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

• Published in: Rheumatology (Oxford, England) Vol. 47; no. 11; pp. 1671 - 1677
• Main Authors: Salvarani, C., Brown, R. D., Calamia, K. T., Christianson, T. J. H., Huston, J., Meschia, J. F., Giannini, C., Miller, D. V., Hunder, G. G.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.11.2008; Oxford Publishing Limited (England)
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Amyloid beta-Peptides - analysis; Biological and medical sciences; Brain - pathology; Brain Chemistry; Cardiovascular system; Cerebral amyloid angiopathy; Cerebral Amyloid Angiopathy - diagnostic imaging; Cerebral Amyloid Angiopathy - drug therapy; Cerebral Amyloid Angiopathy - pathology; Cerebral Angiography; Cerebral biopsy; Diseases of the osteoarticular system; Female; Follow-Up Studies; Gadolinium; Glucocorticoids - therapeutic use; Humans; Investigative techniques, diagnostic techniques (general aspects); Magnetic Resonance Imaging; Male; Medical sciences; Middle Aged; Prednisone - therapeutic use; Primary central nervous system vasculitis; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Radiopharmaceuticals; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Therapy; Treatment Outcome; Vasculitis, Central Nervous System - diagnostic imaging; Vasculitis, Central Nervous System - drug therapy; Vasculitis, Central Nervous System - pathology; Young Adult; Therapy; Cerebral amyloid angiopathy; Primary central nervous system vasculitis; Cerebral biopsy; Cerebral angiography; Human; Radiodiagnosis; Angiography; Rheumatology; Central nervous system; Cardiovascular disease; Encephalon; Vascular disease; Treatment; Vasculitis; Biopsy; Comparative study
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/ken328

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.