Systemic Wegener's granulomatosis with severe orbito-ocular involvement

Area of Science:

• Ophthalmology
• Rheumatology
• Immunology

Background:

• Wegener's granulomatosis (WG), now known as Granulomatosis with Polyangiitis (GPA), is a rare autoimmune disease.
• Ocular and orbital manifestations are significant causes of morbidity in WG/GPA.

Observation:

• A 56-year-old male with limited systemic WG presented with pulmonary fibrosis, pansinusitis, and mastoiditis.
• He developed severe necrotizing anterior scleritis, peripheral ulcerative keratitis, and orbital apex syndrome.
• Diagnostic workup confirmed positive c-ANCA and anti-PR3 antibodies.

Findings:

• Despite aggressive immunosuppression with cyclophosphamide and prednisolone, visual prognosis was poor.
• Irreversible ischemic optic neuropathy, extensive corneoscleral melt, and corneal neovascularization contributed to vision loss.
• This case illustrates the potential severity of ocular and orbital involvement in WG/GPA.

Implications:

• Highlights the extensive range of orbital and ocular surface complications possible in WG/GPA.
• Emphasizes the critical need for ophthalmologic vigilance in patients diagnosed with WG/GPA.
• Underscores the challenges in preserving vision despite optimal immunosuppressive therapy for severe ocular WG/GPA.