{
  "abstract": "Background Reports of primary angiitis of the central nervous system (PACNS) are mainly restricted to clinically suspected cases, but biopsy-proven ones are rare. Here, we present results from a large multicentre cohort of patients with biopsy-proven PACNS (BP-PACNS). In particular, we provide insights into characteristics and treatment responses of PACNS subtypes.Methods BP-PACNS patients treated between 1999 and 2021 were analysed. The outcome was assessed by the modified Rankin Scale (mRS). Between-group comparisons were performed by Kruskal-Wallis, χ 2 or Fisher’s exact tests.Results In total, 57 patients were analysed (52% male). Of these, n=37 (65%) had a lymphocytic (L-PACNS), n=9 (16%) an amyloid-beta-related angiitis (ABRA), n=8 (14%) a granulomatous (G-PACNS) and n=3 (5%) a necrotising (N-PACNS) PACNS subtype. At the time of diagnosis, age differed significantly between groups (median age (years) L-PACNS 47, ABRA 64.5, G-PACNS 37, N-PACNS 65; p=0.008). The clinical course was mostly monophasic in L-PACNS and ABRA (65% and 75%, respectively), while relapsing-remitting in G-PACNS (63%). Median mRS at last follow up was 2 (IQR 1.25–4) in the study group. Worst outcome (median mRS 4) and highest mortality (25%) were seen in G-PACNS. Good induction treatment response was achieved in 77% of all BP-PACNS patients but was low in those with G-PACNS (29%).Conclusions In this large, multicentre series of BP-PACNS patients, G-PACNS had a worse functional outcome, a predominant relapsing-remitting disease and a poorer response to the induction therapy. An optimal first-line treatment regimen for G-PACNS patients should be further examined and established in larger studies to improve the outcome of G-PACNS patients.",
  "authors": [
    {
      "affiliations": [
        "Department of Neurology and Neurovascular Center, University Hospital Schleswig-Holstein, Kiel, Germany"
      ],
      "name": "Milani Deb-Chatterji"
    },
    {
      "affiliations": [
        "Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany"
      ],
      "name": "Parnia Aghel"
    },
    {
      "affiliations": [
        "Institute for Medical Biometry and Epidemiology, University Hospital Hamburg Eppendorf, Hamburg, Germany"
      ],
      "name": "Hans Pinnschmidt"
    },
    {
      "affiliations": [
        "Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany"
      ],
      "name": "Christina Krüger"
    },
    {
      "affiliations": [
        "Department of Neurology and experimental Neurology, and Center for Stroke Research (CSB), Berlin, Germany",
        "Department of Neurology, Martin Gropius Krankenhaus, Eberswalde, Germany"
      ],
      "name": "Juliane Herm"
    },
    {
      "affiliations": [
        "Department of Vascular Neurology, Center of Neurology, University Hospital Bonn, Bonn, Germany"
      ],
      "name": "Julia Layer"
    },
    {
      "affiliations": [
        "Department of Neurology, Asklepios Fachklinikum Teupitz, Teupitz, Germany"
      ],
      "name": "Hebun Erdur"
    },
    {
      "affiliations": [
        "Department of Vascular Neurology, Center of Neurology, University Hospital Bonn, Bonn, Germany"
      ],
      "name": "Felix J Bode"
    },
    {
      "affiliations": [
        "Department of Neurology and experimental Neurology, and Center for Stroke Research (CSB), Berlin, Germany",
        "Berlin Institute of Health (BIH), Berlin, Germany"
      ],
      "name": "Christian H Nolte"
    },
    {
      "affiliations": [
        "Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany"
      ],
      "name": "Tim Magnus"
    }
  ],
  "title": "Biopsy-proven PACNS: results from the large, multicentre cohort of cerebral vasculitis patients",
  "uid": "3c086f31-05ca-5630-9718-dd7f7c099133"
}