Siakallis Loizos, Tziakouri-Shiakalli Chrysa, Georgiades Christos S
Department of Radiology, Attikon University Hospital, Athens, Greece.
Department of Radiology, Nicosia General Hospital, Nicosia, Cyprus.
Semin Ultrasound CT MR. 2014 Jun;35(3):225-39. doi: 10.1053/j.sult.2013.12.006. Epub 2013 Dec 24.
Amyloidosis is a collection of pathophysiologically related disease entities caused by the extracellular deposition of abnormal fibrillar proteins called amyloid. The accumulation of amyloid may be systemic, involving many organs, or localized manifesting as infiltration of individual organs, or in the form of a focal, tumorlike lesion. Amyloidosis may develop in the setting of underlying conditions, usually chronic inflammatory diseases, in which case it is termed secondary, or it may involve no underlying disease and thus be primary or idiopathic. Amyloid infiltration leads to pathology through the disruption of normal tissue structure and function or through cytotoxic effects of intermediate forms of protein aggregates. Clinical manifestations of the disease vary and are nonspecific, increasing the need of imaging during the investigation of the disease. Imaging findings are diverse and not pathognomonic; however, combined with the patient's clinical history they can raise the suspicion of amyloidosis and direct toward its confirmation by biopsy. Radiologists should be familiar with the appearance of amyloidosis in various modalities to aid the early identification of the disease and direct toward prompt treatment planning. Such knowledge would provide the radiologist with an opportunity to contribute to patient care and aid reducing the high morbidity and mortality of the disease.
淀粉样变性是一组病理生理相关的疾病实体，由称为淀粉样蛋白的异常纤维状蛋白在细胞外沉积引起。淀粉样蛋白的积累可以是全身性的，累及多个器官，也可以是局限性的，表现为单个器官的浸润，或以局灶性、肿瘤样病变的形式出现。淀粉样变性可在潜在疾病的背景下发生，通常是慢性炎症性疾病，在这种情况下称为继发性淀粉样变性，或者它可能不涉及潜在疾病，因此是原发性或特发性的。淀粉样蛋白浸润通过破坏正常组织结构和功能或通过蛋白质聚集体中间形式的细胞毒性作用导致病理改变。该疾病的临床表现多样且无特异性，这增加了在疾病调查过程中进行影像学检查的必要性。影像学表现多种多样，不具有特异性；然而，结合患者的临床病史，它们可以增加对淀粉样变性的怀疑，并通过活检来确诊。放射科医生应熟悉淀粉样变性在各种检查方式中的表现，以帮助早期识别该疾病并指导及时的治疗方案制定。这些知识将为放射科医生提供一个为患者护理做出贡献的机会，并有助于降低该疾病的高发病率和死亡率。
