Sinagra Gianfranco, Emdin Michele, Merlo Marco, Vergaro Giuseppe, Aimo Alberto, Biagini Elena, Imazio Massimo, Porcari Aldostefano, Limongelli Giuseppe, Cipriani Alberto, Canepa Marco, Musumeci Beatrice, Cameli Matteo, Crotti Lia, Di Bella Gianluca, Di Lenarda Andrea, Cappelli Francesco, Chimenti Cristina, Obici Laura, Iacoviello Massimo, Perlini Stefano, Pieroni Maurizio, Metra Marco, Oliva Fabrizio, Perrone Filardi Pasquale, Colivicchi Furio, Indolfi Ciro
S.C. Cardiologia, Dipartimento Cardiotoracovascolare, Centro per la Diagnosi e Cura delle Cardiomiopatie, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI) e Università degli Studi di Trieste, Trieste - European Reference Network for rare, low prevalence, or complex diseases of the Heart (ERN GUARD-Heart).
Health Science Interdisciplinary Center, Scuola Superiore Sant'Anna, Pisa - Fondazione Toscana Gabriele Monasterio, Pisa.
G Ital Cardiol (Rome). 2023 Feb;24(2):93-98. doi: 10.1714/3963.39416.
The perspective on amyloidosis has changed deeply over the last 10 years following major advances in diagnosis and treatment options, especially in cardiac amyloidosis. This intrinsically heterogeneous disease exposes to the risk of fragmentation of knowledge and requires the interaction among experts of different specialties and subspecialties. Suspicion of disease, timely recognition and confirmation of final diagnosis, prognostic stratification, clinical management and therapeutic strategies represent essential steps to be taken. Missing or delaying the diagnosis may have dramatic impact on patient outcome, as in the case of chemotherapy in unrecognized light-chain amyloidosis. Therefore, there is an urgent need for the foundation of an Italian Amyloidosis Network to deal with the challenges of this condition and orient clinical management at national and local levels. The present consensus document aims to provide the rationale and scopes of the Italian Amyloidosis Network, which has been conceived as an organizational framework for professionals managing patients with amyloidosis.
在过去10年里，随着诊断和治疗方法的重大进展，尤其是在心脏淀粉样变性方面，对淀粉样变性的认识发生了深刻变化。这种本质上异质性的疾病面临着知识碎片化的风险，需要不同专业和亚专业的专家之间进行互动。疾病的怀疑、最终诊断的及时识别和确认、预后分层、临床管理和治疗策略是必须采取的关键步骤。漏诊或延误诊断可能会对患者的预后产生巨大影响，比如未被识别的轻链淀粉样变性患者接受化疗的情况。因此，迫切需要建立一个意大利淀粉样变性网络，以应对这种疾病带来的挑战，并在国家和地方层面指导临床管理。本共识文件旨在提供意大利淀粉样变性网络的基本原理和范围，该网络被构想为管理淀粉样变性患者的专业人员的组织框架。
