The Intersection of Autoimmunity and Ocular Disease: Ocular Manifestations of Granulomatosis with Polyangiitis in Focus: A Case Report

Abstract

Introduction: Granulomatosis with Polyangiitis is a rare type of systemic vasculitis which affects multiple organs.
The ocular manifestations are diverse and are reported in 13- 60% of patients with a risk of permanent visual loss.
Prompt diagnosis and management is required, which are critical for visual and systemic outcomes.
Case report: A 40-year-old female with Granulomatosis with Polyangiitis presented with painful diminished
vision in left eye. On examination, the patient had a saddle nose deformity, conjunctival chemosis with congestion,
bilateral axial proptosis, and total ophthalmoplegia with painful eye movements. Visual acuity was 6/60 in the
right eye and perception of light in the left. Grade 3 RAPD was noted in the left eye and both eyes had posterior
subcapsular cataracts. Blood tests indicated a systemic inflammatory response with elevated anti-PR3 antibodies
(181 Ru/ml). MRI of the brain and orbits revealed bilateral orbital involvement with heterogenous enhancement
in the medial extraconal and intraconal spaces (L>R), perioptic neuritis on the left, and thickening of all recti
muscles. Paranasal sinus hyperostosis and lamina papyracea erosion were also noted. She was treated with IV
methylprednisolone followed by oral steroids; pain resolved, but vision did not improve. She was subsequently
lost to follow-up.
A year later, the patient presented with a one-week history of pain, redness, and photophobia in the left eye. The right
eye’s anterior and posterior segments were normal. The left eye showed a peripheral corneal ulcer with adjacent
scleritis. The patient was referred to a rheumatologist and started on systemic immunosuppressives, including
IV methylprednisolone followed by a tapering course of oral steroids and continued oral cyclophosphamide.
Ocular inflammation stabilized with resolution of scleritis. ENT evaluation and septal biopsy confirmed GPA.
Pulmonology assessment and CT thorax revealed nodular lesions and fibrosis, indicating lung involvement. The
patient is now stable and under regular multidisciplinary follow-up.
Conclusion: This case highlights the vision-threatening ocular manifestations of Granulomatosis with Polyangiitis,
including orbital inflammation, peripheral ulcerative keratitis, and scleritis. It emphasises on the importance of
early diagnosis and timely intervention in controlling disease progression. Regular follow-up remains crucial for monitoring systemic involvement and maintaining visual function.