Skip to main content

Advertisement

SpringerLink
Log in

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Hajj-Ali RA, Calabrese LH (2014) Diagnosis and classification of central nervous system vasculitis. J Autoimmun 48–49:149–152

    Article  PubMed  Google Scholar 

  2. Lie JT (1997) Classification and histopathologic spectrum of central nervous system vasculitis. Neurol Clin 15:805–819

    Article  CAS  PubMed  Google Scholar 

  3. Calabrese LH, Duna GF, Lie JT (1997) Vasculitis in the central nervous system. Arthritis Rheum 40:1189–1201

    Article  CAS  PubMed  Google Scholar 

  4. 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

    Article  CAS  PubMed  Google Scholar 

  5. Birnbaum J, Hellmann DB (2009) Primary angiitis of the central nervous system. Arch Neurol 66:704–709

    PubMed  Google Scholar 

  6. 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

    Article  PubMed  Google Scholar 

  7. 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

    PubMed  Google Scholar 

  8. Salvarani C, Brown RD Jr, Calamia KT et al (2011) Rapidly progressive primary central nervous system vasculitis. Rheumatology (Oxford) 50:349–358

    Article  Google Scholar 

  9. Alrawi A, Trobe JD, Blaivas M, Musch DC (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53:858–860

    Article  CAS  PubMed  Google Scholar 

  10. 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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. Berlit P (2010) Diagnosis and treatment of cerebral vasculitis. Ther Adv Neurol Disord 3:29–42

    Article  PubMed Central  PubMed  Google Scholar 

  13. Salvarani C, Brown RD Jr, Hunder GG (2012) Adult primary central nervous system vasculitis. Lancet 380(9843):767–777

    Article  PubMed  Google Scholar 

  14. 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

    Article  CAS  Google Scholar 

  15. Salvarani C, Brown RD Jr, Calamia KT et al (2007) Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 62:442–451

    Article  PubMed  Google Scholar 

  16. Melms A, Kötter I, Holle JU (2012) Neurological manifestations of vasculitis and primary central nervous system vasculitis. Z Rheumatol 71:551–563

    Article  PubMed  Google Scholar 

  17. 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

    Article  PubMed  Google Scholar 

  18. Zulian F, Vallongo C, Woo P et al (2005) Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 52:2873–2881

    Article  PubMed  Google Scholar 

  19. Ajili F, Mersni A, Métoui L et al (2011) Central nervous system involvement in systemic sclerosis. Rev Med Interne 32:e122–e124

    Article  CAS  PubMed  Google Scholar 

  20. 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

    Article  PubMed  Google Scholar 

  21. 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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  Google Scholar 

  23. 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

    Article  PubMed  Google Scholar 

  24. Langford CA (2010) Vasculitis. J Allergy Clin Immunol 125(2 Suppl 2):S216–S225

    Article  PubMed  Google Scholar 

  25. Hajj-Ali RA (2010) Primary angiitis of the central nervous system: differential diagnosis and treatment. Best Pract Res Clin Rheumatol 24:413–426

    Article  PubMed  Google Scholar 

  26. Néel A, Pagnoux C, Guillevin L, Hamidou M (2012) Central nervous system vasculitides: an update. Rev Med Interne 33:381–389

    Article  PubMed  Google Scholar 

  27. Pagnoux C, Neel A (2009) Primary angiitis of the central nervous system. Clin Exp Rheumatol 27(1 Suppl 52):S95–S107

    PubMed  Google Scholar 

  28. Gomes LJ (2010) The role of imaging in the diagnosis of central nervous system vasculitis. Curr Allergy Asthma Rep 10:163–170

    Article  PubMed  Google Scholar 

  29. 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

    CAS  PubMed  Google Scholar 

  30. 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

    Article  CAS  PubMed  Google Scholar 

  31. Bley TA, Ostendorf B, Scherer A, Kellner H, Schmidt WA (2012) Magnetic resonance angiography in rheumatology. Z Rheumatol 71:430–435

    Article  CAS  PubMed  Google Scholar 

  32. 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

    Article  Google Scholar 

  33. Magnus T, Melms A, Kötter I, Holle JU (2012) Neurological manifestations of vasculitis and primary central nervous system vasculitis. Z Rheumatol 71:551–563

    Article  CAS  PubMed  Google Scholar 

  34. 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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  35. 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

    Article  CAS  PubMed  Google Scholar 

  36. Iannetti L, Zito R, Bruschi S, et al (2012) Recent understanding on diagnosis and management of central nervous system vasculitis in children, 2012: 698327

  37. Kim YS, Park BS, Baek W, Kim SH (2012) Massive vasculitic cerebellar infarction in patient with systemic lupus erythematosus. Neurol India 60:106–108

    Article  PubMed  Google Scholar 

  38. 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

    Article  PubMed  Google Scholar 

  39. 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

    Article  PubMed Central  PubMed  Google Scholar 

  40. 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

    Article  Google Scholar 

  41. Pathak R, Gabor AJ (1991) Scleroderma and central nervous system vasculitis. Stroke 22:410–413

    Article  CAS  PubMed  Google Scholar 

  42. 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

    PubMed Central  PubMed  Google Scholar 

  43. 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

    Article  PubMed  Google Scholar 

Download references

Conflict of interest

We declare that we have no conflicts of interest.

Author information

Authors and Affiliations

  1. Hospital de Especialidades “Dr. Antonio Fraga Mouret,” Centro Médico Nacional La Raza, Internal Medicine Department, Instituto Mexicano del Seguro Social, Seris y Zaachila S/N Col. La Raza, 02990, México City, Mexico

    Olga Vera-Lastra, Jesús Sepúlveda-Delgado, María del Pilar Cruz-Domínguez, Moisés Casarrubias-Ramírez, Luis F. Pineda-Galindo & Arturo Olvera-Acevedo

  2. Neurology Department, Hospital de Especialidades “Dr. Antonio Fraga Mouret,” Centro Médico Nacional LaRaza, Instituto Mexicano del Seguro Social, México City, Mexico

    Luis E. Molina-Carrión

  3. Clinical Research Unit, Hospital de Especialidades “Dr. Antonio Fraga Mouret,” Centro Médico Nacional LaRaza, Instituto Mexicano del Seguro Social, México City, Mexico

    Gabriela Medina

  4. Imagenology Deparment, Hospital de Especialidades “Dr. Antonio Fraga Mouret,” Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México City, Mexico

    Claudia Hernández-Gonzalez

  5. Direction of Education and Research, Hospital de Especialidades “Dr. Antonio Fraga Mouret,” Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México City, Mexico

    Luis J. Jara

  6. Facultad de Medicina, División de Estudios de Posgrado, Universidad Nacional Autónoma de México, México City, Mexico

    Olga Vera-Lastra, Jesús Sepúlveda-Delgado, María del Pilar Cruz-Domínguez, Gabriela Medina, Moisés Casarrubias-Ramírez, Luis E. Molina-Carrión, Luis F. Pineda-Galindo, Arturo Olvera-Acevedo & Luis J. Jara

Authors
  1. Olga Vera-Lastra
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jesús Sepúlveda-Delgado
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. María del Pilar Cruz-Domínguez
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gabriela Medina
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Moisés Casarrubias-Ramírez
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Luis E. Molina-Carrión
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Luis F. Pineda-Galindo
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Arturo Olvera-Acevedo
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Claudia Hernández-Gonzalez
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Luis J. Jara
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Olga Vera-Lastra.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-014-2831-8

Keywords

Search

Navigation