Deb-Chatterji Milani, Schuster Simon, Haeussler Vivien, Gerloff Christian, Thomalla Götz, Magnus Tim
Department of Neurology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany.
Front Neurol. 2019 Jun 6;10:568. doi: 10.3389/fneur.2019.00568. eCollection 2019.
Primary angiitis of the central nervous system (PACNS) is an inflammatory brain disease affecting the medium and small vessels of the CNS. Although recent data of patients with PACNS have advanced the understanding of the disease, the diagnosis remains challenging. Clinical presentation of PACNS is broad and unspecific and the majority of the diagnostic approaches are hallmarked by a low specificity. Thus, PACNS is commonly misdiagnosed. In addition, due to its potential aggressive course which may be altered by an adequate immunosuppressive treatment, delineation from other vasculopathies and PACNS mimics is crucial. New diagnostic tools and biomarkers which increase specificity and facilitate the diagnosis for patients with suspected PACNS are highly desirable. This short review summarizes the current procedures within the diagnostic process and aims to illustrate its difficulties and challenges. Furthermore, it highlights emerging biomarkers in the cerebrospinal fluid and peripheral venous blood as well as novel potential imaging tools that may corroborate the diagnosis. With new imaging techniques and a panel of biomarkers the certainty of the diagnosis may be increased and diagnostic processes more accelerated in the future.
中枢神经系统原发性血管炎（PACNS）是一种影响中枢神经系统中小血管的炎性脑病。尽管近期有关PACNS患者的数据增进了对该疾病的认识，但诊断仍然具有挑战性。PACNS的临床表现广泛且缺乏特异性，大多数诊断方法的特点是特异性较低。因此，PACNS常被误诊。此外，由于其潜在的侵袭性病程可能会因适当的免疫抑制治疗而改变，因此将其与其他血管病变及PACNS模仿症区分开来至关重要。非常需要能够提高特异性并有助于疑似PACNS患者诊断的新诊断工具和生物标志物。这篇简短综述总结了诊断过程中的当前程序，旨在阐明其困难和挑战。此外，它还强调了脑脊液和外周静脉血中新兴的生物标志物以及可能有助于确诊的新型潜在成像工具。借助新的成像技术和一系列生物标志物，未来诊断的确定性可能会提高，诊断过程也会更加加速。
