Amyloidosis is a common complication of poorly controlled familial Mediterranean fever (FMF). A variety of organs including kidneys, heart, liver, thyroid and adrenal glands may be clinically affected. However, involvement of adrenal glands leading to significant inefficiency is rarely seen in FMF patients with amyloidosis. The impairment of neuroendocrine immune system in FMF together with proteinuria in renal amyloidosis is a challenge while interpreting adrenal function tests. Here we present a case report of a 42-year-old man with FMF and renal failure due to amyloidosis whose disease course was complicated by adrenal insufficiency.
DESCRIPTIONAn 80-year-old man presented with a 3-monthhistory of hoarseness that developed gradually and remained with the same intensity afterwards.He had neither a history of constitutional symptoms nor cardiorespiratory complaint. He worked as a farmer for 40 years and never smoked. He was taking atenelolol 100 mg daily for hypertension.He was initially referred to the otolaryngologist. On direct laryngoscopy, the movement of arytenoid cartilage and true vocal cord on the left side was impaired and no discrete mucosal lesion was seen. He was referred to the pulmonology clinic to undergo fiberoptic bronchoscopy as there was ground glass opacity over the lateral segment of the right middle lobe and suspicious lymph adenopathy in the aorto-pulmonary window.He was hypertensive with right arm blood pressure of 170/110 mm Hg. Otherwise, the rest of the examination was normal. Bronchoscopic examination was deterred as thrombosed dissection of the aortic arch was the most probable diagnosis. Figure 1 (A-D) Axial, coronal and sagittal sections of thoracic aorta angiogram show aortic atherosclerosis and mural thrombosis in dissected aortic arch extending to the descending aorta.
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