Granulomatosis with polyangiitis presenting as axial proptosis and corneal perforation-a case report

Granulomatosis with polyangiitis (GPA) is a rare, aggressive, and rapidly progressive multisystem disease that primarily affects the respiratory tract, kidneys, and vascular system. Although less common, GPA can affect the eye through corneal involvement, scleral inflammation, and orbital mass formation. This report highlights a case in which sudden axial proptosis and corneal perforation revealed underlying GPA.

A 32-year-old female presented with a 20-day history of painful left eye proptosis, purulent discharge, photophobia, and progressive vision loss, which eventually led to no perception of light at presentation. She also reported recurrent episodes of congestion in both eyes over two years, intermittent episodes of haematuria, and chronic cough. On general examination, she was conscious and oriented to time, place, and person. Her vital signs were stable; however, she had pallor and bilateral chest crepitations. Ophthalmic examination revealed 6/6 vision in the right eye; however, the patient denied perceiving light in the left eye. The left eye exhibited axial proptosis, conjunctival congestion, chemosis, and a small corneal perforation with iris prolapse and a flat anterior chamber. A B-scan ruled out endophthalmitis, suggesting a non-infectious aetiology. On further imaging, CT orbit revealed a lacrimal fossa pseudotumor causing proptosis. HRCT of the chest revealed multiple cavitating lung nodules in both lungs. Systemic evaluation revealed normocytic normochromic anaemia, leucocytosis, thrombocytosis, elevated ESR (87 mm/hr), CRP (156 mg/L), and rheumatoid factor (184 IU/mL). Urinalysis revealed haematuria and proteinuria, suggesting renal involvement. ANCA ELISA was strongly positive for anti-PR3 (338 U/mL), confirming a diagnosis of granulomatosis with polyangiitis (GPA). She was treated with topical and intravenous antibiotics and tissue adhesive with bandage contact lens application for corneal perforation. Systemic immunosuppression was achieved with intravenous methylprednisolone and cyclophosphamide. She was discharged on oral steroids with scheduled follow-up visits in the ophthalmology and rheumatology departments.

This case highlights the importance of recognizing ocular signs as potential indicators of systemic vasculitis. Thus, timely diagnosis can help avert life-threatening systemic complications, thereby minimizing both the morbidity and mortality of the disease.