Adult primary central nervous system vasculitis treatment and course: Analysis of one hundred sixty-three patients

Carlo Salvarani, Robert D. Brown, Teresa J H Christianson, John Huston, Caterina Giannini, Dylan V. Miller, Gene G. Hunder

Research output: Contribution to journalArticle

Abstract

Objective To describe the treatment and outcomes of patients with primary central nervous system (CNS) vasculitis. Methods We retrospectively studied a cohort of 163 consecutive patients with primary CNS vasculitis who were seen at the Mayo Clinic over a 29-year period. We analyzed treatments, treatment responses, and factors predictive of outcomes. Results A favorable response was observed in 85% of patients treated with prednisone alone and in 80% of patients treated with prednisone and cyclophosphamide. Relapses were observed in 27% of patients, and 25% of patients had discontinued therapy by the time of the last followup visit. Treatment with prednisone alone was associated with more frequent relapses (odds ratio [OR] 2.90), while large vessel involvement (OR 6.14) and cerebral infarcts at the time of diagnosis (OR 3.32) were associated with a poor response to treatment. Prominent gadolinium-enhanced cerebral lesions or meninges were linked with continued treatment at the last followup encounter (OR 2.28). Higher disability scores at the last followup visit were associated with increasing age at the time of diagnosis (OR 1.44) and cerebral infarctions (OR 3.74), while lower disability scores were associated with gadolinium-enhanced cerebral lesions or meninges (OR 0.35) and cerebral amyloid angiopathy (OR 0.24). Increased mortality was associated with increasing age at diagnosis (hazard ratio [HR] 1.39), diagnosis by angiography (HR 3.28), cerebral infarction (HR 4.44), and large vessel involvement (HR 4.98), while reduced mortality was associated with gadolinium-enhanced cerebral lesions or meninges (HR 0.20). Conclusion The majority of patients with primary CNS vasculitis responded to treatment. Recognition of findings at diagnosis that predict the course or outcome may aid in decision-making regarding therapy.

Original languageEnglish
Pages (from-to)1637-1645
Number of pages9
JournalArthritis and Rheumatology
Volume67
Issue number6
DOIs
Publication statusPublished - Jun 1 2015

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Central Nervous System Vasculitis
Odds Ratio
Meninges
Gadolinium
Prednisone
Cerebral Infarction
Therapeutics
Cerebral Amyloid Angiopathy
Recurrence
Mortality
Cyclophosphamide
Decision Making
Angiography

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Rheumatology
  • Medicine(all)

Cite this

Salvarani, C., Brown, R. D., Christianson, T. J. H., Huston, J., Giannini, C., Miller, D. V., & Hunder, G. G. (2015). Adult primary central nervous system vasculitis treatment and course: Analysis of one hundred sixty-three patients. Arthritis and Rheumatology, 67(6), 1637-1645. https://doi.org/10.1002/art.39068

Adult primary central nervous system vasculitis treatment and course : Analysis of one hundred sixty-three patients. / Salvarani, Carlo; Brown, Robert D.; Christianson, Teresa J H; Huston, John; Giannini, Caterina; Miller, Dylan V.; Hunder, Gene G.

In: Arthritis and Rheumatology, Vol. 67, No. 6, 01.06.2015, p. 1637-1645.

Research output: Contribution to journalArticle

Salvarani, C, Brown, RD, Christianson, TJH, Huston, J, Giannini, C, Miller, DV & Hunder, GG 2015, 'Adult primary central nervous system vasculitis treatment and course: Analysis of one hundred sixty-three patients', Arthritis and Rheumatology, vol. 67, no. 6, pp. 1637-1645. https://doi.org/10.1002/art.39068
Salvarani, Carlo ; Brown, Robert D. ; Christianson, Teresa J H ; Huston, John ; Giannini, Caterina ; Miller, Dylan V. ; Hunder, Gene G. / Adult primary central nervous system vasculitis treatment and course : Analysis of one hundred sixty-three patients. In: Arthritis and Rheumatology. 2015 ; Vol. 67, No. 6. pp. 1637-1645.
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N2 - Objective To describe the treatment and outcomes of patients with primary central nervous system (CNS) vasculitis. Methods We retrospectively studied a cohort of 163 consecutive patients with primary CNS vasculitis who were seen at the Mayo Clinic over a 29-year period. We analyzed treatments, treatment responses, and factors predictive of outcomes. Results A favorable response was observed in 85% of patients treated with prednisone alone and in 80% of patients treated with prednisone and cyclophosphamide. Relapses were observed in 27% of patients, and 25% of patients had discontinued therapy by the time of the last followup visit. Treatment with prednisone alone was associated with more frequent relapses (odds ratio [OR] 2.90), while large vessel involvement (OR 6.14) and cerebral infarcts at the time of diagnosis (OR 3.32) were associated with a poor response to treatment. Prominent gadolinium-enhanced cerebral lesions or meninges were linked with continued treatment at the last followup encounter (OR 2.28). Higher disability scores at the last followup visit were associated with increasing age at the time of diagnosis (OR 1.44) and cerebral infarctions (OR 3.74), while lower disability scores were associated with gadolinium-enhanced cerebral lesions or meninges (OR 0.35) and cerebral amyloid angiopathy (OR 0.24). Increased mortality was associated with increasing age at diagnosis (hazard ratio [HR] 1.39), diagnosis by angiography (HR 3.28), cerebral infarction (HR 4.44), and large vessel involvement (HR 4.98), while reduced mortality was associated with gadolinium-enhanced cerebral lesions or meninges (HR 0.20). Conclusion The majority of patients with primary CNS vasculitis responded to treatment. Recognition of findings at diagnosis that predict the course or outcome may aid in decision-making regarding therapy.

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