Comparison of patients with biopsy positive and negative primary angiitis of the central nervous system
Ahmad Nehme, Caroline Arquizan, Alexis Régent, Clothilde Isabel, Nelly Dequatre, B. Guillon, Jean Capron, Olivier Detante, Sylvain Lanthier, Alexandre Y. Poppe, Grégoire Boulouis, Sophie Godard, Benjamin Terrier, Christian Pagnoux, Achille Aouba, Emmanuel Touzé, Hubert de Boysson, the Cohort of Patients with PACNS Study Group, Xavier Ayrignac, Hélène Zéphir, A. Néel, A. Mékinian, Laurent Létourneau‐Guillon, Laurent Derex, Mathieu Gerfaud‐Valentin, Véronique Quenardelle, Laurent Kremer, Cédric Gollion, David Weisenburger, M. Vautier, Stéphanie Machado, Adil Maarouf, Lisa Humbertjean, Stéphane Vannier, Christian Denier, Barbara Casolla, Saskia Bresch, Michaël Levraut, Hassan Hosseini, Nicolas Lambert, Marie Subréville, T. Papo, Igor Sibon, Chloé Comarmond-Ortoli, Mikaël Mazighi, Philippe Kerschen, Denis Sablot, Marie Gaudron, Alexandra Audemard‐Verger, F. Closs-Prophette, Jérémie Papassin, L. Daelman, Mathieu Zuber, Michaël Obadia, Fernando Pico, Paul Cantagrel, J. Graveleau, Louis Poncet‐Megemont, Thomas Ancel
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.