TY - JOUR
T1 - Primary central nervous system vasculitis mimicking brain tumor
T2 - Comprehensive analysis of 13 cases from a single institutional cohort of 191 cases
AU - Salvarani, Carlo
AU - Brown, Robert D.
AU - Christianson, Teresa J.H.
AU - Huston, John
AU - Morris, Jonathan M.
AU - Giannini, Caterina
AU - Hunder, Gene G.
N1 - Publisher Copyright:
© 2018 Elsevier Ltd
PY - 2019/2
Y1 - 2019/2
N2 - Objective: To describe the clinical, laboratory, and imaging features and course of patients with primary central nervous system vasculitis (PCNSV) presenting with an intracranial tumor-like mass (TLM). Methods: We retrospectively studied a cohort of 191 consecutive patients with PCNSV seen at the Mayo Clinic, Rochester, MN over a 35-year period (1982–2017). 13/191 patients presented with a TLM. We compared the findings in these 13 patients with those from the 178 without this presentation. Results: In 13 of 191 (6.8%) patients with TLM the diagnosis of PCNSV was established by cerebral biopsy. Granulomatous vasculitis was found in 11/13 patients, accompanied by vascular deposits of β-amyloid peptide in 7. Compared to the 178 patients without TLM, the patients with TLM were more likely to be male (p = 0.04), and less likely to have a transient ischemic attack (p = 0.023), bilateral cerebral infarcts (p = 0.018), or vasculitic lesions on angiography (p = 0.045). They were more likely to have seizures (p = 0.022), gadolinium-enhanced lesions (p = 0.007), and amyloid angiopathy (p = 0.046). All 13 patients responded to therapy and 8/13 (61.5%) had a Rankin disability score of 0 at last visit. Overall, high disability scores (Rankin scores 4–6) at last follow-up were associated with increasing age (odds ratio, OR, 1.49) and cerebral infarction (OR, 3.47), but were less likely in patients with gadolinium-enhanced lesions (OR, 0.36) and amyloid angiopathy (OR, 0.21). Conclusion: In PCNSV a TLM at presentation represents a definable subgroup of patients with a favourable treatment response.
AB - Objective: To describe the clinical, laboratory, and imaging features and course of patients with primary central nervous system vasculitis (PCNSV) presenting with an intracranial tumor-like mass (TLM). Methods: We retrospectively studied a cohort of 191 consecutive patients with PCNSV seen at the Mayo Clinic, Rochester, MN over a 35-year period (1982–2017). 13/191 patients presented with a TLM. We compared the findings in these 13 patients with those from the 178 without this presentation. Results: In 13 of 191 (6.8%) patients with TLM the diagnosis of PCNSV was established by cerebral biopsy. Granulomatous vasculitis was found in 11/13 patients, accompanied by vascular deposits of β-amyloid peptide in 7. Compared to the 178 patients without TLM, the patients with TLM were more likely to be male (p = 0.04), and less likely to have a transient ischemic attack (p = 0.023), bilateral cerebral infarcts (p = 0.018), or vasculitic lesions on angiography (p = 0.045). They were more likely to have seizures (p = 0.022), gadolinium-enhanced lesions (p = 0.007), and amyloid angiopathy (p = 0.046). All 13 patients responded to therapy and 8/13 (61.5%) had a Rankin disability score of 0 at last visit. Overall, high disability scores (Rankin scores 4–6) at last follow-up were associated with increasing age (odds ratio, OR, 1.49) and cerebral infarction (OR, 3.47), but were less likely in patients with gadolinium-enhanced lesions (OR, 0.36) and amyloid angiopathy (OR, 0.21). Conclusion: In PCNSV a TLM at presentation represents a definable subgroup of patients with a favourable treatment response.
KW - Cerebral amyloid angiopathy
KW - Cerebral vasculitis
KW - Disability
KW - PCNSV
KW - Prognosis
KW - Prognostic factors
KW - Tumor
KW - Tumor-like mass
UR - http://www.scopus.com/inward/record.url?scp=85060571122&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85060571122&partnerID=8YFLogxK
U2 - 10.1016/j.jaut.2018.10.001
DO - 10.1016/j.jaut.2018.10.001
M3 - Article
C2 - 30528173
AN - SCOPUS:85060571122
SN - 0896-8411
VL - 97
SP - 22
EP - 28
JO - Journal of Autoimmunity
JF - Journal of Autoimmunity
ER -