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Comparative Study
. 2024 Jul 1;63(7):1973-1979.
doi: 10.1093/rheumatology/kead542.

Comparison of patients with biopsy positive and negative primary angiitis of the central nervous system

Ahmad Nehme  1 , Caroline Arquizan  2 , Alexis Régent  3 , Clothilde Isabel  4 , Nelly Dequatre  5 , Benoît Guillon  6 , Jean Capron  7 , Olivier Detante  8 , Sylvain Lanthier  9 , Alexandre Y Poppe  10 , Grégoire Boulouis  11 , Sophie Godard  12 , Benjamin Terrier  3 , Christian Pagnoux  13 , Achille Aouba  14 , Emmanuel Touzé  1 , Hubert de Boysson  14 ; Cohort of Patients with PACNS Study Group
Collaborators, Affiliations
Free article
Comparative Study

Comparison of patients with biopsy positive and negative primary angiitis of the central nervous system

Ahmad Nehme et al. Rheumatology (Oxford). .
Free article

Abstract

Objective: There is limited evidence on when to obtain a central nervous system (CNS) biopsy in suspected primary angiitis of the central nervous system (PACNS). Our objective was to identify which clinical and radiological characteristics were associated with a positive biopsy in PACNS.

Methods: From the multicentre retrospective Cohort of Patients with Primary Vasculitis of the CNS (COVAC), we included adults with PACNS based on a positive CNS biopsy or otherwise unexplained intracranial stenoses with additional findings supportive of vasculitis. Baseline findings were compared between patients with a positive and negative biopsy using logistic regression models.

Results: Two hundred patients with PACNS were included, among which a biopsy was obtained in 100 (50%) and was positive in 61 (31%). Patients with a positive biopsy were more frequently female (odds ratio [OR] 2.90; 95% CI: 1.25, 7.10; P = 0.01) and more often presented with seizures (OR 8.31; 95% CI: 2.77, 33.04; P < 0.001) or cognitive impairment (OR 2.58; 95% CI: 1.11, 6.10; P = 0.03). On imaging, biopsy positive patients more often had non-ischaemic parenchymal or leptomeningeal gadolinium enhancement (OR 52.80; 95% CI: 15.72, 233.06; P < 0.001) or ≥1 cerebral microbleed (OR 8.08; 95% CI: 3.03, 25.13; P < 0.001), and less often had ≥1 acute brain infarct (OR 0.02; 95% CI: 0.004, 0.08; P < 0.001). In the multivariable model, non-ischaemic parenchymal or leptomeningeal gadolinium enhancement (adjusted OR 8.27; 95% CI: 1.78, 38.46; P < 0.01) and absence of ≥1 acute brain infarct (adjusted OR 0.13; 95% CI: 0.03, 0.65; P = 0.01) were significantly associated with a positive biopsy.

Conclusion: Baseline clinical and radiological characteristics differed between biopsy positive and negative PACNS. These results may help physicians individualize the decision to obtain a CNS biopsy in suspected PACNS.

Keywords: biopsy; cerebral vasculitis; primary angiitis of the central nervous system.

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