Abstract
The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal neurological manifestations were similar in both groups, headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA), antineutrophil cytoplasmic antibodies (ANCA), low complement, and rheumatoid factor were more frequent in SCNSV (p < 0.05). In cerebrospinal fluid, pleocytosis and proteins were higher in PCNSV (p < 0.05). Periventricular and subcortical hyperintense lesions were observed in cranial magnetic resonance imaging in both vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in all patients in both groups. CB showed gliosis and lymphocytic infiltration within and around the walls in four patients and granulomatous infiltration in the other patients. After treatment, the Kaplan-Meier survival curve showed a higher relapse-free survival in PCNSV (p < 0.05). Neurological manifestations and neuroimaging findings were similar in both groups of vasculitides, but general symptoms, joint, musculoskeletal, and peripheral neuropathy were preponderant in SCNSV. After treatment with IV-CYC and GCS, patients with PCNSV had a higher relapse-free survival than those with SCNSV.
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Hajj-Ali RA, Calabrese LH (2014) Diagnosis and classification of central nervous system vasculitis. J Autoimmun 48–49:149–152
Lie JT (1997) Classification and histopathologic spectrum of central nervous system vasculitis. Neurol Clin 15:805–819
Calabrese LH, Duna GF, Lie JT (1997) Vasculitis in the central nervous system. Arthritis Rheum 40:1189–1201
Calabrese LH, Furlan AJ, Gragg LA, Ropos TJ (1992) Primary angiitis of the central nervous system: diagnostic criteria and clinical approach. Cleve Clin J Med 59:293–306
Birnbaum J, Hellmann DB (2009) Primary angiitis of the central nervous system. Arch Neurol 66:704–709
Mandell DM, Matouk CC, Farb RI et al (2012) Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results. Stroke 43:860–862
Zuccoli G, Pipitone N, Haldipur A, Brown RD Jr, Hunder G, Salvarani C (2011) Imaging findings in primary central nervous system vasculitis. Clin Exp Rheumatol 29(1 Suppl 64):S104–S109
Salvarani C, Brown RD Jr, Calamia KT et al (2011) Rapidly progressive primary central nervous system vasculitis. Rheumatology (Oxford) 50:349–358
Alrawi A, Trobe JD, Blaivas M, Musch DC (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53:858–860
Alba MA, Espígol-Frigolé G, Prieto-González S et al (2011) Central nervous system vasculitis: still more questions than answers. Curr Neuropharmacol 9:437–448
Geri G, Saadoun D, Guillevin R, Crozier S, Lubetzki C, Mokhtari K et al (2014) Central nervous system vasculitis: a series of 31 cases. Clin Rheumatol 33:105–110
Berlit P (2010) Diagnosis and treatment of cerebral vasculitis. Ther Adv Neurol Disord 3:29–42
Salvarani C, Brown RD Jr, Hunder GG (2012) Adult primary central nervous system vasculitis. Lancet 380(9843):767–777
Calabrese LH, Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 67:20–39
Salvarani C, Brown RD Jr, Calamia KT et al (2007) Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 62:442–451
Melms A, Kötter I, Holle JU (2012) Neurological manifestations of vasculitis and primary central nervous system vasculitis. Z Rheumatol 71:551–563
Unterman A, Nolte JE, Boaz M, Abady M, Shoenfeld Y, Zandman-Goddard G (2011) Neuropsychiatric syndromes in systemic lupus erythematosus: a meta-analysis. Semin Arthritis Rheum 41:1–11
Zulian F, Vallongo C, Woo P et al (2005) Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 52:2873–2881
Ajili F, Mersni A, Métoui L et al (2011) Central nervous system involvement in systemic sclerosis. Rev Med Interne 32:e122–e124
Hasiloglu ZI, Albayram S, Tasmali K, Erer B, Selcuk H, Islak C (2012) A case of primary Sjögren’s syndrome presenting primarily with central nervous system vasculitic involvement. Rheumatol Int 32:805–807
Ashjazadeh N, Borhani Haghighi A, Samangooie S, Moosavi H (2003) Neuro-Behcet’s disease: a masquerader of multiple sclerosis. A prospective study of neurologic manifestations of Behcet’s disease in 96 Iranian patients. Exp Mol Pathol 74:17–22
von Bierbrauer A, Pelzer C, Fischer V (2008) Severe chronic headache with depression induced by pachymeningitis in Wegener’s granulomatosis. Dtsch Med Wochenschr 133:185–188
Pizzanelli C, Catarsi E, Pelliccia V et al (2011) Primary angiitis of the central nervous system: report of eight cases from a single Italian center. J Neurol Sci 307:69–73
Langford CA (2010) Vasculitis. J Allergy Clin Immunol 125(2 Suppl 2):S216–S225
Hajj-Ali RA (2010) Primary angiitis of the central nervous system: differential diagnosis and treatment. Best Pract Res Clin Rheumatol 24:413–426
Néel A, Pagnoux C, Guillevin L, Hamidou M (2012) Central nervous system vasculitides: an update. Rev Med Interne 33:381–389
Pagnoux C, Neel A (2009) Primary angiitis of the central nervous system. Clin Exp Rheumatol 27(1 Suppl 52):S95–S107
Gomes LJ (2010) The role of imaging in the diagnosis of central nervous system vasculitis. Curr Allergy Asthma Rep 10:163–170
Stone JH, Pomper MG, Roubenoff R, Mille TJ, Hellmann DB (1994) Sensitivities of noninvasive tests for central nervous system vasculitis: a comparison of lumbar puncture, computed tomography, and magnetic resonance imaging. J Rheumatol 21:1277–1282
Aviv RI, Benseler SM, DeVeber G, Silverman ED, Tyrrell PN, Tsang LM et al (2007) Angiography of primary central nervous system angiitis of childhood: conventional angiography versus magnetic resonance angiography at presentation. AJNR Am J Neuroradiol 28:9–15
Bley TA, Ostendorf B, Scherer A, Kellner H, Schmidt WA (2012) Magnetic resonance angiography in rheumatology. Z Rheumatol 71:430–435
De Boysson H, Zuber M, Naggara O et al (2014) Primary angiitis of the central nervous system: description of the first fifty-two adult patients enrolled in the French cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheum 66:1315–1326
Magnus T, Melms A, Kötter I, Holle JU (2012) Neurological manifestations of vasculitis and primary central nervous system vasculitis. Z Rheumatol 71:551–563
Barile-Fabris L, Ariza-Andraca R, Olguín-Ortega L et al (2005) Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann Rheum Dis 64:620–625
Cupps TR, Moore PM, Fauci AS (1983) Isolated angiitis of the central nervous system. Prospective diagnostic and therapeutic experience. Am J Med 74:97–105
Iannetti L, Zito R, Bruschi S, et al (2012) Recent understanding on diagnosis and management of central nervous system vasculitis in children, 2012: 698327
Kim YS, Park BS, Baek W, Kim SH (2012) Massive vasculitic cerebellar infarction in patient with systemic lupus erythematosus. Neurol India 60:106–108
Acioly MA, Farina EM, Dalmônico AC, Aguiar LR (2012) Severe cerebral vasculitis in systemic lupus erythematosus: from stroke to multiple fusiform aneurysms. Eur Neurol 67:352–353
Karageorgas TP, Tseronis DD, Mavragani CP (2011) Activation of type I interferon pathway in systemic lupus erythematosus: association with distinct clinical phenotypes. J Biomed Biotechnol 2011:273907
Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin L (2006) Central nervous system involvement in Wegener granulomatosis. Medicine (Baltimore) 85:54–65
Pathak R, Gabor AJ (1991) Scleroderma and central nervous system vasculitis. Stroke 22:410–413
Amaral TN, Marques Neto JF, Lapa AT, Peres FA, Guirau CR, Appenzeller S (2012) Neurologic involvement in scleroderma en coup de sabre. Autoimmune Dis 2012:719685
Fauchais AL, Magy L, Vidal E (2012) Central and peripheral neurological complications of primary Sjögren’s syndrome. Presse Med 41(9 Pt 2):e485–e493
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Vera-Lastra, O., Sepúlveda-Delgado, J., Cruz-Domínguez, M.P. et al. Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis. Clin Rheumatol 34, 729–738 (2015). https://doi.org/10.1007/s10067-014-2831-8
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DOI: https://doi.org/10.1007/s10067-014-2831-8