CNS Vasculitis

Abstract

Central nervous system vasculitis (CNSV) is a complex disease that poses considerable diagnostic and therapeutic challenges. It is divided into primary angiitis of the CNS (PACNS), or secondary angiitis of the CNS when associated with systemic conditions. Clinical presentation can be extremely variable and there is no classic presentation. In addition, there is no single diagnostic test and the sensitivity/specificity of all currently available tests is suboptimal. As such, an exhaustive approach with thorough historical data, physical examination, and corroborating investigations should be performed to exclude or confirm a diagnosis of CNSV. Infectious, neoplastic, and autoimmune conditions should be carefully evaluated. Knowledge of CNSV mimics is crucial given the therapeutic and prognostic implications. Reversible cerebral vasoconstriction syndrome is now recognized as the most common mimicker of PACNS. Much progress has been made in the understanding of PACNS histopathology, and specific clinical subsets and their response to treatment. Contrary to historical reports, recent PACNS cohorts achieved favorable clinical outcomes when early diagnosis and prompt treatment was initiated. Successful treatment with newer drugs has also been reported in PACNS cases. Newer imaging modalities hold promise for distinguishing inflammatory from noninflammatory vascular lesions. In this review, the authors provide a broad overview with updates on the types of adult CNSV along with its clinical assessment, diagnosis, and treatment.

Keywords

CNS vasculitis - primary angiitis of the CNS - secondary angiitis of the CNS - CNS vasculitis mimics - reversible cerebral vasoconstriction syndrome

• References

• 1 Salvarani C, Brown Jr RD, Calamia KT , et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007; 62 (5) 442-451
• 2 Salvarani C, Brown Jr RD, Hunder GG. Adult primary central nervous system vasculitis. Lancet 2012; 380 (9843) 767-777
• 3 Benseler SM, Silverman E, Aviv RI , et al. Primary central nervous system vasculitis in children. Arthritis Rheum 2006; 54 (4) 1291-1297
• 4 de Boysson H, Zuber M, Naggara O , et al; fortheFrenchVasculitisStudyGroup(FVSG) and the French NeuroVascular Society (SFNV). Revised primary angiitis of the central nervous system: description of the first 52 adults enrolled in the french COVAC' cohort. Arthritis Rheum 2014;
• 5 Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum 1997; 40 (7) 1189-1201
• 6 Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol 2011; 10 (6) 561-572
• 7 John S, Hajj-Ali RA. Headache in autoimmune diseases. Headache 2014; 54 (3) 572-582
• 8 Lie JT. Angiitis of the central nervous system. Curr Opin Rheumatol 1991; 3 (1) 36-45
• 9 Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 1988; 67 (1) 20-39
• 10 Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009; 66 (6) 704-709
• 11 Miller DV, Salvarani C, Hunder GG , et al. Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol 2009; 33 (1) 35-43
• 12 Salvarani C, Brown Jr RD, Calamia KT , et al. Primary central nervous system vasculitis presenting with intracranial hemorrhage. Arthritis Rheum 2011; 63 (11) 3598-3606
• 13 Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012; 11 (10) 906-917
• 14 Provenzale JM, Allen NB. Neuroradiologic findings in polyarteritis nodosa. AJNR Am J Neuroradiol 1996; 17 (6) 1119-1126
• 15 Honda H, Hasegawa T, Morokawa N, Kato N, Inoue K. [A case of MPO-ANCA related vasculitis with transient leukoencephalopathy and multiple cerebral hemorrhage]. Rinsho Shinkeigaku 1996; 36 (9) 1089-1094
• 16 Han S, Rehman HU, Jayaratne PS, Carty JE. Microscopic polyangiitis complicated by cerebral haemorrhage. Rheumatol Int 2006; 26 (11) 1057-1060
• 17 Ito Y, Suzuki K, Yamazaki T, Yoshizawa T, Ohkoshi N, Matsumura A. ANCA-associated vasculitis (AAV) causing bilateral cerebral infarction and subsequent intracerebral hemorrhage without renal and respiratory dysfunction. J Neurol Sci 2006; 240 (1-2) 99-101
• 18 Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 1993; 33 (1) 4-9
• 19 Borhani Haghighi A, Pourmand R, Nikseresht AR. Neuro-Behçet disease. A review. Neurologist 2005; 11 (2) 80-89
• 20 Chow FC, Marra CM, Cho TA. Cerebrovascular disease in central nervous system infections. Semin Neurol 2011; 31 (3) 286-306
• 21 Pfister HW, Borasio GD, Dirnagl U, Bauer M, Einhäupl KM. Cerebrovascular complications of bacterial meningitis in adults. Neurology 1992; 42 (8) 1497-1504
• 22 Levi M, van der Poll T, Büller HR. Bidirectional relation between inflammation and coagulation. Circulation 2004; 109 (22) 2698-2704
• 23 Vergouwen MD, Schut ES, Troost D, van de Beek D. Diffuse cerebral intravascular coagulation and cerebral infarction in pneumococcal meningitis. Neurocrit Care 2010; 13 (2) 217-227
• 24 Czartoski T, Hallam D, Lacy JM, Chun MR, Becker K. Postinfectious vasculopathy with evolution to moyamoya syndrome. J Neurol Neurosurg Psychiatry 2005; 76 (2) 256-259
• 25 Holmes MD, Brant-Zawadzki MM, Simon RP. Clinical features of meningovascular syphilis. Neurology 1984; 34 (4) 553-556
• 26 Topakian R, Stieglbauer K, Nussbaumer K, Aichner FT. Cerebral vasculitis and stroke in Lyme neuroborreliosis. Two case reports and review of current knowledge. Cerebrovasc Dis 2008; 26 (5) 455-461
• 27 Calabrese LH, Estes M, Yen-Lieberman B , et al. Systemic vasculitis in association with human immunodeficiency virus infection. Arthritis Rheum 1989; 32 (5) 569-576
• 28 Calabrese LH. Infection with the human immunodeficiency virus type 1 and vascular inflammatory disease. Clin Exp Rheumatol 2004; ; 22 (6) (Suppl. 36) S87-S93
• 29 Mazzoni P, Chiriboga CA, Millar WS, Rogers A. Intracerebral aneurysms in human immunodeficiency virus infection: case report and literature review. Pediatr Neurol 2000; 23 (3) 252-255
• 30 Nagel MA, Cohrs RJ, Mahalingam R , et al. The varicella zoster virus vasculopathies: clinical, CSF, imaging, and virologic features. Neurology 2008; 70 (11) 853-860
• 31 O'Dwyer JP, Al-Moyeed BA, Farrell MA , et al. Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature. Eur J Neurol 2013; 20 (1) 71-78
• 32 Ellis SG, Verity MA. Central nervous system involvement in systemic lupus erythematosus: a review of neuropathologic findings in 57 cases, 1955–1977. Semin Arthritis Rheum 1979; 8 (3) 212-221
• 33 Watts RA, Mooney J, Lane SE, Scott DG. Rheumatoid vasculitis: becoming extinct?. Rheumatology (Oxford) 2004; 43 (7) 920-923
• 34 Citron BP, Halpern M, McCarron M , et al. Necrotizing angiitis associated with drug abuse. N Engl J Med 1970; 283 (19) 1003-1011
• 35 Singhal AB, Hajj-Ali RA, Topcuoglu MA , et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol 2011; 68 (8) 1005-1012
• 36 Rennebohm R, Susac JO, Egan RA, Daroff RB. Susac's syndrome—update. J Neurol Sci 2010; 299 (1-2) 86-91
• 37 Joutel A, Corpechot C, Ducros A , et al. Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia. Nature 1996; 383 (6602) 707-710
• 38 Provenzale JM, Barboriak DP, Allen NB, Ortel TL. Antiphospholipid antibodies: findings at arteriography. AJNR Am J Neuroradiol 1998; 19 (4) 611-616
• 39 Pomper MG, Miller TJ, Stone JH, Tidmore WC, Hellmann DB. CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography. AJNR Am J Neuroradiol 1999; 20 (1) 75-85
• 40 Hurst RW, Grossman RI. Neuroradiology of central nervous system vasculitis. Semin Neurol 1994; 14 (4) 320-340
• 41 Appenzeller S, Faria AV, Zanardi VA, Fernandes SR, Costallat LT, Cendes F. Vascular involvement of the central nervous system and systemic diseases: etiologies and MRI findings. Rheumatol Int 2008; 28 (12) 1229-1237
• 42 Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OA. Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges. Arch Neurol 1993; 50 (9) 925-930
• 43 Alhalabi M, Moore PM. Serial angiography in isolated angiitis of the central nervous system. Neurology 1994; 44 (7) 1221-1226
• 44 Duna GF, Calabrese LH. Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system. J Rheumatol 1995; 22 (4) 662-667
• 45 MacLaren K, Gillespie J, Shrestha S, Neary D, Ballardie FW. Primary angiitis of the central nervous system: emerging variants. QJM 2005; 98 (9) 643-654
• 46 Kadkhodayan Y, Alreshaid A, Moran CJ, Cross III DT, Powers WJ, Derdeyn CP. Primary angiitis of the central nervous system at conventional angiography. Radiology 2004; 233 (3) 878-882
• 47 Obusez EC, Hui F, Hajj-Ali RA , et al. High-resolution MRI vessel wall imaging: spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system vasculitis. AJNR Am J Neuroradiol 2014;
• 48 Cupps TR, Moore PM, Fauci AS. Isolated angiitis of the central nervous system. Prospective diagnostic and therapeutic experience. Am J Med 1983; 74 (1) 97-105
• 49 Parisi JE, Moore PM. The role of biopsy in vasculitis of the central nervous system. Semin Neurol 1994; 14 (4) 341-348
• 50 Alrawi A, Trobe JD, Blaivas M, Musch DC. Brain biopsy in primary angiitis of the central nervous system. Neurology 1999; 53 (4) 858-860
• 51 Molloy ES, Langford CA. Advances in the treatment of small vessel vasculitis. Rheum Dis Clin North Am 2006; 32 (1) 157-172 , xx.
• 52 Salvarani C, Brown Jr RD, Calamia KT , et al. Efficacy of tumor necrosis factor alpha blockade in primary central nervous system vasculitis resistant to immunosuppressive treatment. Arthritis Rheum 2008; 59 (2) 291-296
• 53 Salvarani C, Brown Jr RD, Huston III J, Morris JM, Hunder GG. Treatment of primary CNS vasculitis with rituximab: case report. Neurology 2014; 82 (14) 1287-1288
• 54 Moore PM. Diagnosis and management of isolated angiitis of the central nervous system. Neurology 1989; 39 (2 Pt 1) 167-173