Limper Marteen, Scirè Carlo Alberto, Talarico Rosaria, Amoura Zahir, Avcin Tadej, Basile Martina, Burmester Gerd, Carli Linda, Cervera Ricard, Costedoat-Chalumeau Nathalie, Doria Andrea, Dörner Thomas, Fonseca João Eurico, Galetti Ilaria, Hachulla Eric, Launay David, Lourenco Filipa, Macieira Carla, Meroni Pierluigi, Montecucco Carlo Maurizio, Moraes-Fontes Maria Francisca, Mouthon Luc, Nalli Cecilia, Ramoni Veronique, Tektonidou Maria, van Laar Jacob M, Bombardieri Stefano, Schneider Matthias, Smith Vanessa, Vieira Ana, Cutolo Maurizio, Mosca Marta, Tincani Angela
Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
RMD Open. 2018 Oct 18;4(Suppl 1):e000785. doi: 10.1136/rmdopen-2018-000785. eCollection 2018.
Antiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies. This review aims to identify existing clinical practice guidelines (CPG) as part of the ERN ReCONNET project, aimed at evaluating existing CPGs or recommendations in rare and complex diseases. Seventeen papers providing important data were identified; however, the literature search highlighted the scarceness of reliable clinical data to develop CPGs. With no formal clinical guidelines in place, diagnosis and treatment of APS is largely based on consensus and expert opinion. Patients' unmet need refers to the understanding of the disease and its clinical picture and implications, the need of education for patients, family members and healthcare providers, as well as to the development of monitoring pathways involving multiple healthcare providers.
抗磷脂综合征（APS）是一种罕见疾病，其特征为静脉和/或动脉血栓形成、妊娠并发症以及存在一类名为抗磷脂抗体的特定自身抗体。本综述旨在识别作为ERN ReCONNET项目一部分的现有临床实践指南（CPG），该项目旨在评估罕见和复杂疾病的现有CPG或建议。共识别出17篇提供重要数据的论文；然而，文献检索突出显示了用于制定CPG的可靠临床数据的稀缺性。由于没有正式的临床指南，APS的诊断和治疗很大程度上基于共识和专家意见。患者未满足的需求包括对该疾病及其临床表现和影响的了解、对患者、家庭成员和医疗服务提供者进行教育的需求，以及涉及多个医疗服务提供者的监测途径的开发。
