Skip to main content
SpringerLink
Log in

Vasculitis and Other Inflammatory Disorders

Imaging Findings

  • Reference work entry
  • First Online:
Clinical Neuroradiology

Abstract

The spectrum of inflammatory and immunologically mediated vessel diseases (IIMVD) englobes a heterogeneous group of rare disorders in which inflammation affecting the vessel wall or the perivascular space, of both arteries and veins, is the result of an immune response precipitated, in the majority of the cases, by an unknown element. Primary and secondary vasculitis, Susac syndrome, CLIPPERS, neurosarcoidosis, and immunoglobulin G4-related disease, included in this category, frequently manifest with very similar clinical and laboratory features. Clinical neuroradiology, with imaging findings incorporated in several of the diagnostic criteria, plays a pivotal role for establishing the diagnosis, serves as guidance for additional examinations during the diagnosis work-up, and enables monitoring disease evolution and treatment. Radiological evaluation using brain MRI is the rule, but, in many cases, diagnosis requires the combination of diverse radiological techniques, mainly those ones allowing assessment of brain vessels such as CT Angiography (CTA), MR Angiography (MRA), or Digital Subtraction Angiography (DSA), or those that can add valuable information to exclude other diagnosis like spinal MRI or CT and MRI of other organs. Nevertheless diagnosis of these disorders is for both clinicians and radiologist difficult and requires a multidisciplinary approach with extensive clinical, radiological, and laboratory evaluation and frequently tissue biopsy.

This publication is endorsed by: European Society of Neuroradiology (www.esnr.org).

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 979.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 1,399.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Bando H, Iguchi G, Fukuoka H, et al. The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature. Eur J Endocrinol. 2013;170(2):161–72.

    Article  CAS  PubMed  Google Scholar 

  • Baptista B, Casian A, Gunawardena H, et al. Neurological manifestations of IgG4-related disease. Curr Treat Options Neurol. 2017;19(4):14.

    Google Scholar 

  • Bougea A, Spantideas N. In: Sattler S, Kennedy-Lydon T, editors. The immunology of cardiovascular homeostasis and pathology. Advances in experimental medicine and biology, vol. 1003. Cham: Springer International Publishing AG; 2017.

    Google Scholar 

  • Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146(1):34–44.

    Article  PubMed  Google Scholar 

  • Dudesek A, Rimmele F, Tesar S, et al. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clin Exp Immunol. 2014;175(3):385–96.

    Article  CAS  Google Scholar 

  • Gerke AK, Hunninghake G. The immunology of sarcoidosis. Clin Chest Med. 2008;29(3):379–90, vii.

    Article  PubMed  Google Scholar 

  • Greco A, De Virgilio A, Gallo A, et al. Susac’s syndrome – pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 2014;13(8):814–21.

    Article  CAS  PubMed  Google Scholar 

  • Hajj-Ali RA, Calabrese LH. Diagnosis and classification of central nervous system vasculitis. J Autoimmun. 2014;48–49:149–52.

    Article  PubMed  Google Scholar 

  • Hoitsma E, Drent M, Sharma OP. A pragmatic approach to diagnosing and treating neurosarcoidosis in the 21st century. Curr Opin Pulm Med. 2010;16(5):472–9.

    Article  PubMed  Google Scholar 

  • Ibitoye RT, Wilkins A, Scolding NJ. Neurosarcoidosis: a clinical approach to diagnosis and management. J Neurol. 2017;264(5):1023–8.

    Article  PubMed  PubMed Central  Google Scholar 

  • Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.

    Google Scholar 

  • Kamisawa T, Zen Y, Pillai S, et al. IgG4-related disease. Lancet. 2015;385(9976):1460–71.

    Article  CAS  Google Scholar 

  • Kleffner I, Dörr J, Ringelstein M, European Susac Consortium (EuSaC), et al. Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016;87(12):1287–95.

    Google Scholar 

  • Lu LX, Della-Torre E, Stone JH, et al. IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 2014;71(6):785–93.

    Article  PubMed  Google Scholar 

  • Mandell DM, Matouk CC, Farb RI, et al. Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results. Stroke. 2012;43(3):860–2.

    Article  PubMed  Google Scholar 

  • Pittock SJ, Debruyne J, Krecke KN, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 2010;133(9):2626–34.

    Article  PubMed  Google Scholar 

  • Salvarani C, Brown RD, Hunder GG. Adult primary central nervous system vasculitis. Lancet. 2012;380(9843):767–77.

    Article  Google Scholar 

  • Simon NG, Parratt JD, Barnett MH, et al. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurol Neurosurg Psychiatry. 2012;83(1):15–22.

    Article  Google Scholar 

  • Spiegel DR, Morris K, Rayamajhi U. Neurosarcoidosis and the complexity in its differential diagnoses. Innov Clin Neurosci. 2012;9(4):10–6.

    PubMed  PubMed Central  Google Scholar 

  • Susac JO, Murtagh FR, Egan RA, et al. MRI findings in Susac’s syndrome. Neurology. 2003;61:1783–7.

    Article  CAS  PubMed  Google Scholar 

  • Susac JO, Egan RA, Rennebohm RM, et al. Susac’s syndrome: 1975–2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 2007;257:270–2.

    Article  PubMed  Google Scholar 

  • Wengenroth M, Jacobi C, Wildemann B. In: Hähnel S, editor. Inflammatory diseases of the brain. Medical radiology. Diagnostic imaging. Berlin/Heidelberg: Springer; 2013.

    Google Scholar 

Recommended Literature

  • Abdel Razek AA, Alvarez H, Bagg S, et al. Imaging spectrum of CNS vasculitis. Radiographics. 2014;34(4):873–94.

    Article  PubMed  Google Scholar 

  • Aliaga ES, Barkhof F. MRI mimics of multiple sclerosis. Handb Clin Neurol. 2014;122:291–316.

    Google Scholar 

  • Berlit P, Kraemer M. Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis. Red flags and pitfalls. Clin Exp Immunol. 2014;175(3):419–24.

    Article  CAS  Google Scholar 

  • Hebel R, Dubaniewicz-Wybieralska M, Dubaniewicz A. Overview of neurosarcoidosis: recent advances. J Neurol. 2015;262(2):258–67.

    Article  PubMed  PubMed Central  Google Scholar 

  • Lacomis D. Neurosarcoidosis. Curr Neuropharmacol. 2011;9(3):429–36.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Rennebohm R, Susac JO, Egan RA, et al. Susac’s syndrome – update. J Neurol Sci. 2010;299(1–2):86–91.

    Article  PubMed  Google Scholar 

  • Rovira A, Sundgren P, Gallucci M. Inflammatory and metabolic disease. Chapter 64. In: Grainger & Allison’s diagnostic radiology. 6th ed. Churchill Livingstone, an imprint of Elvesier Limited, 2015.

    Google Scholar 

  • Rovira Á, Auger C, Rovira A. Other noninfectious inflammatory disorders. Handb Clin Neurol. 2016;135:425–46.

    Google Scholar 

  • Tobin WO, Guo Y, Krecke KN, et al. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 2017;140(9):2415–25.

    Article  PubMed  Google Scholar 

  • Twilt M, Benseler SM. Central nervous system vasculitis in adults and children. Handb Clin Neurol. 2016;133:283–300.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Radiology and Nuclear Medicine, VU University Medical Centre, Amsterdam, The Netherlands

    Esther Sánchez Aliaga

Authors
  1. Esther Sánchez Aliaga
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Esther Sánchez Aliaga .

Editor information

Editors and Affiliations

  1. Department of Radiology and Nuclear Medicine, VU University Medical Centre (VUmc), Amsterdam, The Netherlands

    Frederik Barkhof

  2. The National Hospital for Neurology and Neurosurgery and University College Hospital (UCH), UCL Institute of Neurology, London, UK

    Hans Rolf Jäger

  3. Section of Neuroradiology and Musculoskeletal Radiology, Department of Biomedical Imaging and Image-guided Therapy, University Hospital Vienna, Vienna, Austria

    Majda M. Thurnher

  4. Department of Radiology and Nuclear Medicine Vall d’Hebron University Hospital Professor of Radiology and Neuroimmunology, Autonomous University of Barcelona Vall d’Hebron Research Institute (VHIR), Barcelona, Spain

    Àlex Rovira

Section Editor information

  1. Department of Radiology, Hospital Universitari Vall d’Hebron, Barcelona, Spain

    Alex Rovira

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Sánchez Aliaga, E. (2019). Vasculitis and Other Inflammatory Disorders. In: Barkhof, F., Jäger, H., Thurnher, M., Rovira, À. (eds) Clinical Neuroradiology. Springer, Cham. https://doi.org/10.1007/978-3-319-68536-6_45

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-68536-6_45

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-68535-9

  • Online ISBN: 978-3-319-68536-6

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation