Rapidly progressive primary central nervous system vasculitis

Carlo Salvarani, Robert D. Brown, Kenneth T. Calamia, Teresa J H Christianson, John Huston, James F. Meschia, Caterina Giannini, Dylan V. Miller, Gene G. Hunder

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

Objective: To describe a subset of cases in a large cohort of patients with primary CNS vasculitis (PCNSV) who appear to have a rapidly progressive clinical course. Method: In the present study, we use our updated cohort of 131 consecutive patients with PCNSV seen over the 25-year period of 1983-2007 at Mayo Clinic, Rochester, MN, USA. The diagnosis of PCNSV was based on brain/spinal cord biopsy or cerebral angiography. The modified Rankin scale was used to identify rapidly progressive disease and included patients with Rankin scores indicating severe disability or death at diagnosis or within 6 months after the diagnosis. We compared patients with rapidly progressive disease to those without. Results: Compared with the 120 patients without rapidly progressive vasculitis, the 11 patients with rapidly progressive vasculitis more frequently had paraparesis/quadriparesis at presentation, angiographic presence of bilateral, large-vessel vasculitis and MRI evidence of cerebral infarctions; those infarctions were more frequently multiple and bilateral, and more frequently involved both the cortex and subcortical regions on initial MRI. Granulomatous and/or necrotizing histopathological patterns of vasculitis were observed in patients with positive biopsies. Conclusion: Rapidly progressive PCNSV appears to form a subset of PCNSV at the worst end of the clinical spectrum of this vasculitis, characterized by bilateral, multiple, large cerebral vessel lesions and multiple CNS infarctions.

Original languageEnglish
Article numberkeq303
Pages (from-to)349-358
Number of pages10
JournalRheumatology
Volume50
Issue number2
DOIs
Publication statusPublished - Feb 2011

Fingerprint

Central Nervous System Vasculitis
Vasculitis
Infarction
Paraparesis
Biopsy
Cerebral Angiography
Quadriplegia
Cerebral Infarction
Spinal Cord
Brain

Keywords

  • Brain MRI
  • Cerebral angiography
  • Cerebral infarcts
  • Primary central nervous system vasculitis
  • Therapy

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

Cite this

Salvarani, C., Brown, R. D., Calamia, K. T., Christianson, T. J. H., Huston, J., Meschia, J. F., ... Hunder, G. G. (2011). Rapidly progressive primary central nervous system vasculitis. Rheumatology, 50(2), 349-358. [keq303]. https://doi.org/10.1093/rheumatology/keq303

Rapidly progressive primary central nervous system vasculitis. / Salvarani, Carlo; Brown, Robert D.; Calamia, Kenneth T.; Christianson, Teresa J H; Huston, John; Meschia, James F.; Giannini, Caterina; Miller, Dylan V.; Hunder, Gene G.

In: Rheumatology, Vol. 50, No. 2, keq303, 02.2011, p. 349-358.

Research output: Contribution to journalArticle

Salvarani, C, Brown, RD, Calamia, KT, Christianson, TJH, Huston, J, Meschia, JF, Giannini, C, Miller, DV & Hunder, GG 2011, 'Rapidly progressive primary central nervous system vasculitis', Rheumatology, vol. 50, no. 2, keq303, pp. 349-358. https://doi.org/10.1093/rheumatology/keq303
Salvarani C, Brown RD, Calamia KT, Christianson TJH, Huston J, Meschia JF et al. Rapidly progressive primary central nervous system vasculitis. Rheumatology. 2011 Feb;50(2):349-358. keq303. https://doi.org/10.1093/rheumatology/keq303
Salvarani, Carlo ; Brown, Robert D. ; Calamia, Kenneth T. ; Christianson, Teresa J H ; Huston, John ; Meschia, James F. ; Giannini, Caterina ; Miller, Dylan V. ; Hunder, Gene G. / Rapidly progressive primary central nervous system vasculitis. In: Rheumatology. 2011 ; Vol. 50, No. 2. pp. 349-358.
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