{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T20:06:17Z","timestamp":1779912377765,"version":"3.53.1"},"reference-count":58,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T00:00:00Z","timestamp":1696204800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Tomography"],"abstract":"<jats:p>Neuroimaging has a key role in identifying small-vessel vasculitis from common diseases it mimics, such as multiple sclerosis. Oftentimes, a multitude of these conditions present similarly, and thus diagnosis is difficult. To date, there is no standardized method to differentiate between these diseases. This review identifies and presents existing scoring tools that could serve as a starting point for integrating artificial intelligence\/machine learning (AI\/ML) into the clinical decision-making process for these rare diseases. A scoping literature review of EMBASE and MEDLINE included 114 articles to evaluate what criteria exist to diagnose small-vessel vasculitis and common mimics. This paper presents the existing criteria of small-vessel vasculitis conditions and mimics them to guide the future integration of AI\/ML algorithms to aid in diagnosing these conditions, which present similarly and non-specifically.<\/jats:p>","DOI":"10.3390\/tomography9050144","type":"journal-article","created":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T04:53:36Z","timestamp":1696222416000},"page":"1811-1828","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Neuroimaging Scoring Tools to Differentiate Inflammatory Central Nervous System Small-Vessel Vasculitis: A Need for Artificial Intelligence\/Machine Learning?\u2014A Scoping Review"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3953-5822","authenticated-orcid":false,"given":"Alameen","family":"Damer","sequence":"first","affiliation":[{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5T 1W7, Canada"},{"name":"Department of Radiology, Radiation Oncology and Medical Physics, University of Ottawa, Ottawa, ON K1N 6N5, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5724-0027","authenticated-orcid":false,"given":"Emaan","family":"Chaudry","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5T 1W7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Eftekhari","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5T 1W7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne M.","family":"Benseler","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, AB T2N 4N1, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frozan","family":"Safi","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5T 1W7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard I.","family":"Aviv","sequence":"additional","affiliation":[{"name":"Department of Radiology, Radiation Oncology and Medical Physics, University of Ottawa, Ottawa, ON K1N 6N5, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2277-3824","authenticated-orcid":false,"given":"Pascal N.","family":"Tyrrell","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5T 1W7, Canada"},{"name":"Institute of Medical Science, Department of Statistical Sciences, University of Toronto, Toronto, ON M5G 1X6, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1038\/nrneurol.2010.1","article-title":"Environmental Factors and Their Timing in Adult-Onset Multiple Sclerosis","volume":"6","author":"Handel","year":"2010","journal-title":"Nat. Rev. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1111\/ene.13819","article-title":"Multiple Sclerosis\u2014A Review","volume":"26","author":"Dobson","year":"2019","journal-title":"Eur. J. Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1336","DOI":"10.1016\/S0140-6736(16)30959-X","article-title":"Diagnosis of Multiple Sclerosis: Progress and Challenges","volume":"389","author":"Brownlee","year":"2017","journal-title":"Lancet"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1093\/brain\/awm329","article-title":"Disability and T2 MRI Lesions: A 20-Year Follow-up of Patients with Relapse Onset of Multiple Sclerosis","volume":"131","author":"Fisniku","year":"2008","journal-title":"Brain"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1038\/nrrheum.2011.197","article-title":"The Spectrum of CNS Vasculitis in Children and Adults","volume":"8","author":"Twilt","year":"2012","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.1016\/S1474-4422(10)70243-X","article-title":"Treatment of Small Vessel Primary CNS Vasculitis in Children: An Open-Label Cohort Study","volume":"9","author":"Hutchinson","year":"2010","journal-title":"Lancet Neurol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1148\/rg.344135028","article-title":"Imaging Spectrum of CNS Vasculitis","volume":"34","author":"Alvarez","year":"2014","journal-title":"RadioGraphics"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"2159","DOI":"10.1002\/art.21144","article-title":"Angiography-Negative Primary Central Nervous System Vasculitis in Children: A Newly Recognized Inflammatory Central Nervous System Disease","volume":"52","author":"Benseler","year":"2005","journal-title":"Arthritis Rheum."},{"key":"ref_9","first-page":"S1","article-title":"Multiple Sclerosis: Diagnosis and Differential Diagnosis","volume":"55","year":"2018","journal-title":"Noro Psikiyatr. Ars."},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Eiden, S., Beck, C., Venhoff, N., Elsheikh, S., Ihorst, G., Urbach, H., and Meckel, S. (2019). High-Resolution Contrast-Enhanced Vessel Wall Imaging in Patients with Suspected Cerebral Vasculitis: Prospective Comparison of Whole-Brain 3D T1 SPACE versus 2D T1 Black Blood MRI at 3 Tesla. PLoS ONE, 14.","DOI":"10.1371\/journal.pone.0213514"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1136\/jnnp-2017-315691","article-title":"Subtypes of Primary Angiitis of the CNS Identified by MRI Patterns Reflect the Size of Affected Vessels","volume":"88","author":"Schuster","year":"2017","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1136\/jnnp-2015-312020","article-title":"High-Resolution Intracranial Vessel Wall Imaging: Imaging beyond the Lumen","volume":"87","author":"Alexander","year":"2016","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"e738","DOI":"10.1097\/MD.0000000000000738","article-title":"An Update of the Mayo Clinic Cohort of Patients with Adult Primary Central Nervous System Vasculitis: Description of 163 Patients","volume":"94","author":"Salvarani","year":"2015","journal-title":"Medicine"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"573","DOI":"10.7326\/0003-4819-147-8-200710160-00010","article-title":"The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) Statement: Guidelines for Reporting Observational Studies","volume":"147","author":"Altman","year":"2007","journal-title":"Ann. Intern. Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/S1474-4422(17)30470-2","article-title":"Diagnosis of Multiple Sclerosis: 2017 Revisions of the McDonald Criteria","volume":"17","author":"Thompson","year":"2018","journal-title":"Lancet Neurol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"102801","DOI":"10.1016\/j.autrev.2021.102801","article-title":"Pediatric Inflammatory Demyelinating Disorders and Mimickers: How to Differentiate with MRI?","volume":"20","author":"Pereira","year":"2021","journal-title":"Autoimmun. Rev."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1016\/S1474-4422(15)00393-2","article-title":"MRI Criteria for the Diagnosis of Multiple Sclerosis: MAGNIMS Consensus Guidelines","volume":"15","author":"Filippi","year":"2016","journal-title":"Lancet Neurol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"S7","DOI":"10.1212\/01.wnl.0000259422.44235.a8","article-title":"Consensus Definitions Proposed for Pediatric Multiple Sclerosis and Related Disorders","volume":"68","author":"Krupp","year":"2007","journal-title":"Neurology"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2059","DOI":"10.1093\/brain\/120.11.2059","article-title":"Comparison of MRI Criteria at First Presentation to Predict Conversion to Clinically Definite Multiple Sclerosis","volume":"120","author":"Barkhof","year":"1997","journal-title":"Brain"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1212\/WNL.38.2.180","article-title":"MRI in the Diagnosis of MS","volume":"38","author":"Paty","year":"1988","journal-title":"Neurology"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1097\/00005792-198801000-00002","article-title":"Primary Angiitis of the Central Nervous System. Report of 8 New Cases, Review of the Literature, and Proposal for Diagnostic Criteria","volume":"67","author":"Calabrese","year":"1988","journal-title":"Medicine"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1002\/ana.21226","article-title":"Primary Central Nervous System Vasculitis: Analysis of 101 Patients","volume":"62","author":"Salvarani","year":"2007","journal-title":"Ann. Neurol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1212\/WNL.39.2.167","article-title":"Diagnosis and Management of Isolated Angiitis of the Central Nervous System","volume":"39","author":"Moore","year":"1989","journal-title":"Neurology"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1212\/WNL.53.4.858","article-title":"Brain Biopsy in Primary Angiitis of the Central Nervous System","volume":"53","author":"Alrawi","year":"1999","journal-title":"Neurology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1001\/archneurol.2009.76","article-title":"Primary Angiitis of the Central Nervous System","volume":"66","author":"Birnbaum","year":"2009","journal-title":"Arch. Neurol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1291","DOI":"10.1002\/art.21766","article-title":"Primary Central Nervous System Vasculitis in Children","volume":"54","author":"Benseler","year":"2006","journal-title":"Arthritis Rheum."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1485","DOI":"10.1212\/01.wnl.0000216139.44259.74","article-title":"Revised Diagnostic Criteria for Neuromyelitis Optica","volume":"66","author":"Wingerchuk","year":"2006","journal-title":"Neurology"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"S63.001","DOI":"10.1212\/WNL.82.10_supplement.S63.001","article-title":"Revised Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorders (S63.001)","volume":"82","author":"Wingerchuk","year":"2014","journal-title":"Neurology"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"34","DOI":"10.7326\/0003-4819-146-1-200701020-00007","article-title":"Narrative Review: Reversible Cerebral Vasoconstriction Syndromes","volume":"146","author":"Calabrese","year":"2007","journal-title":"Ann. Intern. Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"e639","DOI":"10.1212\/WNL.0000000000006917","article-title":"RCVS2 Score and Diagnostic Approach for Reversible Cerebral Vasoconstriction Syndrome","volume":"92","author":"Rocha","year":"2019","journal-title":"Neurology"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1093\/brain\/awh415","article-title":"Pathogenesis, Diagnosis and Treatment of Rasmussen Encephalitis: A European Consensus Statement","volume":"128","author":"Bien","year":"2005","journal-title":"Brain"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1016\/S1474-4422(22)00431-8","article-title":"Diagnosis of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: International MOGAD Panel Proposed Criteria","volume":"22","author":"Banwell","year":"2023","journal-title":"Lancet Neurol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1186\/s12974-018-1144-2","article-title":"MOG Encephalomyelitis: International Recommendations on Diagnosis and Antibody Testing","volume":"15","author":"Jarius","year":"2018","journal-title":"J. Neuroinflammation"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1001\/jamaneurol.2018.1814","article-title":"Association of MOG-IgG Serostatus With Relapse After Acute Disseminated Encephalomyelitis and Proposed Diagnostic Criteria for MOG-IgG\u2013Associated Disorders","volume":"75","author":"Majed","year":"2018","journal-title":"JAMA Neurol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1136\/practneurol-2018-002002","article-title":"The Diagnosis of Primary Central Nervous System Vasculitis","volume":"20","author":"Rice","year":"2020","journal-title":"Pract. Neurol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"293","DOI":"10.3949\/ccjm.59.3.293","article-title":"Primary Angiitis of the Central Nervous System: Diagnostic Criteria and Clinical Approach","volume":"59","author":"Calabrese","year":"1992","journal-title":"Clevel. Clin. J. Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"e1093","DOI":"10.1212\/NXI.0000000000001093","article-title":"Primary Angiitis of the CNS","volume":"8","author":"Beuker","year":"2021","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1016\/S1474-4422(11)70081-3","article-title":"Primary Angiitis of the CNS","volume":"10","author":"Singhal","year":"2011","journal-title":"Lancet Neurol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.berh.2009.12.003","article-title":"Primary Angiitis of the Central Nervous System: Differential Diagnosis and Treatment","volume":"24","year":"2010","journal-title":"Best Pract. Res. Clin. Rheumatol."},{"key":"ref_40","first-page":"1277","article-title":"Sensitivities of Noninvasive Tests for Central Nervous System Vasculitis: A Comparison of Lumbar Puncture, Computed Tomography, and Magnetic Resonance Imaging","volume":"21","author":"Stone","year":"1994","journal-title":"J. Rheumatol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s11940-017-0460-7","article-title":"Reversible Cerebral Vasoconstriction Syndrome: Recognition and Treatment","volume":"19","author":"Calic","year":"2017","journal-title":"Curr. Treat. Options Neurol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"568","DOI":"10.3389\/fneur.2019.00568","article-title":"Primary Angiitis of the Central Nervous System: New Potential Imaging Techniques and Biomarkers in Blood and Cerebrospinal Fluid","volume":"10","author":"Schuster","year":"2019","journal-title":"Front. Neurol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"524","DOI":"10.4103\/aian.AIAN_106_21","article-title":"High-Resolution Vessel Wall Imaging in Primary Angiitis of Central Nervous System","volume":"24","author":"Sundaram","year":"2021","journal-title":"Ann. Indian Acad. Neurol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"3091","DOI":"10.1093\/brain\/awm256","article-title":"The Clinical and Radiological Spectrum of Reversible Cerebral Vasoconstriction Syndrome. A Prospective Series of 67 Patients","volume":"130","author":"Ducros","year":"2007","journal-title":"Brain"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1756286418785071","DOI":"10.1177\/1756286418785071","article-title":"Primary Angiitis of the Central Nervous System: Diagnosis and Treatment","volume":"11","author":"Beuker","year":"2018","journal-title":"Ther. Adv. Neurol. Disord."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1016\/j.jacr.2019.05.012","article-title":"Improving Workflow Efficiency for Mammography Using Machine Learning","volume":"17","author":"Kyono","year":"2020","journal-title":"J. Am. Coll. Radiol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1016\/j.media.2012.02.005","article-title":"Machine Learning and Radiology","volume":"16","author":"Wang","year":"2012","journal-title":"Med. Image Anal."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1347","DOI":"10.1056\/NEJMra1814259","article-title":"Machine Learning in Medicine","volume":"380","author":"Rajkomar","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1146","DOI":"10.1212\/WNL.0000000000007654","article-title":"Meschia CNS Small Vessel Disease","volume":"92","author":"Cannistraro","year":"2019","journal-title":"Neurology"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1177\/0883073819845827","article-title":"Epidemiology of Pediatric-Onset Multiple Sclerosis: A Systematic Review of the Literature","volume":"34","author":"Jeong","year":"2019","journal-title":"J. Child Neurol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1417","DOI":"10.1586\/erc.10.124","article-title":"Systematic Review of Reversible Cerebral Vasoconstriction Syndrome","volume":"8","author":"Sattar","year":"2010","journal-title":"Expert Rev. Cardiovasc. Ther."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"102714","DOI":"10.1016\/j.autrev.2020.102714","article-title":"Primary CNS Vasculitis: A Systematic Review on Clinical Characteristics Associated with Abnormal Biopsy and Angiography","volume":"20","author":"Krawczyk","year":"2021","journal-title":"Autoimmun. Rev."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1007\/s12072-021-10291-7","article-title":"Application of Machine Learning in Liver Transplantation: A Review","volume":"16","author":"Tran","year":"2022","journal-title":"Hepatol. Int."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1148\/radiol.2018171820","article-title":"Current Applications and Future Impact of Machine Learning in Radiology","volume":"288","author":"Choy","year":"2018","journal-title":"Radiology"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1097\/RLI.0000000000000735","article-title":"Deep Learning on Conventional Magnetic Resonance Imaging Improves the Diagnosis of Multiple Sclerosis Mimics","volume":"56","author":"Rocca","year":"2021","journal-title":"Investig. Radiol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"3923","DOI":"10.1038\/s41467-020-17419-7","article-title":"Improving the Accuracy of Medical Diagnosis with Causal Machine Learning","volume":"11","author":"Richens","year":"2020","journal-title":"Nat. Commun."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1860","DOI":"10.1001\/jamainternmed.2016.6001","article-title":"Comparison of Physician and Computer Diagnostic Accuracy","volume":"176","author":"Semigran","year":"2016","journal-title":"JAMA Intern. Med."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1038\/s41746-020-0221-y","article-title":"An Overview of Clinical Decision Support Systems: Benefits, Risks, and Strategies for Success","volume":"3","author":"Sutton","year":"2020","journal-title":"NPJ Digit. Med."}],"container-title":["Tomography"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2379-139X\/9\/5\/144\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:04:04Z","timestamp":1760130244000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2379-139X\/9\/5\/144"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,2]]},"references-count":58,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2023,10]]}},"alternative-id":["tomography9050144"],"URL":"https:\/\/doi.org\/10.3390\/tomography9050144","relation":{},"ISSN":["2379-139X"],"issn-type":[{"value":"2379-139X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,2]]}}}