Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms

Jina Yeo; Inpyeong Hwang; Chul-Ho Sohn; Eunyoung Emily Lee; Soon-Tae Lee; Eun Bong Lee; Jin Kyun Park

doi:10.3389/fmed.2022.913203

Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms

Front Med (Lausanne). 2022 Jun 20:9:913203. doi: 10.3389/fmed.2022.913203. eCollection 2022.

Authors

Jina Yeo^{1

2}, Inpyeong Hwang³, Chul-Ho Sohn^{3

4}, Eunyoung Emily Lee^{2

5}, Soon-Tae Lee⁶, Eun Bong Lee², Jin Kyun Park²

Affiliations

¹ Division of Rheumatology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea.
² Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital and Seoul National University Hospital College of Medicine, Seoul, South Korea.
³ Department of Radiology, Seoul National University Hospital, Seoul, South Korea.
⁴ Department of Radiology, Seoul National University College of Medicine, and Institute of Radiation Medicine, Seoul National University Medical Research Center, Seoul, South Korea.
⁵ Division of Rheumatology, Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, South Korea.
⁶ Department of Neurology, Seoul National University Hospital and College of Medicine, Seoul, South Korea.

Abstract

Background: 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).

Methods: 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).

Results: 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.

Conclusions: 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.

Keywords: CNS vasculopathy; antiphospholipid syndrome; magnetic resonance angiography; proliferative vasculopathy; stenosis and obstruction.