Artículos

/ Publicado el 17 de septiembre de 2012

Enfermedad infrecuente de causa desconocida

Vasculitis primaria del sistema nervioso central en el adulto

Su diagnóstico temprano es importante porque el tratamiento con corticoides, junto con citotóxicos o sin ellos, a menudo evita resultados adversos.

Autor/a: Dres. Salvarani C, Brown Jr RD, Hunder GG.

Fuente: Lancet 2012; 380: 767–77.

Indice
1. Referencias
2. Artículo

Referencias
1 Cravioto H, Feigin I. Noninfectious granulomatous angiitis with a predilection for the nervous system. Neurology 1959; 9: 599–609.
2 Harbitz F. Unknown forms of arteritis, with special reference to their relation to syphilitic arteritis and periarteritis nodosa.
Am J Med Sci 1922; 163: 250–72.
3 Sandhu R, Alexander WS, Hornabrook RW, Stehbens WE. Granulomatous angiitis of the CNS. Arch Neurol 1979; 36: 433–35.
4 Harrison PE Jr. Granulomatous angiitis of the central nervous system. Case report and review. J Neurol Sci 1976; 29: 335–41.
5 Newman W, Wolf A. Non-infectious granulomatous angiitis involving
the central nervous system. Trans Am Neurol Assoc 1952; 56: 114–17.
6 Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OAF. Idiopathic granulomatous angiitis of the central nervous system: diagnostic challenges. Arch Neurol 1993; 50: 925–30.
7 McCormick HM, Neubuerger KT. Giant-cell arteritis involving small meningeal and intracerebral vessels. J Neuropathol Exp Neurol 1958; 17: 471–78.
8 Cupps TR, Moore PM, Fauci AS. Isolated angiitis of the central nervous system. Prospective diagnostic and therapeutic experience. Am J Med 1983; 74: 97–105.
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: 20–39.
10 Calabrese LH, Gragg LA, Furlan AJ. Benign angiopathy: a distinct
subset of angiographically defi ned primary angiitis of the central nervous system. J Rheumatol 1993; 20: 2046–50.
11 Lie JT. Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature. Hum Pathol 1992; 23: 164–71.
12 Moore PM. Diagnosis and management of isolated angiitis of the central nervous system. Neurology 1989; 39: 167–73.
13 Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med 2007; 146: 34–44.
14 Benseler SM, Silverman E, Aviv RI, et al. Primary central nervous system vasculitis in children. Arthritis Rheum 2006; 54: 1291–97.
15 Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous
system vasculitis: analysis of 101 patients. Ann Neurol 2007; 62: 442–51.
16 Duna GF, Calabrese LH. Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system. J Rheumatol 1995; 22: 662–67.
17 Harris KG, Tran DD, Sickels WJ, Cornell SH, Yuh WT. Diagnosing
intracranial vasculitis: the roles of MR and angiography. AJNR Am J Neuroradiol 1994; 15: 317–30.
18 Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009; 66: 704–09.
19 Swartz RH, Bhuta SS, Farb RI, et al. Intracranial arterial wall imaging using high-resolution 3-tesla contrast-enhanced MRI. Neurology 2009; 72: 627–34.
20 Kadkhodayan Y, Alreshaid A, Moran CJ, Cross DT 3rd, Powers WJ,
Derdeyn CP. Primary angiitis of the central nervous system at conventional angiography. Radiology 2004; 233: 878–82.
21 Salvarani C, Brown RD Jr, Calamia KT, et al. Angiography-negative
primary central nervous system vasculitis: a syndrome involving small cerebral vessels. Medicine (Baltimore) 2008; 87: 264–71.
22 Parisi JE, Moore PM. The role of biopsy in vasculitis of the central nervous system. Semin Neurol 1994; 4: 341–48.
23 Miller DV, Salvarani C, Hunder GG, et al. Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol 2009; 33: 35–43.
24 Alrawi A, Trobe JD, Blaivas M, Musch DC. Brain biopsy in primary angiitis of the central nervous system. Neurology 1999; 53: 858–60.
25 Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum 1997; 40: 1189–201.
26 Nagel MA, Cohrs RJ, Mahalingam R, et al. The varicella zoster virus
vasculopathies: clinical, CSF, imaging, and virologic features. Neurology 2008; 70: 853–60.
27 Thomas L, Davidson M, McCluskey RT. Studies of PPLO infection. I. The production of cerebral polyarteritis by Mycoplasma gallisepticum in turkeys; the neurotoxic property of the mycoplasma. J Exp Med 1966; 123: 897–912.
28 Arthur G, Margolis G. Mycoplasma-like structures in granulomatous
angiitis of the central nervous system. Case reports with light and electron microscopic studies. Arch Pathol Lab Med 1977; 101: 382–87.
29 Iwase T, Ojika K, Mitake S, et al. Involvement of CD45RO+ T lymphocyte
infiltration in a patient with primary angiitis of the central nervous system restricted to small vessels. Eur Neurol 2001; 45: 184–85.
30 Williams PL, Leib SL, Kamberi P, et al. Levels of matrix metalloproteinase-9 within cerebrospinal fl uid in a rabbit model of coccidioidal meningitis and vasculitis. J Infect Dis 2002; 186: 1692–95.
31 Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology (Oxford) 2008; 47: 1671–77.
32 Winkler DT, Bondolfi L, Herzig MC, et al. Spontaneous hemorrhagic stroke in a mouse model of cerebral amyloid angiopathy. J Neurosci 2001; 21: 1619–27.
33 Eng JA, Frosch MP, Choi K, Rebeck GW, Greenberg SM. Clinical manifestations of cerebral amyloid angiopathy-related infl ammation. Ann Neurol 2004; 55: 250–56.
34 Lie JT. Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature. Hum Pathol 1992; 23: 164–71.
35 Salvarani C, Brown RD Jr, Calamia KT, et al. Primary CNS vasculitis with spinal cord involvement. Neurology 2008; 70: 2394–400.
36 Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH. Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum 2002; 47: 662–69.
37 Woolfenden AR, Tong DC, Marks MP, Ali AO, Albers GW. Angiographically defi ned primary angiitis of the CNS: is it really benign? Neurology 1998; 51: 183–88.
38 Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous system vasculitis with prominent leptomeningeal enhancement: a subset with a benign outcome. Arthritis Rheum 2008; 58: 595–603.
39 Salvarani C, Brown RD Jr, Calamia KT, et al. Rapidly progressive primary central nervous system vasculitis. Rheumatology (Oxford) 2001; 50: 349–58.
40 Molloy ES, Singhal AB, Calabrese LH. Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system. Ann Rheum Dis 2008; 67: 1732–35.
41 Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous system vasculitis presenting with intracranial hemorrhage. Arthritis Rheum 2011; 63: 3598–606.
42 Ferris EJ, Levine HL. Cerebral arteritis: classifi cation. Radiology 1983; 109: 327–41.
43 Alhalabi M, Moore PM. Serial angiography in isolated angiitis of the central nervous system. Neurology 1994; 44: 1221–26.
44 Eleftheriou D, Cox T, Saunders D, Klein NJ, Brogan PA, Ganesan V.
Investigation of childhood central nervous system vasculitis: magnetic resonance angiography versus catheter cerebral angiography. Dev Med Child Neurol 2010; 52: 863–67.
45 Pomper MG, Miller TJ, Stone JH, Tidmore WC, Hellmann DB. CNS vasculitis in autoimmune disease: MR imaging fi ndings and correlation with angiography. AJNR Am J Neuroradiol 1999; 20: 75–85.
46 Wasserman BA, Stone JH, Hellmann DB, Pomper MG. Reliability of normal fi ndings on MR imaging for excluding the diagnosis of vasculitis of the central nervous system. AJR Am J Roentgenol 2001; 177: 455–59.
47 Cloft HJ, Phillips CD, Dix JE, McNulty BC, Zagardo MT, Kallmes D. Correlation of angiography and MR imaging in cerebral vasculitis. Acta Radiol 1999; 40: 83–87.
48 Greenan TJ, Grossman RI, Goldberg HI. Cerebral vasculitis: MR imaging and angiographic correlation. Radiology 1992; 182: 65–72.
49 Bekiesińska-Figatowska M. T2-hyperintense foci on brain MR imaging. Med Sci Monit 2004; 10 (suppl 3): 80–87.
50 Kuker W, Gaertner S, Nagele T, et al. Vessel wall contrast enhancement: a diagnostic sign of cerebral vasculitis. Cerebrovasc Dis 2008; 26: 23–29.
51 Shoemaker EI, Lin ZS, Rae-Grant AD, Little B. Primary angiitis of the central nervous system: unusual MR appearance. AJNR Am J Neuroradiol 1994; 15: 331–34.
52 Salvarani C, Brown RD Jr, Huston J 3rd, Hunder GG. Prominent perivascular enhancement in primary central nervous system vasculitis. Clin Exp Rheumatol 2008; 26 (suppl 49): S111.
53 Kuker W. Cerebral vasculitis: imaging signs revisited. Neuroradiology 2007; 49: 471–79.
54 White ML, Hadley WL, Zhang Y, Dogar MA. Analysis of central nervous system vasculitis with diff usion-weighted imaging and apparent diff usion
coefficient mapping of the normal-appearing brain. AJNR Am J Neuroradiol 2007; 28: 933–37.
55 Hou WH, Liu X, Duan YY, et al. Evaluation of transcranial color-coded duplex sonography for cerebral artery stenosis or occlusion. Cerebrovasc Dis 2009; 27: 479–84.
56 Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol 2009; 9: 256–67.
57 Melica G, Brugieres P, Lascaux AS, Levy Y, Lelievre JD. Primary
vasculitis of the central nervous system in patients infected with HIV-1 in the HAART era. J Med Virol 2009; 81: 578–81.
58 Gaa J, Weidauer S, Sitzer M, Lanfermann H, Zanella FE. Cerebral
vasculitis due to Treponema pallidum infection: MRI and MRA findings. Eur Radiol 2004; 14: 746–47.
59 Kakumani PL, Hajj-Ali RA. A forgotten cause of central nervous system vasculitis. J Rheumatol 2009; 36: 655.
60 Cacoub P, Saadoun D, Limal N, Leger JM, Maisonobe T. Hepatitis C
virus infection and mixed cryoglobulinaemia vasculitis: a review of neurological complications. AIDS 2005; 19 (suppl 3): S128–34.
61 Bilge I, Sadikoğlu B, Emre S, Sirin A, Aydin K, Tatli B. Central nervous system vasculitis secondary to parvovirus B19 infection in a pediatric renal transplant patient. Pediatr Nephrol 2005; 20: 529–33.
62 Golden MP, Hammer SM, Wanke CA, Albrecht MA. Cytomegalovirus vasculitis. Case reports and review of the literature. Medicine (Baltimore) 1994; 73: 246–55.
63 Ovetchkine P, Brugieres P, Seradj A, Reinert P, Cohen R. An 8-y-old
boy with acute stroke and radiological signs of cerebral vasculitis after recent Mycoplasma pneumoniae infection. Scand J Infect Dis 2002; 34: 307–09.
64 Heinrich A, Khaw AV, Ahrens N, Kirsch M, Dressel A. Cerebral vasculitis as the only manifestation of Borrelia burgdorferi infection in a 17-year-old patient with basal ganglia infarction. Eur Neurol 2003; 50: 109–12.
65 Starke JR. Tuberculosis of the central nervous system in children. Semin Pediatr Neurol 1999; 6: 318–31.
66 Marra CM. Neurologic complications of Bartonella henselae infection. Curr Opin Neurol 1995; 8: 164–69.
67 Bleck TP. Central nervous system involvement in rickettsial diseases. Neurol Clin 1999; 17: 801–12.
68 Zeller V, Lortholary O. Vasculitis secondary to fungal infections. Presse Med 2004; 33: 1385–88.
69 Nagashima T, Miyanoshita A, Sakiyama Y, Ozaki Y, Stan AC, Nagashima K. Cerebral vasculitis in chronic mucocutaneous candidosis: autopsy case report. Neuropathology 2000; 20: 309–14.
70 Barinagarrementeria F, Cantu C. Frequency of cerebral arteritis in subarachnoid cysticercosis: an angiographic study. Stroke 1998; 29: 123–25.
71 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: 4–9.
72 Ghinoi A, Zuccoli G, Pipitone N, Salvarani C. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis involving the central nervous system: case report and review of the literature. Clin Exp Rheumatol 2010; 28: 759–66.
73 Sehgal M, Swanson JW, DeRemee RA, Colby TV. Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc 1995; 70: 337–41.
74 Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999; 78: 26–37.
75 Al-Araji A, Kidd DP. Neuro-Behcet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 2009; 8: 192–204.
76 Oran I, Memis A, Parildar M, Yunten N. Multiple intracranial aneurysms in polyarteritis nodosa: MRI and angiography. Neuroradiology 1999; 41: 436–39.
77 Goncalves C, Ferreira G, Mota C, Vilarinho A. Cerebral vasculitis in Henoch-Schonlein purpura. An Pediatr (Barc) 2004; 60: 188–89 (in Spanish).
78 Tabarki B, Mahdhaoui A, Selmi H, Yacoub M, Essoussi AS. Kawasaki disease with predominant central nervous system involvement. Pediatr Neurol 2001; 25: 239–41.
79 Salvarani C, Giannini C, Miller DV, Hunder G. Giant cell arteritis: Involvement of intracranial arteries. Arthritis Rheum 2006; 55: 985–89.
80 Ringleb PA, Strittmatter EI, Loewer M, et al. Cerebrovascular manifestations of Takayasu arteritis in Europe. Rheumatology 2005; 44: 1012–15.
81 Hirohata S. Histopathology of central nervous system lesions in Behcet’s disease. J Neurol Sci 2008; 267: 41–47.
82 Hanly JG, Walsh NM, Sangalang V. Brain pathology in systemic lupus erythematosus. J Rheumatol 1992; 19: 732–41.
83 Everett CM, Graves TD, Lad S, et al. Aggressive CNS lupus vasculitis in the absence of systemic disease activity. Rheumatology (Oxford) 2008; 47: 107–09.
84 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: 212–21.
85 Caballol Pons N, Montala N, Valverde J, Brell M, Ferrer I, Martinez-Yelamos S. Isolated cerebral vasculitis associated with rheumatoid arthritis. Joint Bone Spine 2010; 77: 361–63.
86 Ramos-Casals M, Solans R, Rosas J, et al, for the GEMESS Study Group. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore) 2008; 87: 210–19.
87 Niemela RK, Hakala M. Primary Sjogren’s syndrome with severe central nervous system disease. Semin Arthritis Rheum 1999; 29: 4–13.
88 Regan M, Haque U, Pomper M, Pardo C, Stone J. Central nervous system vasculitis as a complication of refractory dermatomyositis. J Rheumatol 2001; 28: 207–11.
89 Graf WD, Milstein JM, Sherry DD. Stroke and mixed connective tissue disease. J Child Neurol 1993; 8: 256–59.
90 Provenzale JM, Barboriak DP, Allen NB, Ortel TL. Antiphospholipid antibodies: findings at arteriography. AJNR Am J Neuroradiol 1998; 19: 611–16.
91 Rosen CL, DePalma L, Morita A. Primary angiitis of the central nervous system as a fi rst presentation in Hodgkin’s disease: a case report and review of the literature. Neurosurgery 2000; 46: 1504–08.
92 Borenstein D, Costa M, Jannotta F, Rizzoli H. Localized isolated angiitis of the central nervous system associated with primary intracerebral lymphoma. Cancer 1988; 62: 375–80.
93 Zuckerman D, Seliem R, Hochberg E. Intravascular lymphoma: the oncologist’s “great imitator”. Oncologist 2006; 11: 496–502.
94 Lawrence WP, Gammal T, Pool WH, et al. Radiological manifestations of neurosarcoidosis. Report of three cases and review of the literature. Clin Radiol 1974; 25: 343–48.
95 Scheid R, Teich N. Neurologic manifestations of ulcerative colitis. Eur J Neurol 2007; 14: 483–92.
96 Ma M, Barnes G, Pulliam J, Jezek D, Baumann RJ, Berger JR. CNS angiitis in graft vs host disease. Neurology 2002; 59: 1994–97.
97 Berlit P. Isolated angiitis of the CNS and bacterial endocarditis: similarities and diff erences. J Neurol 2009; 256: 792–95.
98 Katchanov J, Siebert E, Klingebiel R, Endres M. Infectious vasculopathy of intracranial large- and medium-sized vessels in neurological intensive care unit: a clinico-radiological study. Neurocrit Care 2010; 12: 369–74.
99 Calabrese LH, Duna GF. Drug-induced vasculitis. Curr Opin Rheumatol 1996; 8: 34–40.
100 Griffi n J, Price DL, Davis L, McKhann GM. Granulomatous angiitis of the central nervous system with aneurysms on multiple cerebral arteries. Trans Am Neurol Assoc 1973; 98: 145–48.
101 Fauci AS, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med 1978; 89: 660–76.
102 Molloy ES, Langford CA. Advances in the treatment of small vessel vasculitis. Rheum Dis Clin North Am 2006; 32: 157–72.
103 Salvarani C, Brown RD Jr, 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: 291–96.
104 Sen ES, Leone V, Abinun M, et al. Treatment of primary angiitis of the central nervous system in childhood with mycophenolate mofetil. Rheumatology (Oxford) 2010; 49: 806–11.
105 Holle JU, Gross WL. Neurological involvement in Wegener’s granulomatosis. Curr Opin Rheumatol 2011; 23: 7–11.