Wegener granulomatosis-associated optic perineuritis.

Area of Science:

• Ophthalmology
• Neurology
• Rheumatology

Background:

• Wegener granulomatosis (WG) is a systemic vasculitis that can affect various organs.
• Optic nerve involvement in WG, such as optic perineuritis (OPN), is rare.
• Hypertrophic pachymeningitis, inflammation of the dura mater, can also occur in WG.

Observation:

• Two male patients, aged 74 and 72, presented with sequential vision loss.
• Both patients exhibited optic perineuritis and hypertrophic pachymeningitis on MRI.
• Diagnosis of WG was supported by positive proteinase-3-antineutrophil cytoplasmic antibody (PR3-ANCA), pulmonary nodules, and other clinical findings.

Findings:

• MRI revealed abnormal enhancement in meninges and optic nerve sheaths, with T2-hyperintense lesions along optic nerves.
• Patient 1 showed no response to initial steroid therapy but improved with rapid steroid administration during the second episode.
• Patient 2 experienced improved vision and reduced MRI lesions with a combination of steroid and cyclophosphamide treatment.

Implications:

• The findings suggest that granulomatous inflammation can extend along anatomical tissue planes from the meninges to the perioptic tissues, causing visual loss in WG patients.
• This case series highlights the importance of considering WG in patients presenting with OPN and hypertrophic pachymeningitis.
• Prompt diagnosis and appropriate immunosuppressive therapy are crucial for managing visual dysfunction in WG.