Urban B A, Fishman E K, Goldman S M, Scott W W, Jones B, Humphrey R L, Hruban R H
Department of Radiology and Radiological Science, Johns Hopkins Hospital, Baltimore, MD 21287.
Radiographics. 1993 Nov;13(6):1295-308. doi: 10.1148/radiographics.13.6.8290725.
Amyloidosis is a rare systemic disease caused by extracellular deposition of an insoluble protein. Although it is usually seen in a systemic form, 10%-20% of cases can be localized. Systemic amyloidosis is subclassified into an idiopathic primary form and a secondary or reactive form. Patients with primary amyloidosis have no underlying condition or disease. Men are affected more than women, and the mean age at presentation is 55-60 years. Some causes of secondary amyloidosis are multiple myeloma (10%-15%), rheumatoid arthritis (20%-25%), tuberculosis (50%), or familial Mediterranean fever (26%-40%). Radiographic studies of 90 patients with biopsy-proved primary or secondary amyloidosis were reviewed. Computed tomographic (CT) scans demonstrated a wide spectrum of disease in the cardiothoracic, gastrointestinal, genitourinary, and musculoskeletal systems. Amyloid deposition simulated both inflammatory and neoplastic conditions. Amorphous or irregular calcifications were occasionally identified within the amyloid deposit. Definitive diagnosis requires biopsy confirmation, as CT findings are nonspecific.
淀粉样变性是一种由不溶性蛋白质在细胞外沉积引起的罕见的全身性疾病。虽然它通常以全身性形式出现,但10%-20%的病例可能是局限性的。全身性淀粉样变性可细分为特发性原发性形式和继发性或反应性形式。原发性淀粉样变性患者没有潜在疾病。男性比女性更容易受到影响,发病的平均年龄为55-60岁。继发性淀粉样变性的一些病因是多发性骨髓瘤(10%-15%)、类风湿性关节炎(20%-25%)、结核病(50%)或家族性地中海热(26%-40%)。对90例经活检证实为原发性或继发性淀粉样变性的患者的影像学研究进行了回顾。计算机断层扫描(CT)显示心胸、胃肠、泌尿生殖和肌肉骨骼系统存在广泛的疾病。淀粉样沉积模拟了炎症和肿瘤情况。淀粉样沉积物内偶尔可发现无定形或不规则钙化。由于CT表现不具有特异性,明确诊断需要活检证实。