Primary Angiitis of the CNS

Beuker, Carolin; Strunk, Daniel R.; Rawal, Rajesh; Schmidt‐Pogoda, Antje; Werring, Nils; Milles, Lennart Steffen; Ruck, Tobias; Wiendl, Heinz; Meuth, Sven G.; Minnerup, Heike; Minnerup, Jens

doi:10.1212/nxi.0000000000001093

Cited by 34 publications

(25 citation statements)

References 55 publications

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“…We identified four reviews [15][16][17][18] pertinent to this Guideline; these differed in their precise focus and none aligned with our inclusion criteria so, although we considered these manuscripts, we did not incorporate their synthesis of results.…”

Section: Literature Searchmentioning

confidence: 99%

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European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS)

Pascarella,

Antonenko,

Boulouis

et al. 2023

European Stroke Journal

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The European Stroke Organisation (ESO) guideline on Primary Angiitis of the Central Nervous System (PACNS), developed according to ESO standard operating procedures (SOP) and Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology, was elaborated to assist clinicians in the diagnostic and treatment pathway of patients with PACNS in their decision making. A working group involving vascular neurologists, neuroradiologists, rheumatologists, a neuropathologist and a methodologist identified 17 relevant clinical questions; these were addressed according to the patient/population, intervention, comparison and outcomes (PICO) framework and systematic literature reviews were performed. Notably, each PICO was addressed with respect to large vessel (LV)-PACNS and small vessel (SV)-PACNS. Data to answer many questions were scarce or lacking and the quality of evidence was very low overall, so, for some PICOs, the recommendations reflect the ongoing uncertainty. When the absence of sufficient evidence precluded recommendations, Expert Consensus Statements were formulated. In some cases, this applied to interventions in the diagnosis and treatment of PACNS which are embedded widely in clinical practice, for example patterns of cerebrospinal fluid (CSF) and Magnetic Resonance Imaging (MRI) abnormalities. CSF analysis for hyperproteinorrachia and pleocytosis does not have evidence supporting their use as diagnostic tools. The working group recommended that caution is employed in the interpretation of non-invasive vascular imaging due to lack of validation and the different sensitivities in comparison with digital subtraction angiography (DSA) and histopathological analyses. Moreover, there is not a neuroimaging pattern specific for PACNS and neurovascular issues are largely underreported in PACNS patients. The group’s recommendations on induction and maintenance of treatment and for primary or secondary prevention of vascular events also reflect uncertainty due to lack of evidence. Being uncertain the role and practical usefulness of current diagnostic criteria and being not comparable the main treatment strategies, it is suggested to have a multidisciplinary team approach in an expert center during both work up and management of patients with suspected PACNS. Highlighting the limitations of the currently accepted diagnostic criteria, we hope to facilitate the design of multicenter, prospective clinical studies and trials. A standardization of neuroimaging techniques and reporting to improve the level of evidence underpinning interventions employed in the diagnosis and management of PACNS. We anticipate that this guideline, the first comprehensive European guideline on PACNS management using GRADE methodology, will assist clinicians to choose the most effective management strategy for PACNS.

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Section: Literature Searchmentioning

confidence: 99%

“…3 Abnormal test results were reported in 74.4% and 75% of patients, respectively. In addition, the systematic review and meta-analysis by Beuker et al 17 reported CSF data on 581/911 patients with abnormalities reported in 75% samples.…”

Section: Diagnosismentioning

confidence: 99%

European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS)

Pascarella,

Antonenko,

Boulouis

et al. 2023

European Stroke Journal

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“…Increasing evidence suggests that angiogram-confirmed PACNS typically involves medium to large vessels, while biopsy-confirmed cases relate to small-vessel vasculitis [8]. Additional clinical and laboratory differences supporting this concept are summarized in Figure 3 [1].…”

Section: Discussionmentioning

confidence: 96%

Headache that Never Went Away

Vithanage,

James,

Karunanathan

et al. 2024

JBM

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Central nervous system (CNS) vasculitis encompasses a broad range of diseases characterized by inflammation of blood vessels within the brain, spinal cord, and meninges. This review focuses on Primary Angiitis of the CNS (PACNS), a unique form affecting only the CNS without other organ involvement. PACNS primarily affects the small-and medium-sized arteries in the brain, leading to neurological symptoms. Early recognition and intervention are essential for optimal management. This case report of a 25-year-old male highlights the diagnostic challenges, where a multidisciplinary approach was necessary to diagnose PACNS, given initial negative results. The paper also explores the etiology and pathogenesis of PACNS and discusses the diagnostic criteria proposed by researchers. Medical professionals primarily base management strategies on induction and maintenance therapies, utilizing a combination of glucocorticoids and immunosuppressants as the standard treatment. However, the exact dosages and administration methods need further research due to the absence of randomized clinical trials. The review underscores the need for continued research to enhance diagnostic and treatment protocols, given the severity of PACNS.

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“…CSF analysis in PACNS may be normal [45] or show pleocytosis and elevated protein [46]. Vascular imaging, including CT, MRI, or catheter angiography, may show vasospasm [48]. Brain biopsy remains the diagnostic gold standard [48].…”

Section: Vasculitismentioning

confidence: 99%

“…Vascular imaging, including CT, MRI, or catheter angiography, may show vasospasm [48]. Brain biopsy remains the diagnostic gold standard [48]. A study examining the incidence of MRI changes among 135 patients with NSCLC receiving ICI therapy found 11 patients with lesions suggestive of ischemic stroke and 4 with lesions suggestive of CNS vasculitis or encephalitis, though other clinical and paraclinical parameters were not reported [49].…”

Section: Vasculitismentioning

confidence: 99%

Neurologic Complications of Cancer Immunotherapy

et al. 2023

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Immunotherapy has revolutionized cancer treatment over the past decade. As it is increasingly introduced into routine clinical practice, immune-related complications have become more frequent. Accurate diagnosis and treatment are essential, with the goal of reduced patient morbidity. This review aims to discuss the various clinical manifestations, diagnosis, treatments, and prognosis of neurologic complications associated with the use of immune checkpoint inhibitors, adoptive T-cell therapies, and T-cell redirecting therapies. We also outline a suggested clinical approach related to the clinical use of these agents.

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Primary Angiitis of the CNS

Cited by 34 publications

References 55 publications

European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS)

European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS)

Headache that Never Went Away

Neurologic Complications of Cancer Immunotherapy

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