Baseline features and functional outcomes in primary central nervous system vasculitis: development and validation of a prognostic model
Nehme A., Sylaja PN., Isabel C., Capron J., Arquizan C., Régent A., Guillon B., Dequatre N., Detante O., Casolla B., Levraut M., Gollion C., Kremer L., Lambert N., Derex L., Humbertjean L., Denier C., Kerschen P., Saadoun D., Vannier S., Sibon I., Maarouf A., Weisenburger-Lile D., Sellimi A., Mazighi M., Lanthier S., Poppe AY., Mathon B., Boulouis G., Godard S., Terrier B., Aouba A., Zuber M., Sundaram S., Paramsivam N., Briant A., Parienti J-J., Touzé E., Pagnoux C., de Boysson H., Zéphir H., Néel A., Mékinian A., Quénardelle V., Machado S., Bresch S., Hosseini H., Comarmond-Ortoli C., Sablot D., Audemard-Verger A., Obadia M., Pico F., Cantagrel P., Graveleau J., Poncet-Megemont L., Ancel T., Peeters A.
Abstract Objectives Few data are available regarding the functional prognosis of adults with primary central nervous system vasculitis (PCNSV). We developed and validated a prognostic model for 12-month functional independence in adults with PCNSV. Methods We conducted a multicentre, international cohort study of adults with PCNSV (COVAC’). The primary end point was functional independence 12 months after the start of corticosteroids or immunosuppressants, defined as a modified Rankin Scale of 0–2. We identified baseline features independently associated with functional independence using multivariable analyses. We assessed discrimination using AUC-ROC and externally validated the model in a geographically distinct, single-centre cohort from India. Results Among the 206 patients included with PCNSV (mean age: 48 years; 41% female), 67 (33%) were diagnosed based on a positive biopsy. At 12 months, 135 (66%) patients were functionally independent and 12 (6%) had died. Favourable prognostic factors were ≥1 intracranial stenosis on CT- or MR-angiogram (OR = 2.99, 95% CI: 1.23–7.68) and headache (OR = 2.67, 95% CI: 1.31–5.59). Unfavourable prognostic factors were an altered level of consciousness (OR = 0.09, 95% CI: 0.02–0.31), ≥1 acute brain infarct (OR = 0.12, 95% CI: 0.04–0.33) and cognitive impairment (OR = 0.22, 95% CI: 0.10–0.46). A prognostic model including these five variables had an AUC of 0.80 (95% CI: 0.74–0.87) in the derivation cohort and 0.67 (95% CI: 0.54–0.81) in the validation cohort. Conclusions Baseline clinical and imaging variables may predict 12-month functional independence in adults with PCNSV. These results may support physicians in prognostication and risk stratification of adults with PCNSV.