Clinical characteristics, inflammation and coagulation status in patients with immunological disease-related chronic cerebrospinal venous insufficiency

Abstract

Background: Immunological disease-related chronic cerebrospinal venous insufficiency (CCSVI) is rarely reported. This study aimed to analyze clinical characteristics, inflammation, and coagulation status in patients with immunological disease-related CCSVI.Methods: Patients with CCSVI were enrolled from 2017 to 2019 and divided into three cohorts based on their immunological disease backgrounds, including groups with confirmed autoimmune disease, with suspected/subclinical autoimmune disease, and with non-immunological etiology. Immunological, inflammatory, and thrombophilia biomarker assay in blood samples were obtained. Mann-Whitney U test or Fisher's exact test was used to compare continuous variables or categorical variables between the CCSVI patients with or without the immunological etiology. Spearman's correlation analysis was conducted among age, baseline neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), interleukin-6 (IL-6), C-reactive protein (CRP), and neuron-specific enolase (NSE) in the three groups.Results: A total of 255 consecutive patients with CCSVI were enrolled, including three subgroups: CCSVI with confirmed autoimmune disease (n=41), CCSVI with suspected/subclinical autoimmune disease (n=116) and CCSVI with non-immunological etiology (n=98). In the first subgroup, a series of 41 cases was confirmed with eight different autoimmune diseases including antiphospholipid syndrome (n=18), Sjögren's syndrome (n=8), immunoglobulin G4-related disease (n=7), Behçet's disease (n=2), autoimmune hepatitis (n=2), Wegener's granulomatosis (n=2), systemic sclerosis (n=1) and AQP4 antibody-positive neuromyelitis optica spectrum disorder (n=1). Groups with immunological etiology did not show a higher incidence of thrombophilia or increased proinflammatory biomarkers (e.g., neutrophil, IL-6). However, patients with non-immunological etiology had a higher baseline level of CRP. Additionally, baseline PLR was moderately correlated to NLR and CRP in CCSVI patients with non-immunological etiology and suspected/subclinical autoimmune disease. Conclusions:The formation of CCSVI may be based on the inflammatory process, facilitated by multiple risk factors, among which medical history of immunological diseases may play a significant role due to the intricate relationship between inflammation and coagulation. Moreover, CCSVI may also cause an independent inflammatory injury in venous walls, leading to focal stenosis or thrombus, without attacks from autoimmune antibodies.

Safety and efficacy comparison between OACs plus single antiplatelet and dual antiplatelet therapy in patients with cerebral venous sinus stenosis poststenting

“…Fortunately, these patients achieved complete revascularization with adequate anticoagulation [ 26 ]. Our previous study revealed that the majority of patients with CVSS had a history of hypercoagulability, immune inflammation, thrombophilia, or thrombosis [ 27 ]. Although stenting could correct the local stenosis immediately, it could not correct their systemic thrombophilia.…”

Optic nerve sheath fenestration for visual impairment in cerebral venous diseases

“…Venous thrombosis often originates from sinus thrombosis; venous thrombosis alone is rare. Various genetic or secondary thrombogenic factors can be the etiology of cerebral venous stenosis, including Factor V Leiden mutation; thrombin G20210A mutation; hyperhomocysteinemia; protein S, protein C, or antithrombin III defect; pregnancy; puerperium; oral contraceptives; obesity; blood disease; infection; trauma; tumor; dehydration; connective tissue disease; nephrotic syndrome; neurosurgery; and COVID-19 related etiology (2)(3)(4)(5)(6)(7)(8).…”

Intensive mannitol slow infusion post‐stenting may attenuate stenting‐related early adverse effects in patients with cerebral venous sinus stenosis

“…However, stenting‐related early adverse effects, such as severe headache, intracranial hemorrhage, and restenosis are still completely eradicate, which largely deteriorate clinical outcomes 6,7,9–11,15 . In addition, the inflammatory process may cause restenosis of CVSS resulting in poor outcomes 16,17 …”

“… 6 , 7 , 9 , 10 , 11 , 15 In addition, the inflammatory process may cause restenosis of CVSS resulting in poor outcomes. 16 , 17 …”

“…6,7,[9][10][11]15 In addition, the inflammatory process may cause restenosis of CVSS resulting in poor outcomes. 16,17 We have noticed that even though the obstruction of venous reflow was corrected, and the mean pressure gradient (MPG) across the stenotic segment was diminished or even disappeared after stenting, patients had even higher serum levels of inflammatory biomarkers and increased brain edema in the initial days post-stenting compared to their baseline, which may explain why patients with CVSS had even more aggravated symptoms in the early period poststenting. Whereby, inhibiting stenting-related inflammation and brain edema may facilitate clinical outcomes.…”