E025 A complex case of giant cell arteritis presenting with visual loss, tongue and scalp infarction and multiple cranial neuropathies

Abstract

Background/Aims Giant cell arteritis (GCA) is the most common of the vasculitides. Visual loss due to vascular occlusion is a well recognised complication of GCA. It is important to remember that other ischaemic complications can occur, leading to atypical clinical features. We present a case of a patient with GCA who did not complain of headaches, but presented with at least five cranial neuropathies, including a right optic neuropathy, ischaemic ulceration of her tongue and a large area of scalp necrosis. Methods We present the results of our patient case review and literature search. Results An 82-year-old retired landlady who was an ex-smoker presented with haemoptysis and was diagnosed with non-small-cell lung cancer. She was treated with chemotherapy and radiotherapy in 2020, then had possible disease progression on CT thorax in 2021. She developed thigh pain, swallowing difficulties and decreased oral intake in 2021, and subsequently developed severe oral ulceration, dehydration and delirium and was admitted to the frailty unit. On examination she had a very large scalp lesion, oral lesions, and multiple cranial neuropathies (right optic neuropathy, right partial third nerve, right seventh nerve, recurrent laryngeal, and bilateral twelfth nerve palsies). Her anterior ischemic optic neuropathy meant she could only see hand movements with the right eye. Blood tests revealed anaemia of chronic disease and a CRP of over 200 mg/L. Ultrasound scan of the temporal arteries showed a halo sign confirming the diagnosis of GCA. CT angiography showed vertebral artery wall thickening and stenosis. She was treated with intravenous methylprednisolone followed by high dose oral prednisolone, and started on methotrexate. She improved symptomatically. The cranial neuropathies resolved and the area of scalp necrosis and ischemic tongue ulceration healed, but vision was not regained. Her inflammatory markers never normalised and she was subsequently started on tocilizumab, with the approval of her oncologist. Her ESR and CRP quickly fell to normal. It remains to be seen how biologic treatment may impact her malignancy. Conclusion This case highlights multiple atypical presenting features of GCA and the need for a high index of suspicion to treat early and avoid irreversible visual loss. A review of the literature reveals that cranial neuropathies secondary to ischemia can be the presenting feature in GCA and multiple cranial neuropathies can occur. This is the first case in the literature, that we are aware of, which involves this number of cranial neuropathies alongside extensive scalp and tongue infarction. These features have all resolved with treatment, with the exception of unilateral permanent visual loss. Disclosure J. Jade: None. V. Barradell: None. A. Coote: None.