Yeo Jina, Hwang Inpyeong, Sohn Chul-Ho, Lee Eunyoung Emily, Lee Soon-Tae, Lee Eun Bong, Park Jin Kyun
Division of Rheumatology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea.
Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital and Seoul National University Hospital College of Medicine, Seoul, South Korea.
Front Med (Lausanne). 2022 Jun 20;9:913203. doi: 10.3389/fmed.2022.913203. eCollection 2022.
Proliferative vasculopathy (PV) associated antiphospholipid syndrome (APS) in the central nervous system is a rare un(der)recognized form of extra-criteria manifestations of APS. This study investigated the angiographic characteristics of cerebral and cervical arteries in patients with PV associated with antiphospholipid antibodies (aPLs).
Patients with aPLs, neurologic symptoms and diffuse luminal narrowing on brain or neck magnetic resonance angiography were selected from electronic medical records. Vascular wall and intraluminal pathology were examined by high-resolution vessel wall MR imaging (VW-MRI).
A total of 11 patients (six men and five women) with PV-aPL, of median (interquartile range) age 42 (34-61) years, were included. Median anticardiolipin antibodies IgG titer was 78.9 (28.2-134.0) units and anti-beta 2 glycoprotein I antibodies (aB2GPIs) IgG titer was 227.2 (0.0-1012.1) units. All patients had diffuse luminal narrowing in the carotid basilar and/or cerebral arteries, five in the internal carotid artery (ICA); two each in the middle cerebral artery (MCA) and vertebral artery; and one each in the basilar artery (BA) and posterior cerebral artery. On VW-MRI, four patients showed concentric thickening of the vascular walls of the ICA and/or MCA and two showed mild eccentric wall thickening of the ICA or BA. All patients received antithrombotic treatment. In two patients with extremely high aB2GPIs titer, diffuse narrowing progressed despite treatment with antithrombotic agents on follow-up imaging.
This study suggests that PV-aPL might be a distinct extra-criteria manifestation of APS that can manifest as long-segmental diffuse stenosis of cerebral and cervical arteries. It should be considered in relatively young patients with neurologic symptoms and aPLs.
中枢神经系统增殖性血管病（PV）相关抗磷脂综合征（APS）是APS一种罕见且未被充分认识的额外标准表现形式。本研究调查了抗磷脂抗体（aPLs）相关PV患者脑动脉和颈动脉的血管造影特征。
从电子病历中选取有aPLs、神经系统症状且脑或颈部磁共振血管造影显示弥漫性管腔狭窄的患者。通过高分辨率血管壁磁共振成像（VW-MRI）检查血管壁和管腔内病理情况。
共纳入11例PV-aPL患者（6例男性和5例女性），年龄中位数（四分位间距）为42（34 - 61）岁。抗心磷脂抗体IgG滴度中位数为78.9（28.2 - 134.0）单位，抗β2糖蛋白I抗体（aB2GPIs）IgG滴度中位数为227.2（0.0 - 1012.1）单位。所有患者在颈内动脉基底动脉和/或脑动脉均有弥漫性管腔狭窄，其中5例累及颈内动脉（ICA）；大脑中动脉（MCA）和椎动脉各2例；基底动脉（BA）和大脑后动脉各1例。在VW-MRI上，4例患者显示ICA和/或MCA血管壁同心性增厚，2例显示ICA或BA轻度偏心性壁增厚。所有患者均接受了抗栓治疗。在2例aB2GPIs滴度极高的患者中，尽管在随访成像中接受了抗栓药物治疗，但弥漫性狭窄仍有进展。
本研究表明，PV-aPL可能是APS一种独特的额外标准表现形式，可表现为脑动脉和颈动脉的长节段弥漫性狭窄。对于有神经系统症状和aPLs的相对年轻患者应予以考虑。
