The diagnosis of primary central nervous system vasculitis

Abstract

<jats:p>The diagnosis of primary central nervous system (CNS) vasculitis is often difficult. There are neither specific clinical features nor a classical clinical course, and no blood or imaging investigations that can confirm the diagnosis. Contrast catheter cerebral angiography is neither specific nor sensitive, yet still underpins the diagnosis in many published studies. Here we describe an approach to its diagnosis, emphasising the importance of obtaining tissue, and present for discussion a new, binary set of diagnostic criteria, dividing cases into only ‘definite’ primary CNS vasculitis, where tissue proof is available, and ‘possible,’ where it is not. We hope that these criteria will be modified and improved by discussion among experts, and that these (improved) criteria may then be adopted and used as the basis for future prospective studies of the clinical features and diagnosis of this difficult and dangerous disorder, particularly for coordinated multicentre therapeutic trials.</jats:p>

Utility of Vessel Wall Imaging in Suspected CNS Vasculitis with Normal Routine Workup

“…A biopsy from the affected cortex and meninges remains the gold standard despite its invasive nature. 6 The imaging changes in our patient were in high-risk structures, thus he denied a brain biopsy. This highlights the role of imaging and serology in establishing the diagnosis.…”

“…After an induction phase achieves remission, transitioning to a lower dose of steroids and less toxic agents like azathioprine is considered. 6 Since thrombosis is often associated with vasculitis, the use of antiplatelet therapy is thought to be beneficial. However, there are conflicting data for antiplatelets use in this setting.…”

Blood–brain barrier: emerging trends on transport models and new-age strategies for therapeutics intervention against neurological disorders

“…A wide variety of conditions like arthritis, encephalopathy and Sjogren’s syndrome are associated with the disease, besides the increased C-Reactive Protein (CRP) and Erythrocyte Sedimentation Rate (ESR) levels. Treatment is done by administration of corticosteroids [ 142 , 143 ].…”

Tocilizumab for severe refractory primary central nervous system vasculitis: A center experience

“…PCNSV presents with clinical, radiological, and pathological variability. The diagnosis is supported by typical signs on angiography, brain biopsy, and exclusion of a systemic condition 4,5 . Given the absence of randomized clinical trials in PCNSV, there are no data regarding the optimal drug treatment and its duration, with treatment options mimicking the ones applied in systemic vasculitis and consisting mostly in glucocorticoids alone or in combination with cyclophosphamide 1,2,6,7 …”