Background Anterior Ischemic Optic Neuritis (AION) is caused by arteritic and non arteritic factors. Giant Cell Arteritis is one of the well-recognised causes of arteritic AION. AION is a rare but recognised manifestation of ANCA associated vasculitis.
Objectives We present a rare case of ANCA associated vasculitis presenting with AION
Methods 65 years old gentleman with no previous co morbidity, but history of nasal crusting, presented to a neighbouring District General Hospital in November 2011 with night sweats and loss of weight. Investigations returned negative for infections/malignancies. He was found to be positive for MPO antibody and was discharged with Prednisolone 30 mgs OD and outpatient appointment for IV Cyclophosphamide. He presented to our department 2 days later with sudden loss of vision in right eye, shortness of breath and decreased sensation and paraesthesia in both hands. Further investigations revealed raised CRP 135 mg/L, impaired renal functions with Serum Creatinine of 130μmol/l, confirmed ANCA positivity for p-ANCA and pathological urinary cast. High resolution CT thorax revealed interstitial lung infiltrates. Nerve conduction study confirmed sensory polyneuropathy with bilateral mononeuritis multiplex of median nerve. Ophthalmological assessment revealed AION of right eye secondary to ANCA associated vasculitis.
Results Patient was commenced on IV methyl prednisolone at 1mg/kg/day for 3 days followed by IV cyclophosphamide 750mg. A week later he developed similar visual symptoms in left eye, and further evaluation confirmed AION. Patient received further IV methylprednisolone for 3 days and plasmapheresis. Following this he received 6 cycles of IV cyclophosphamide as per BSR guidelines on management of ANCA associated vasculitis. There was no significant improvement in his vision and he was registered blind. However the pulmonary infiltrates subsided completely and renal functions normalised. The sensory neuropathy has shown a steady improvement. Azathioprine was tried as maintenance treatment but stopped due to recurrent respiratory tract infections. Currently patient is in remission on Mycophenolate Mofetil 2 grams/day and tapering dose of oral prednisolone.
Conclusions ANCA associated vasculitis can present with AION. Early referral to a tertiary centre is essential as the disease can run an aggressive course.
Duran et al. ANCA-associated small vessel vasculitis presenting with ischemic optic neuropathy. Neurology2004;62(1):152-3
Shichinohe N et al. Arteritic anterior ischemic optic neuropathy with positive myeloperoxidase antineutrophil cytoplasmic antibody. Jpn J Ophthalmol 2010;54(4):344-8
Acknowledgements Department of Rheumatology, Department of Ophthalmology and Department of Renal Medicine, Hull Royal Infirmary, Hull and East Yorkshire Hospital NHS Trust, Hull.
Disclosure of Interest None Declared
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