TY - JOUR
T1 - Disease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System-A Multicenter Cohort Study in 163 Patients
AU - Fisse, Anna Lena
AU - Bonberg, Nadine
AU - Beuker, Carolin
AU - Pfeuffer, Claudia
AU - Heidenreich, Andreas
AU - Krüger, Christina
AU - Deb-Chatterji, Milani
AU - Becker, Jana
AU - Gerner, Stefan T
AU - Küpper, Clemens
AU - Nitsch, Louisa
AU - Kestner, Roxane-Isabelle
AU - Krause, Lars Udo
AU - Herm, Juliane
AU - Katalinic, Alexander
AU - Karch, André
AU - Gold, Ralf
AU - Wiendl, Heinz
AU - Schäbitz, Wolf-Rüdiger
AU - Petzold, Gabor C
AU - Pfeilschifter, Waltraud
AU - Ringelstein, Marius
AU - Doeppner, Thorsten R
AU - Kellert, Lars
AU - Huttner, Hagen B
AU - Kraemer, Markus
AU - Magnus, Tim
AU - Haeusler, Karl Georg
AU - Minnerup, Heike
AU - Minnerup, Jens
N1 - Publisher Copyright:
© 2025 The Author(s). Annals of Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.
PY - 2025
Y1 - 2025
N2 - OBJECTIVE: The aim was to determine patient, disease, and treatment characteristics associated with outcome in patients with primary angiitis of the central nervous system (PACNS) in a large multicenter German cohort.METHODS: In a retrospective, observational cohort study, we analyzed 163 adult patients who met the diagnostic criteria for PACNS. Data were collected from January 2004 to December 2018 in 13 tertiary care centers in Germany. Survival, recurrence-free survival and long-term global disability were assessed.RESULTS: Of 163 patients with PACNS (median [interquartile range (IQR)] age 48 [39-59.5] years; 73 [45%] women), 29 (18%) died, 84 (52%) had a poor outcome (modified Rankin scale [mRS] 3-6 at last follow-up), and 82 (50%) patients relapsed. Poorer survival was associated with patient age (hazard ratio [HR], 1.96 [95% confidence interval (CI), 1.41-2.74] per 10 years), longer time between initial manifestation and diagnosis (HR, 1.01 [95% CI, 1.00-1.01] per month), and necrotizing subtype (HR, 10.2 [95% CI, 2.18-48.2]). Long-term disability was associated with older age (odds ratio [OR], 1.40 [95% CI, 1.07-1.86] per 10 years), and worse mRS score at diagnosis (OR, 4.43 [95% CI, 1.97-10.4]). Patients treated with cyclophosphamide alone or in combination with steroids had a lower incidence of relapse than untreated patients (HR, 0.44 [95% CI, 0.22-0.86]; HR, 0.47 [95% CI, 0.24-0.92]).INTERPRETATION: In patients with PACNS, the long-term outcome depends on several patient and disease characteristics. Our results favor treatment with cyclophosphamide alone or in combination with steroids, because this was associated with a reduction in the relapse rate. ANN NEUROL 2025.
AB - OBJECTIVE: The aim was to determine patient, disease, and treatment characteristics associated with outcome in patients with primary angiitis of the central nervous system (PACNS) in a large multicenter German cohort.METHODS: In a retrospective, observational cohort study, we analyzed 163 adult patients who met the diagnostic criteria for PACNS. Data were collected from January 2004 to December 2018 in 13 tertiary care centers in Germany. Survival, recurrence-free survival and long-term global disability were assessed.RESULTS: Of 163 patients with PACNS (median [interquartile range (IQR)] age 48 [39-59.5] years; 73 [45%] women), 29 (18%) died, 84 (52%) had a poor outcome (modified Rankin scale [mRS] 3-6 at last follow-up), and 82 (50%) patients relapsed. Poorer survival was associated with patient age (hazard ratio [HR], 1.96 [95% confidence interval (CI), 1.41-2.74] per 10 years), longer time between initial manifestation and diagnosis (HR, 1.01 [95% CI, 1.00-1.01] per month), and necrotizing subtype (HR, 10.2 [95% CI, 2.18-48.2]). Long-term disability was associated with older age (odds ratio [OR], 1.40 [95% CI, 1.07-1.86] per 10 years), and worse mRS score at diagnosis (OR, 4.43 [95% CI, 1.97-10.4]). Patients treated with cyclophosphamide alone or in combination with steroids had a lower incidence of relapse than untreated patients (HR, 0.44 [95% CI, 0.22-0.86]; HR, 0.47 [95% CI, 0.24-0.92]).INTERPRETATION: In patients with PACNS, the long-term outcome depends on several patient and disease characteristics. Our results favor treatment with cyclophosphamide alone or in combination with steroids, because this was associated with a reduction in the relapse rate. ANN NEUROL 2025.
UR - http://www.scopus.com/inward/record.url?scp=105008750639&partnerID=8YFLogxK
UR - https://www.mendeley.com/catalogue/76a5f68c-8b86-3949-950f-b5abcd4b46fc/
U2 - 10.1002/ana.27295
DO - 10.1002/ana.27295
M3 - Journal articles
C2 - 40546217
SN - 0364-5134
JO - Annals of Neurology
JF - Annals of Neurology
ER -