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.
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Sánchez Aliaga, E. (2019). Vasculitis and Other Inflammatory Disorders. In: Barkhof, F., Jager, R., Thurnher, M., Rovira Cañellas, A. (eds) Clinical Neuroradiology. Springer, Cham. https://doi.org/10.1007/978-3-319-61423-6_45-1
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DOI: https://doi.org/10.1007/978-3-319-61423-6_45-1
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