Evidence map›Paper›PMID 40334177›Full record

ArticleNeurology(R) neuroimmunology & neuroinflammation2025

Biopsy-Confirmed Small Vessel Primary CNS Vasculitis: Clinical Features and Impact of Early Intensive Treatment on Remission.

Sumanth P Reddy, Sara C LaHue, Sarah Goglin, Sharon A Chung, Shane Poole, Riley Bove, Melike Pekmezci, Tarik Tihan, Jeffrey M Gelfand

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Article in Neurology(R) neuroimmunology & neuroinflammation, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

3 citing papers in PubMed.

  1. Article
  2. Article
  3. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Sumanth P ReddyUCSF Weill Institute for Neurosciences, Department of Neurology, University of California, San Francisco.ORCID 0000-0002-6275-6001
Sara C LaHueUCSF Weill Institute for Neurosciences, Department of Neurology, University of California, San Francisco.
Sarah GoglinDivision of Rheumatology, Department of Medicine, University of California, San Francisco; and.
Sharon A ChungDivision of Rheumatology, Department of Medicine, University of California, San Francisco; and.
Shane PooleUCSF Weill Institute for Neurosciences, Department of Neurology, University of California, San Francisco.ORCID 0000-0002-3203-151X
Riley BoveUCSF Weill Institute for Neurosciences, Department of Neurology, University of California, San Francisco.ORCID 0000-0002-2034-8800
Melike PekmezciNeuropathology Division, Department of Pathology, University of California, San Francisco.
Tarik TihanNeuropathology Division, Department of Pathology, University of California, San Francisco.ORCID 0000-0003-4551-2555
Jeffrey M GelfandUCSF Weill Institute for Neurosciences, Department of Neurology, University of California, San Francisco.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

BACKGROUND AND

objectivesThis single-center retrospective cohort study sought to characterize the clinical spectrum of small vessel predominant primary CNS vasculitis (sv-PCNSV) and to investigate the impact of early intensive immunosuppressive therapy on remission.

methodsWe analyzed data of patients diagnosed with biopsy-proven sv-PCNSV at our institution between 2009 and 2023. "Early intensive treatment" (EIT) was defined by cyclophosphamide therapy within 3 months of immunosuppressive treatment initiation. Patients in the "escalation treatment" (ESC) group initially received glucocorticoids, either as monotherapy or in conjunction with azathioprine, mycophenolate mofetil, or methotrexate.

resultsTwenty-six patients (50% female) met the study criteria, including 7 with amyloid-beta-related angiitis (ABRA). The median age at onset was 55.5 years (range 20-82), and headache (76.9%) and altered mental status (61.5%) were common presenting symptoms. Neuroimaging commonly showed bihemispheric T2/FLAIR lesions (77%) and abnormal gadolinium enhancement (88.5%), but intracranial vascular irregularities indicating large or medium vessel involvement were rare (11.5%). Among patients with non-ABRA sv-PCNSV (n = 19), some demonstrated spinal cord involvement (15.8%) and others exhibited isolated unihemispheric disease (21.1%). Although CSF testing (n = 23) often demonstrated mild pleocytosis, a notable minority of patients (17.4%) had a normal CSF analysis. Six patients (23.1%) underwent repeat brain biopsy because of initial nondiagnostic findings. Remission was achieved in all patients in the EIT group (n = 12/12), in contrast to 78.6% of patients in the ESC group (n = 11/14). Time to remission was significantly shorter among patients in the EIT group compared with the ESC group (median 5 vs 19 months, hazard ratio = 0.24, 95% CI [0.10-0.63], DISCUSSION: This study highlights the considerable clinical heterogeneity in sv-PCNSV, a rare but serious condition. Early, aggressive treatment with cyclophosphamide is associated with a shorter time to remission, and further validation in prospective studies is warranted.

Indexed as

GlucocorticoidsImmunosuppressive AgentsVasculitis, Central Nervous SystemAdultAgedAged, 80 and overBiopsyCyclophosphamideFemaleHumansMaleMiddle AgedRemission InductionRetrospective StudiesYoung AdultCyclophosphamideGlucocorticoidsImmunosuppressive Agents

Identifiers

PMID40334177
PMCPMC12063240

What Socratic holds

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LicenceCC BY-NC-ND
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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.