What are the vision changes associated with Granulomatosis with Polyangiitis (GPA) vasculitis?

From the Guidelines

Vision changes in GPA (Granulomatosis with Polyangiitis) vasculitis require immediate medical attention as they can lead to permanent vision loss, and treatment should be guided by the most recent evidence, such as the european position paper on rhinosinusitis and nasal polyps 2020 1. The most common vision changes include eye redness, pain, blurred vision, double vision, and sudden vision loss, which occur due to inflammation of blood vessels supplying the eye, retina, or optic nerve. According to the european position paper on rhinosinusitis and nasal polyps 2020 1, diagnosis can be delayed by 6 months or longer, rendering the condition potentially life-threatening, and the delay was greatest when patients presented with eye symptoms (>14 months). Some key points to consider in the management of GPA vasculitis include:

• Treatment typically involves high-dose corticosteroids (prednisone 1mg/kg/day) combined with immunosuppressive therapy such as cyclophosphamide (2mg/kg/day) or rituximab (375mg/m² weekly for 4 weeks) 1.
• For severe cases threatening vision, methylprednisolone pulse therapy (500-1000mg IV daily for 3 days) may be initiated before transitioning to oral steroids.
• Patients should be co-managed by rheumatology and ophthalmology specialists with regular eye examinations to monitor disease activity.
• Long-term maintenance therapy with azathioprine, methotrexate, or mycophenolate mofetil is usually required after remission is achieved.
• These medications work by suppressing the abnormal immune response that causes blood vessel inflammation in GPA, protecting the delicate structures of the eye from further damage and preserving vision. It is also important to note that the primary open-angle glaucoma preferred practice pattern 1 provides guidance on the clinical findings characteristic of primary open-angle glaucoma, which may be relevant in the management of GPA vasculitis. However, the european position paper on rhinosinusitis and nasal polyps 2020 1 is the most relevant and recent evidence for the management of GPA vasculitis, and should be prioritized in clinical decision-making. Some key considerations in the management of GPA vasculitis include:
• The Birmingham Vasculitis Activity Score (BVAS) and the Vasculitis Damage Index (VDI) can be used to measure disease activity and damage.
• The ELK-classification of organ involvement (ENT, lung, kidney) can be used to classify the extent of disease involvement.
• Regular monitoring of disease activity and adjustment of treatment as needed is crucial to prevent long-term damage and preserve vision.

From the Research

Vision Changes with GPA Vasculitis

• Vision changes can occur in patients with Granulomatosis with Polyangiitis (GPA) due to the disease's effect on small and medium vessels, including those in the eyes 2.
• Ocular manifestations of GPA can include rhinitis, sinubronchitis, exophthalmos, and granulomatous lesions in the orbit 2.
• Treatment with biological therapies such as rituximab and infliximab has improved the management of ocular manifestations in GPA 2, 3.
• However, vision loss can still occur in patients with GPA, and it is often associated with pain and orbital findings 4.
• Atypical painless vision loss has also been reported in patients with GPA, highlighting the importance of collaboration amongst sub-specialties and obtaining tissue samples for diagnosis 4.

Risk Factors for Vision Loss

• Age and presence of coronary artery disease are risk factors for permanent vision loss in patients with Giant Cell Arteritis (GCA) 5.
• Diabetes and atherosclerosis are also associated with an increased risk of ocular involvement in GCA 5.
• Patients with GCA and ocular involvement are more likely to have baseline vascular risk factors and fewer constitutional symptoms than patients without ocular involvement 5.

Treatment Options

• High-dose corticosteroid therapy is the mainstay of treatment for GPA and GCA, and it is often administered intravenously or orally to prevent further vision loss and treat systemic vasculitis 6.
• Adjunctive immunomodulatory therapy may be considered in patients experiencing relapsing inflammation despite high doses of corticosteroids or those with corticosteroid-induced complications 6.
• Emerging evidence supports the use of tocilizumab, methotrexate, aspirin, angiotensin receptor blockers, and statins as adjunctive therapies for GCA 6.