Observational studyAnnals of neurology2025
Disease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System-A Multicenter Cohort Study in 163 Patients.
Observational study in Annals of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
2 citing papers in PubMed.
- Primary central nervous system vasculitis: an update.Journal of neurology · 2026Review
- A comparative study of large-vessel and small-vessel primary angiitis of the central nervous system: insights from a Chinese single-center retrospective cohort.Frontiers in immunology · 2025Article
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Authors and funding
30 authors.
Funding
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Abstract
objectiveThe 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.
methodsIn 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.
resultsOf 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]).
interpretationIn 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;98:883-893.
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