We describe the case of a patient who presented with symptoms of persistent headaches, left-sided facial pain and blurry vision of the left eye. The patient had recovered from a herpes zoster infection of the V1 division of the trigeminal nerve 1 month prior. Serum inflammatory markers were elevated, raising concern for temporal arteritis. Empiric high-dose prednisone was initiated. Bilateral temporal artery biopsies were performed but did not show evidence of vasculitis or multinucleated giant cells. Instead, extracellular material deposits were present within the vessel walls. Congo red staining was diagnostic for amyloidosis. Liquid chromatography and mass spectrometry identified the amyloid fibrils to be transthyretin-type (ATTR) consistent with age-related amyloidosis. Temporal artery involvement of amyloidosis is rare but when present is most often due to light chain amyloidosis. Based on our review of the literature, only a few cases of temporal artery ATTR amyloidosis have been reported.
- connective tissue disease
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Presented at This article was presented as an abstract by Dr Riley at the North American Young Rheumatology Investigator Forum on 9 September 2020.
Contributors MR and MK: collection of medical information and data collection for the case. Both authors drafted and edited significant portions of the manuscript. AI: obtained the biopsy slide images and authoring portions of the manuscript. RP-W: review, editing and final approval of the manuscript. All listed authors agreed on the final draft of the manuscript and associated images. All authors agree to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article will be investigated and resolved.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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