Disease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System—A Multicenter Cohort Study in 163 Patients

Fisse AL, Bonberg N, Beuker C, Pfeuffer C, Heidenreich A, Krüger C, Deb-Chatterji M, Becker J, Gerner S, Küpper C, Nitsch L, Kestner RI, Krause LU, Herm J, Katalinic A, Karch A, Gold R, Wiendl H, Schäbitz WR, Petzold GC, Pfeilschifter W, Ringelstein M, Doeppner TR, Kellert L, Huttner HB, Kraemer M, Magnus T, Haeusler KG, Minnerup H, Minnerup J (2025)

Publication Type: Journal article

Publication year: 2025

Journal

DOI: 10.1002/ana.27295

Abstract

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.

Authors with CRIS profile

Involved external institutions

Katholisches Klinikum Bochum (St. Josef- und St. Elisabeth-Hospital gGmbH) Germany (DE) Westfälische Wilhelms-Universität (WWU) Münster Germany (DE) Universität zu Lübeck Germany (DE) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) Alfried Krupp Krankenhaus Germany (DE) kbo-Isar-Amper-Klinikum gemeinnützige GmbH Germany (DE) Universitätsklinikum Bonn Germany (DE) Universitätsklinikum Frankfurt am Main (KGU) Germany (DE) Klinikum Osnabrück Germany (DE) Charité - Universitätsmedizin Berlin Germany (DE) Universitätsklinikum Freiburg Germany (DE) Universität Bielefeld Germany (DE) Universitätsklinikum Düsseldorf Germany (DE) Justus-Liebig-Universität Gießen Germany (DE) Klinikum der Universität München Germany (DE) Universitätsklinikum Würzburg Germany (DE)

How to cite

APA:

Fisse, A.L., Bonberg, N., Beuker, C., Pfeuffer, C., Heidenreich, A., Krüger, C.,... Minnerup, J. (2025). Disease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System—A Multicenter Cohort Study in 163 Patients. Annals of Neurology. https://doi.org/10.1002/ana.27295

MLA:

Fisse, Anna Lena, et al. "Disease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System—A Multicenter Cohort Study in 163 Patients." Annals of Neurology (2025).

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