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

Author:

Pascarella Rosario1ORCID,Antonenko Katherina2,Boulouis Grégoire3,De Boysson Hubert4ORCID,Giannini Caterina5,Heldner Mirjam R2,Kargiotis Odysseas6ORCID,Nguyen Thanh N7ORCID,Rice Claire M89ORCID,Salvarani Carlo10,Schmidt-Pogoda Antje11,Strbian Daniel12ORCID,Hussain Salman13,Zedde Marialuisa14ORCID

Affiliation:

1. Neuroradiology Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy

2. Department of Neurology, University Hospital and University of Bern, Bern, Switzerland

3. Neuroradiology - Diagnostic and Interventional Neuroradiology, CIC-IT 1415, INSERM 1253 iBrain, Tours University Hospital, Centre Val de Loire Region, France

4. Service de Médecine Interne, CHU de Caen, Avenue de la Côte de Nacre, Caen, France; Université Caen Normandie, Caen, France

5. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

6. Stroke Unit, Metropolitan Hospital, Ethnarchou Makariou 9, Piraeus, Greece

7. Neurology, Radiology, Boston Medical Center, Boston University Chobanian and Avedisian School of Medicine, Boston, MA, USA

8. Neurology, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK

9. Department of Neurology, Southmead Hospital, North Bristol NHS Trust, Bristol, UK

10. Rheumatology, Rheumatology Unit, Azienda Ospedaliera-IRCCS di Reggio Emilia and Università di Modena e Reggio Emilia, Reggio Emilia, Italy

11. Department of Neurology with Institute of Translational Neurology, Münster University Hospital, Albert-Schweitzer-Campus 1, Münster, Germany

12. Department of Neurology, Helsinki University Central Hospital HUCH, Helsinki, Finland

13. European Stroke Organisation, Basel, Switzerland

14. Neurology Unit, Stroke Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy

Abstract

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.

Publisher

SAGE Publications

Subject

Cardiology and Cardiovascular Medicine,Neurology (clinical)

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