflammation in GPA can cause compressive optic neuropathy, leading to vision loss.

2. Scleritis and Episcleritis

• Scleritis: GPA can lead to inflammation of the sclera (the white outer layer of the eye), which causes severe pain, redness, and tenderness. Scleritis is often associated with severe systemic disease and may lead to thinning of the sclera (scleromalacia) and perforation if untreated.
• Episcleritis: A milder, more superficial form of scleral inflammation, episcleritis causes redness and discomfort but is typically less painful and severe than scleritis.

3. Keratitis

• Peripheral Ulcerative Keratitis (PUK): In severe cases, GPA can cause thinning and ulceration of the peripheral cornea, leading to pain, photophobia (light sensitivity), and vision loss if not treated promptly. PUK is a serious complication that can result in corneal perforation and blindness.

4. Uveitis

• GPA can cause inflammation of the uvea, the middle layer of the eye. Uveitis may present as redness, pain, blurred vision, and floaters. It can involve either the anterior uvea (iritis) or the posterior segment (choroiditis or retinitis), leading to significant vision problems.

5. Retinal Vasculitis

• Inflammation of the retinal blood vessels (retinal vasculitis) can occur, leading to retinal hemorrhages, exudates, and, in severe cases, retinal detachment. This can significantly affect vision if the central retina (macula) is involved.

6. Optic Nerve Involvement

• Optic Neuritis: Inflammation of the optic nerve can cause sudden vision loss in one or both eyes. This may be due to direct involvement of the optic nerve by GPA or secondary to orbital inflammation compressing the optic nerve.

7. Nasolacrimal Duct Obstruction

• Chronic inflammation in GPA can cause blockage of the nasolacrimal duct, leading to excessive tearing (epiphora) and an increased risk of eye infections, such as dacryocystitis.

Ocular involvement in GPA can be subtle or dramatic, and diagnosis often requires a multidisciplinary approach. Important steps in diagnosing GPA with ocular involvement include:

1. Clinical Evaluation

• A detailed eye examination by an ophthalmologist is crucial, especially when symptoms like pain, redness, swelling, or vision changes occur. The clinician may use imaging techniques like orbital CT or MRI scans to assess orbital masses or inflammation.

2. Laboratory Tests

• ANCA Testing: The presence of anti-neutrophil cytoplasmic antibodies (particularly c-ANCA) is a hallmark of GPA and aids in diagnosis.
• Biopsy: In cases where tissue is accessible, such as conjunctival or orbital biopsy, histopathological examination showing granulomas and vasculitis can confirm the diagnosis.

3. Imaging

• Imaging techniques like ultrasound biomicroscopy (UBM) or B-scan echography may be used to assess the extent of inflammation in the eye. MRI or CT scans of the orbit can help detect orbital masses or pseudotumors.

The treatment of GPA requires immunosuppressive therapy to control the underlying systemic vasculitis and prevent further damage. Ocular manifestations often respond to systemic treatment, but local therapies are sometimes needed to address specific eye-related issues.

1. Systemic Immunosuppressive Therapy

• Corticosteroids: High-dose systemic corticosteroids are the cornerstone of treatment to control inflammation. These can be administered orally or intravenously, depending on disease severity.
• Immunosuppressants: Drugs like cyclophosphamide, methotrexate, or azathioprine are often used to reduce the immune system’s attack on blood vessels.
• Biologic Agents: Rituximab, a monoclonal antibody, has been increasingly used in GPA treatment, especially in patients with severe or refractory disease.

2. Local Ocular Treatments

• Topical or Systemic Corticosteroids: For conditions like scleritis or uveitis, topical steroid drops or injections may be used in conjunction with systemic therapy.
• Surgical Intervention: In cases of nasolacrimal duct obstruction or corneal perforation, surgical procedures such as dacryocystorhinostomy (DCR) or corneal transplantation may be necessary.

3. Monitoring and Follow-Up

• Regular follow-up with both an ophthalmologist and a rheumatologist is essential to monitor disease progression and treatment efficacy. Early intervention can prevent irreversible damage to the eye and vision loss.

With early diagnosis and appropriate treatment, the prognosis for patients with GPA has improved significantly. However, ocular complications can lead to vision loss if not managed promptly. Long-term immunosuppressive therapy is often required to maintain disease remission and prevent relapse.

Granulomatosis with Polyangiitis is a serious autoimmune disease that can significantly impact ocular health. From orbital inflammation to retinal vasculitis and optic neuritis, the disease manifests in various ways, requiring prompt diagnosis and aggressive treatment. Collaboration between ophthalmologists and rheumatologists is critical for managing the disease and preserving vision in affected patients.

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