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Rheumatology Advance Access originally published online on August 27, 2008
Rheumatology 2008 47(11):1671-1677; doi:10.1093/rheumatology/ken328
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy

C. Salvarani1, R. D. Brown, Jr2, K. T. Calamia3, T. J. H. Christianson4, J. Huston, III5, J. F. Meschia6, C. Giannini7, D. V. Miller7 and G. G. Hunder8

1Department of Neurology, Mayo Clinic, Rochester, MN, USA, 2Department of Neurology, Mayo Clinic, Rochester, MN, 3Division of Rheumatology, Mayo Clinic, Jacksonville, FL, 4Division of Biostatistics, 5Department of Radiology, Mayo Clinic, Rochester, MN, 6Department of Neurology, Mayo Clinic, Jacksonville, FL, 7Division of Anatomic Pathology and 8Division of Rheumatology, Mayo Clinic, Rochester, MN, USA.

Correspondence to: R. D. Brown Jr, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. E-mail: brown{at}mayo.edu


  Abstract

Objectives. To describe the clinical features and outcomes of patients with primary central nervous system vasculitis (PCNSV) and cerebral amyloid angiopathy (CAA) from a large cohort of consecutive patients with PCNSV treated at a single institution.

Methods. We identified 101 consecutive patients with PCNSV admitted between January 1983 and December 2003. PCNSV diagnoses were based on findings from a central nervous system (CNS) biopsy (n = 31) and conventional angiography (n = 70). CNS tissue specimens from 49 cases were examined histologically, and 49 were stained for amyloid deposits. Those with vascular amyloid deposits (CAA) were compared with those without histological evidence of amyloid deposition.

Results. Eight cases (26%) with CNS biopsy specimens positive for PCNSV also showed findings of CAA. Compared with patients with PCNSV only, these patients were older at diagnosis, predominantly male, had a more acute onset, a higher frequency of cognitive dysfunction and showed prominent gadolinium-enhanced leptomeningeal lesions with MRI. Histologically, all had a granulomatous vascular inflammatory pattern. Six patients responded promptly to therapy. Outcomes at last follow-up were similar in the two groups.

Conclusions. PCNSV with CAA appears to form a clinical subset of PCNSV. The vasculitis influences the clinical findings to a greater degree than the presence of amyloid deposits in the vessels.

KEY WORDS: Cerebral amyloid angiopathy, Cerebral angiography, Cerebral biopsy, Primary central nervous system vasculitis, Therapy

Present address: C. Salvarani, Unitá Operativa di Reumatologia, Arcispedale S. Maria Nuova, Reggio Emilia, Italy.

Submitted 19 October 2007; revised version accepted 11 July 2008.
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