Primary Systemic Al Amyloidosis Presenting as Temporal Arteritis

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. 1. Primary Systemic Al Amyloidosis Presenting with Temporal Arteritis Giant cell arteritis (GCA), also called temporal arteritis, is a vasculitis that affects large-and middle-sized blood vessels in individuals older than 50 years of age. A temporal artery biopsy (TAB) is the gold standard test in the diagnosis of GCA [1]. Because corticosteroid therapy is required for more than 1 year in most cases, the pathological confirmation of this vasculitis is advisable. AL amyloidosis is a systemic disorder that can present with a variety of symptoms or signs, including heavy proteinuria, edema, hepatosplenomegaly, otherwise, unexplained heart failure, and the carpal tunnel syndrome [2]. It often requires high clinical suspicion because symptoms are miscellaneous and easily mimicked by more common disorders. Hereby, we describe a case of a 67-year-old man who presented initially with GCA; however, a minor salivary gland biopsy then revealed AL amyloidosis. Hence, temporal artery biopsy should be performed for definitive diagnosis of temporal arteritis. 2. Case Report A 67-year-old man was applied to our hospital with a one-year history of weakness, fatigue, headache, extremity, and jaw claudication. Two months earlier he developed bilateral lack of vision and diagnosed ischemic optic neuropathy by ophthalmologist and hence started high dose corticosteroid treatment. Eye findings were improved by this treatment. On examination, the patient appeared well and his vital signs were normal. The temporal arteries were noted to be thickened, nodular, and decreased pulsation (Figure 1). Temporal arteritis was considered because of these findings and methotrexate was added to the patient therapy. Temporal artery biopsy was not considered in consequence of receiving corticosteroid therapy for two months. Figure 1: (a) Macroglossia due to amyloidosis and (b) enlarged and nodular temporal artery. During the followup, just after corticosteroid dose reduced, the patient was hospitalized to reevaluate because of recurrent complaints of increasing difficulty in walking,