medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/2079-701X-2013-4-79-81medsovet-989Research ArticleПрактикаPracticeПервичный (изолированный) ангиит ЦНСPrimary (isolated) angiitis of the central nervous systemПизоваН. В.PizovaN. V.noemail@neicon.ruЯрославская государственная медицинская академияРоссияYaroslavl State Medical AcademyRussian Federation201330122013047981Copyright © Пизова Н.В., 20132013Пизова Н.В.Pizova N.V.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/989

Первичный (изолированный) ангиит (васкулит) ЦНС (ПАЦНС) – это редкая форма васкулитов неизвестной этиологии, при которой в процесс вовлекаются сосуды мелкого и/или среднего калибра головного и/или спинного мозга и мозговых оболочек.

Primary (isolated) angiitis (vasculitis) of the CNS (PACNS) is a rare form of vasculitis of unknown etiology primarily affecting small and/or medium-sized vessels of the brain and/or spinal cord and meninges [3, 4, 20].

поражения центральной нервной системыголовная больпоражения сосудовангиографияdamage to the central nervous systemheadachevascular lesionsangiographyReferencesAkassoglou K., Douni E., Bauer J., et al. Exclusive tumor necrosis factor (TNF) signaling by the p75TNF receptor triggers inflammatory ischemia in the CNS of transgenic mice // Proc. Natl. Acad. Sci. USA. 2003. №100. P. 709–714.Akassoglou K., Douni E., Bauer J., et al. Exclusive tumor necrosis factor (TNF) signaling by the p75TNF receptor triggers inflammatory ischemia in the CNS of transgenic mice // Proc. Natl. Acad. Sci. USA. 2003. №100. P. 709–714.Amlie-Lefond C., Kleinschmidt-DeMasters B.K., Mahalingam R., et al. The vasculopathy of varicella-zoster virus encephalitis. // Ann. Neurol. 1995. №37. P. 784–790.Amlie-Lefond C., Kleinschmidt-DeMasters B.K., Mahalingam R., et al. The vasculopathy of varicella-zoster virus encephalitis. // Ann. Neurol. 1995. №37. P. 784–790.Birnbaum J., Hellmann D.B. Primary angiitis of the central nervous system. // Arch. Neurol. 2009. №66. P. 704–709.Birnbaum J., Hellmann D.B. Primary angiitis of the central nervous system. // Arch. Neurol. 2009. №66. P. 704–709.Calabrese L.H., Mallek J.A. Primary angiitis of the central nervous system, report of 8 new cases: review of the literature and proposal for diagnostic criteria // Medicine. 1988. №67. P. 20–39.Calabrese L.H., Mallek J.A. Primary angiitis of the central nervous system, report of 8 new cases: review of the literature and proposal for diagnostic criteria // Medicine. 1988. №67. P. 20–39.Calabrese L.H., Duna G.F., Lie J.T. Vasculitis in the central nervous system // Arthritis Rheum. 1997. №40. P. 1189–1201.Calabrese L.H., Duna G.F., Lie J.T. Vasculitis in the central nervous system // Arthritis Rheum. 1997. №40. P. 1189–1201.Calabrese L.H. Infection with the human immunodeficiency virus type 1 and vascular inflammatory disease. Clin. Exp. Rheumatol. 2004;22(l):87-93.Calabrese L.H. Infection with the human immunodeficiency virus type 1 and vascular inflammatory disease. Clin. Exp. Rheumatol. 2004;22(l):87-93.Cloft H.J., Phillips C.D., Dix J.E. et al. Correlation of angiography and MR imaging in cerebral vasculitis. Acta. Radiol. 1999;40:83–7.Cloft H.J., Phillips C.D., Dix J.E. et al. Correlation of angiography and MR imaging in cerebral vasculitis. Acta. Radiol. 1999;40:83–7.de Groot K., Harper L., Jayne D.R. et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann. Intern. Med. 2009;150:670–80.de Groot K., Harper L., Jayne D.R. et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann. Intern. Med. 2009;150:670–80.Gray F., Hurtrel M., Hurtrel B. Early central nervous system changes in human immunodeficiency virus (HIV)-infection. Neuropathol. Appl. Neurobiol. 1993;19;3–9.Gray F., Hurtrel M., Hurtrel B. Early central nervous system changes in human immunodeficiency virus (HIV)-infection. Neuropathol. Appl. Neurobiol. 1993;19;3–9.Greenan, T.J., Grossman R.I., Goldberg H.I. Cerebral vasculitis, MR imaging and angiographic correlation. Radiology. 1992;182:65-72.Greenan, T.J., Grossman R.I., Goldberg H.I. Cerebral vasculitis, MR imaging and angiographic correlation. Radiology. 1992;182:65-72.Hajj-Ali R.A., Calabrese L. Central nervous system vasculitis. Curr. Opin. Rheumatol. 2009;21:10–18.Hajj-Ali R.A., Calabrese L. Central nervous system vasculitis. Curr. Opin. Rheumatol. 2009;21:10–18.Harris K.G., Tran D.D., Sickels W.J. et al. Diagnosing intracranial vasculitis, the roles of MR and angiography. AJNR Am. J. Neuroradiol. 1994;15:317–30.Harris K.G., Tran D.D., Sickels W.J. et al. Diagnosing intracranial vasculitis, the roles of MR and angiography. AJNR Am. J. Neuroradiol. 1994;15:317–30.Hellmann D.B., Roubenoff R., Healy R.A., Wang H. Central nervous system angiography, Safety and predictors of a positive result in 125 consecutive patients evaluated for possible vasculitis. J. Rheumatol. 1992;19:568–72.Hellmann D.B., Roubenoff R., Healy R.A., Wang H. Central nervous system angiography, Safety and predictors of a positive result in 125 consecutive patients evaluated for possible vasculitis. J. Rheumatol. 1992;19:568–72.Hirohata S., Tanimoto K., Ito K. Elevation of cerebrospinal fluid interleukin-6 activity in patients with vasculitides and central nervous system involvement. Clin. Immunol. Immunopathol. 1993;66:225–9.Hirohata S., Tanimoto K., Ito K. Elevation of cerebrospinal fluid interleukin-6 activity in patients with vasculitides and central nervous system involvement. Clin. Immunol. Immunopathol. 1993;66:225–9.Jayne D., Rasmussen N., Andrassy K. et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N. Engl. J. Med. 2003;349:36–44.Jayne D., Rasmussen N., Andrassy K. et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N. Engl. J. Med. 2003;349:36–44.Jones R.B., Cohen-Tervaert J.W., Hauser T. et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N. Engl. J. Med. 2010;363:21–20.Jones R.B., Cohen-Tervaert J.W., Hauser T. et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N. Engl. J. Med. 2010;363:21–20.Kraemer M., Berlit P. Primary central nervous system vasculitis: clinical experiences with 21 new European cases. Rheumatol. Int.Kraemer M., Berlit P. Primary central nervous system vasculitis: clinical experiences with 21 new European cases. Rheumatol. Int.Kraemer M., Berlit P. Systemic, secondary and infectious causes for cerebral vasculitis: clinical experience with 16 new European cases. Rheumatol. Int. 2010;30:1471-6.Kraemer M., Berlit P. Systemic, secondary and infectious causes for cerebral vasculitis: clinical experience with 16 new European cases. Rheumatol. Int. 2010;30:1471-6.Lie J.T. 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.Lie J.T. 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.Lie J.T. Classification and histopathologic spectrum of central nervous system vasculitis. Neurol. Clin. 1997;15:805-19.Lie J.T. Classification and histopathologic spectrum of central nervous system vasculitis. Neurol. Clin. 1997;15:805-19.Linnemann C.C.Jr., Alvira M.M. Pathogenesis of varicella-zoster angiitis in the CNS. Arch. Neurol. 1980;37:239-40.Linnemann C.C.Jr., Alvira M.M. Pathogenesis of varicella-zoster angiitis in the CNS. Arch. Neurol. 1980;37:239-40.MacLaren K., Gillespie J., Shrestha S. et al. Primary angiitis of the central nervous system: emerging variants. Q. J. Med. 2005;98:643-54.MacLaren K., Gillespie J., Shrestha S. et al. Primary angiitis of the central nervous system: emerging variants. Q. J. Med. 2005;98:643-54.Miller D.V., Salvarani C., Hunder G.G. et al. Biopsy findings in primary angiitis of the central nervous system. Am. J. Surg. Pathol. 2009;33:35-43.Miller D.V., Salvarani C., Hunder G.G. et al. Biopsy findings in primary angiitis of the central nervous system. Am. J. Surg. Pathol. 2009;33:35-43.Molloy E.S., Singhal A.B., Calabrese L.H. Tumour-like mass lesion, an under-recognised presentation of primary angiitis of the central nervous system. Ann. Rheum. Dis. 2008;67:1732-35.Molloy E.S., Singhal A.B., Calabrese L.H. Tumour-like mass lesion, an under-recognised presentation of primary angiitis of the central nervous system. Ann. Rheum. Dis. 2008;67:1732-35.Mukhtyar C., Guillevin L., Cid M.C. et al. EULAR recommendations for the management of primary medium vessel vasculitis. Ann. Rheum. Dis. 2009;68:310-17.Mukhtyar C., Guillevin L., Cid M.C. et al. EULAR recommendations for the management of primary medium vessel vasculitis. Ann. Rheum. Dis. 2009;68:310-17.Myung J., Kim B., Yoon B.W. et al. B-cell dominant lymphocytic primary angiitis of the central nervous system, Four biopsy-proven cases. Neuropathology. 2010;30:123-30.Myung J., Kim B., Yoon B.W. et al. B-cell dominant lymphocytic primary angiitis of the central nervous system, Four biopsy-proven cases. Neuropathology. 2010;30:123-30.Pagnoux C., Mahr A., Hamidou M.A. et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N. Engl. J. Med. 2008;359:2790-803.Pagnoux C., Mahr A., Hamidou M.A. et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N. Engl. J. Med. 2008;359:2790-803.Reuter J.D. Cytomegalovirus induces T-cell independent apoptosis in brain during immunodeficiency. J. Clin. Virol. 2005;32:218-23.Reuter J.D. Cytomegalovirus induces T-cell independent apoptosis in brain during immunodeficiency. J. Clin. Virol. 2005;32:218-23.Salvarani C., Brown R.D.Jr., Calamia K.T. et al. Primary central nervous system vasculitis, analysis of 101 patients. Ann. Neurol. 2007;62:442-51.Salvarani C., Brown R.D.Jr., Calamia K.T. et al. Primary central nervous system vasculitis, analysis of 101 patients. Ann. Neurol. 2007;62:442-51.Salvarani C., Brown R.D., Calamia K.T. et al. Primary CNS vasculitis with prominent leptomeningeal enhancement. A subset with a benign outcome. Arthritis. Rheum. 2008;58:595-603.Salvarani C., Brown R.D., Calamia K.T. et al. Primary CNS vasculitis with prominent leptomeningeal enhancement. A subset with a benign outcome. Arthritis. Rheum. 2008;58:595-603.Salvarani C., Brown R.D., Calamia K.T. et al. Angiography negative primary central nervous system vasculitis. A syndrome involving small cerebral vessels. Medicine. 2008;87:264-71.Salvarani C., Brown R.D., Calamia K.T. et al. Angiography negative primary central nervous system vasculitis. A syndrome involving small cerebral vessels. Medicine. 2008;87:264-71.Salvarani C., Brown R.D.Jr., Calamia K.T. et al. Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology. 2008;47:1671-7.Salvarani C., Brown R.D.Jr., Calamia K.T. et al. Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology. 2008;47:1671-7.Salvarani C., Brown R.D.Jr., Calamia K.T. et al. Efficacy of tumor necrosis factor alpha blockade in primary central nervous system vasculitis resistant to immunosuppressive treatment. Arthritis Rheum. 2008;59:291-6.Salvarani C., Brown R.D.Jr., Calamia K.T. et al. Efficacy of tumor necrosis factor alpha blockade in primary central nervous system vasculitis resistant to immunosuppressive treatment. Arthritis Rheum. 2008;59:291-6.Scolding N.J., Jayne D.R., Zajicek J.P. et al. The syndrome of cerebral vasculitis, recognition; diagnosis and management. Q. J. Med. 1997;90:61-73.Scolding N.J., Jayne D.R., Zajicek J.P. et al. The syndrome of cerebral vasculitis, recognition; diagnosis and management. Q. J. Med. 1997;90:61-73.Scolding N.J. Central Nervous System Vasculitis. Semin. Immunopathol. 2009;31:527-36.Scolding N.J. Central Nervous System Vasculitis. Semin. Immunopathol. 2009;31:527-36.Sethna M.P., Lampson L.A. Immune modulation within the brain, recruitment of inflammatory cells and increased major histocompatibility antigen expression following intracerebral injection of interferon-gamma. J. Neuroimmunol. 1991;34:121-32.Sethna M.P., Lampson L.A. Immune modulation within the brain, recruitment of inflammatory cells and increased major histocompatibility antigen expression following intracerebral injection of interferon-gamma. J. Neuroimmunol. 1991;34:121-32.Stone J.H., Pomper M.G., Roubenoff R. et al. Sensitivities of noninvasive tests for central nervous system vasculitis, a comparison of lumbar puncture; computed tomography; and magnetic resonance imaging. J. Rheumatol. 1994;21:1277-82.Stone J.H., Pomper M.G., Roubenoff R. et al. Sensitivities of noninvasive tests for central nervous system vasculitis, a comparison of lumbar puncture; computed tomography; and magnetic resonance imaging. J. Rheumatol. 1994;21:1277-82.Stone J.H., Merkel P.A., Spiera R. et al. Rituximab versus cyclophophamide for ANCA-associated vasculitis. N. Eng. J. Med. 2010;363:221-32.Stone J.H., Merkel P.A., Spiera R. et al. Rituximab versus cyclophophamide for ANCA-associated vasculitis. N. Eng. J. Med. 2010;363:221-32.Tokunaga M., Saito K., Kawabata D. et al. Efficacy of rituximab (anti-CD20) for refractory systemic lupus erythematosus involving the central nervous system. Ann. Rheum. Dis. 2007;66:470-5.Tokunaga M., Saito K., Kawabata D. et al. Efficacy of rituximab (anti-CD20) for refractory systemic lupus erythematosus involving the central nervous system. Ann. Rheum. Dis. 2007;66:470-5.Vassallo R., Remstein E., Parisi J.E. et al. Multiple cerebral infarctions from nonbacterial thrombotic endocarditis mimicking cerebral vasculitis. Mayo Clin. Proc. 1999;74:798-802.Vassallo R., Remstein E., Parisi J.E. et al. Multiple cerebral infarctions from nonbacterial thrombotic endocarditis mimicking cerebral vasculitis. Mayo Clin. Proc. 1999;74:798-802.White M.L., Hadley W.L., Zhang Y., Dogar M.A. Analysis of central nervous system vasculitis with diffusion-weighted imaging and apparent diffusion coefficient mapping of the normalappearing brain. Am. J. Neuroradiol. 2007;28:933-7.White M.L., Hadley W.L., Zhang Y., Dogar M.A. Analysis of central nervous system vasculitis with diffusion-weighted imaging and apparent diffusion coefficient mapping of the normalappearing brain. Am. J. Neuroradiol. 2007;28:933-7.Yankner B.A., Skolnik P.R., Shoukimas G.M. et al. Cerebral granulomatous angiitis associated with isolation of human T-lymphotropic virus type III from the central nervous system. Ann. Neurol. 1986;20:362-4.Yankner B.A., Skolnik P.R., Shoukimas G.M. et al. Cerebral granulomatous angiitis associated with isolation of human T-lymphotropic virus type III from the central nervous system. Ann. Neurol. 1986;20:362-4.

The authors declare that there are no conflicts of interest present.