Primary Systemic Al Amyloidosis Presenting as Temporal Arteritis

Abstract

Temporal arteritis is most common vasculitis in elderly and imitated by miscellaneous disorders. Temporal artery biopsy is the gold standard test in the diagnosis of giant cell arteritis (GCA). Hereby, we describe a case of a 67-year-old man who presented initially with temporal arteritis; however, a lip biopsy then revealed AL amyloidosis. In this respect, temporal artery biopsy should be performed for definitive diagnosis of GCA particularly patients with systemic symptoms and treatment resistant.

Bilateral Non-arteritic Anterior Ischaemic Optic Neuropathy as the Presentation of Systemic Amyloidosis

“…There has also been a report of a 67-year-old patient who presented with fatigue, headache, jaw claudication, and visual disturbance, was suspected to have GCA and treated with high-dose corticosteroids, but later developed nodular stricture of his lips and a persistently elevated ESR leading to a lip biopsy that confirmed systemic amyloidosis. 2 Ischaemic optic neuropathy was not established as the cause of the visual disturbance because the patient died from a myocardial infarction prior to temporal artery biopsy. Ischaemic optic neuropathy has also been described in familial amyloidotic polyneuropathy.…”

“…Amyloidosis is a systemic condition caused by deposition of insoluble abnormal amyloid fibrils intra-or extracellularly. There are few previously reported cases of optic neuropathy in the context of light-chain (AL) amyloidosis, [1][2][3] none of which occurred without typical symptoms or laboratory findings suggestive of giant cell arteritis (GCA). We present a case of bilateral sequential optic neuropathy, presumed ischaemic, as the presenting manifestation of systemic amyloidosis.…”