Article
Rheumatology
Ahmad Nehme, Caroline Arquizan, Alexis Regent, Clothilde Isabel, Nelly Dequatre, Benoit Guillon, Jean Capron, Olivier Detante, Sylvain Lanthier, Alexandre Y. Poppe, Gregoire Boulouis, Sophie Godard, Benjamin Terrier, Christian Pagnoux, Achille Aouba, Emmanuel Touze, Hubert de Boysson
Summary: This study aimed to identify the clinical and radiological characteristics associated with a positive biopsy in PACNS. The results showed that female patients, seizures, cognitive impairment, non-ischaemic parenchymal or leptomeningeal gadolinium enhancement, and the absence of acute brain infarct were more frequently associated with a positive biopsy.
Article
Clinical Neurology
Ahmad Nehme, Sylvain Lanthier, Marion Boulanger, Achille Aouba, Patrice Cacoub, David Jayne, Jean-Paul Makhzoum, Christian Pagnoux, Maxime Rheaume, Benjamin Terrier, Emmanuel Touze, Hubert de Boysson
Summary: This survey explores the current management of PACNS in real-world clinical practice, highlighting areas where physicians lack evidence-based research and/or clinical guidelines.
JOURNAL OF NEUROLOGY
(2023)
Review
Immunology
Shabeer Ahmad Paul, Devlina Roy, Gouranga Prasad Mondal, Ramesh Bhattacharyya, Kartik Chandra Ghosh, Sarbajit Das, Hema Krishna, Chandrakanta Patra, Jyoti Kiran, Julian Benito-Leon
Summary: Primary angiitis of the central nervous system is a rare disease without systemic involvement. The diagnostic approach and management strategies of this disease remain a challenge. Clinical presentation is nonspecific and definitive diagnosis requires brain biopsy. Universally accepted diagnostic criteria and alternative treatment drugs are needed.
JOURNAL OF NEUROIMMUNOLOGY
(2022)
Article
Clinical Neurology
Veit Michael Stoecklein, Lars Kellert, Maximilian Patzig, Clemens Kuepper, Armin Giese, Viktoria Ruf, Jonathan Weller, Friedrich-Wilhelm Kreth, Florian Schoeberl
Summary: This study evaluated the diagnostic accuracy and safety of ESBB in patients with suspected PACNS. Results showed that 7 out of 23 patients were diagnosed with PACNS, while 7 patients were identified with alternative diagnoses. Shorter time period between symptom onset and ESBB significantly increased diagnostic yield.
JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Carlo Salvarani, Jonas Paludo, Gene G. Hunder, Stephen M. Ansell, Caterina Giannini, Joseph E. Parisi, John Huston, Matthew J. Koster, Kenneth J. Warrington, Stefania Croci, Robert D. Brown
Summary: This study used RNA sequencing to explore the gene expression profile of primary central nervous system vasculitis (PCNSV). The results indicated dysfunction in endosomes, mitochondria, and ribosomes, alterations in protein synthesis, and involvement of noncoding RNAs in PCNSV. Pathway analysis revealed the activation of dendritic cell maturation, antigen processing, and neuroinflammation in PCNSV, while oxidative phosphorylation was inhibited. CIBERSORT estimation suggested higher prevalence of activated NK cells, M1 macrophages, memory B cells, and follicular helper T cells in PCNSV samples.
ANNALS OF NEUROLOGY
(2023)
Article
Rheumatology
Shyamashis Das, Rudra Prosad Goswami, Debanjali Sinha, A. Shobhana, Sukalyan Purkayastha, Ashis Datta
Summary: This study observed the diagnosis and treatment effectiveness of primary central nervous system vasculitis (PCNSV). The results showed that MMF may be an effective immunosuppressive therapy for adult PCNSV.
CLINICAL RHEUMATOLOGY
(2023)
Review
Pharmacology & Pharmacy
Constantin Hecker, Tobias Welponer, Manfred Herold, Eugen Trinka, Erasmia Broussalis, Monika Killer-Oberpfalzer
Summary: Central nervous system vasculitis is a rare disease with challenging diagnosis. This review provides an overview of its pathophysiology, current therapeutic guidelines, and novel treatment strategies.
DRUG DISCOVERY TODAY
(2022)
Article
Rheumatology
Hugo Lopez-Arevalo, Maria Emilia Romero Noboa, Dijo Joseph, Ehizogie Edigin, Shilpa Arora, Augustine Manadan
Summary: The study compared the clinical features and outcomes of RCVS and PACNS, finding that RCVS patients were more likely to have cerebral aneurysms, stroke, and hemiplegia/hemiparesis following SAH, while PACNS patients were more likely to have stroke, nontraumatic SAH, seizures, and higher in-hospital mortality rates.
CLINICAL RHEUMATOLOGY
(2022)
Review
Clinical Neurology
A. Nehme, M. Boulanger, A. Aouba, C. Pagnoux, M. Zuber, E. Touze, H. de Boysson
Summary: The clinical manifestations of central nervous system vasculitis are diverse, and can be classified based on the size of involved vessels. Diagnosis relies on markers of vascular disease and inflammation, as well as cerebrospinal fluid and vascular imaging. Treatment primarily involves immunosuppression, and a multidisciplinary approach is necessary.
REVUE NEUROLOGIQUE
(2022)
Article
Clinical Neurology
Hitomi Onomura, Soma Furukawa, Suguru Nishida, Satoshi Kitagawa, Mari Yoshida, Yasuhiro Ito
Summary: This case study describes a specific subtype of primary angiitis of the central nervous system (PACNS) in a 17-year-old girl. The lesions were confined to the left hemisphere, and detailed imaging findings were described. The patient received immunosuppressive treatment and showed gradual improvement.
Article
Biotechnology & Applied Microbiology
Toshiki Ariyoshi, Madoka Hoshide, Takahiro Motonaga, Yuno Korenaga, Yoshihiro Azuma, Takuya Ichimura, Takeshi Matsushige, Shunji Hasegawa
Summary: This article reports a case of cPACNS occurring after COVID-19 vaccination. The patient presented with symptoms of headache and fever after vaccination, and examination revealed vascular stenosis. After treatment, the symptoms improved.
HUMAN VACCINES & IMMUNOTHERAPEUTICS
(2023)
Article
Clinical Neurology
Ahmet Kursat Karaman, Bora Korkmazer, Serdar Arslan, Ugur Uygunoglu, Ercan Karaarslan, Osman Kizilkilic, Naci Kocer, Civan Islak
Summary: This study demonstrates the diagnostic value of VWI in differentiating PACNS from other vasculopathies and its role in post-treatment follow-up. VWI findings were crucial in confirming PACNS diagnosis and distinguishing it from other conditions, with high sensitivity and specificity. Vessel wall enhancement regression during follow-up suggests that VWI may be a useful adjunct for monitoring PACNS patients post-treatment.
Article
Pediatrics
Morgane Belcour, Pauline Dontaine, Anne Monier, Laetitia Lebrun, Isabelle Salmon, Olivier De Witte, Serge Goldman, Xavier De Tiege, Alec Aeby
Summary: PACNS is a rare inflammatory disease affecting central nervous system vessels, and its diagnosis is challenging. This study demonstrates that combining MRI and PET can increase the diagnostic yield of this disease.
FRONTIERS IN PEDIATRICS
(2022)
Review
Clinical Neurology
Gelsomina Mansueto, Giuseppe Lanza, Francesco Fisicaro, Danielle Alaouieh, Emily Hong, Sara Girolami, Marco Montella, Alessandro Feola, Mario Di Napoli
Summary: This review provides a comprehensive update on the clinical assessment, diagnosis, complications, and treatment of PCNSV. Recent developments and advances in technology have improved diagnostic accuracy and provided new insights for preventing misdiagnosis. However, treatment for PCNSV remains largely empirical.
CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS
(2022)
Article
Medicine, General & Internal
Shamik Bhattacharyya, R. Gilberto Gonzalez, Bart K. Chwalisz, Samantha N. Champion
Summary: A 64-year-old woman presented with symptoms of cognitive impairment, headache, and memory loss for 6 weeks. MRI findings showed abnormal signals in the subarachnoid and leptomeningeal regions on FLAIR sequences. A diagnostic test was performed to determine the cause.
NEW ENGLAND JOURNAL OF MEDICINE
(2021)
Article
Rheumatology
Gaelle Chedeville, Katherine McGuire, David A. Cabral, Natalie J. Shiff, Dax G. Rumsey, Jean-Philippe Proulx-Gauthier, Heinrike Schmeling, Roberta A. Berard, Michelle Batthish, Gordon Soon, Kerstin Gerhold, Tommy Gerschman, Alessandra Bruns, Ciaran M. Duffy, Lori B. Tucker, Jaime Guzman
Summary: This study aims to describe the frequency and severity of medication side effects (SEs) in children with Juvenile Idiopathic Arthritis (JIA) and their impact on health-related quality of life (HRQoL). The results show that two-thirds of children with JIA experience SEs within 1 year of diagnosis, which negatively affects their HRQoL.
ARTHRITIS CARE & RESEARCH
(2022)
Letter
Gastroenterology & Hepatology
Molly J. Dushnicky, Karen A. Beattie, Tania Cellucci, Liane Heale, Mary Zachos, Mary Sherlock, Michelle Batthish
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2022)
Article
Rheumatology
Molly J. Dushnicky, Catherine Campbell, Karen A. Beattie, Roberta Berard, Tania Cellucci, Mercedes Chan, Tommy Gerschman, Nicole Johnson, Lillian Lim, Nadia Luca, Paivi Miettunen, Kimberly A. Morishita, Jean-Philippe Proulx-Gauthier, Dax G. Rumsey, Heinrike Schmeling, Rosie Scuccimarri, Herman Tam, Jaime Guzman, Michelle Batthish
Summary: The COVID-19 pandemic has not significantly impacted the initial presentation of JIA and JIA-related research in Canada. There was no observed delay in time to presentation or increased severity at presentation. However, there was a decrease in registry enrollment during the pandemic.
Article
Rheumatology
Ruud H. J. Verstegen, Peter Shrader, Stephen J. Balevic, Timothy Beukelman, Colleen Correll, Anne Dennos, Thomas Phillips, Brian M. Feldman
Summary: This study aimed to determine the dose-response relationship of tumor necrosis factor (TNF) inhibition in the treatment of juvenile idiopathic arthritis (JIA). The results did not confirm the hypothesis that increased dosing of TNF inhibitors leads to improved treatment outcomes.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Pediatrics
William Daniel Soulsby, Nayimisha Balmuri, Victoria Cooley, Linda M. Gerber, Erica Lawson, Susan Goodman, Karen Onel, Bella Mehta
Summary: Social determinants of health have a significant impact on the outcomes of rheumatoid arthritis treatment. Our study found that community poverty level, race/ethnicity, and economic factors are associated with disease activity and functional disability in polyarticular juvenile idiopathic arthritis patients.
PEDIATRIC RHEUMATOLOGY
(2022)
Article
Rheumatology
Jennifer J. Y. Lee, Simon W. M. Eng, Jaime Guzman, Ciaran M. Duffy, Lori B. Tucker, Kiem Oen, Rae S. M. Yeung, Brian M. Feldman
Summary: This study compared the ILAR and PRINTO JIA classification criteria in a cohort of JIA patients. The results showed that a large proportion of JIA patients were unclassifiable using the PRINTO criteria, and the alignment with clinicobiologic subtypes and adult forms of arthritis was not as good as the ILAR criteria.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Rheumatology
Jonathan Park, Michelle Batthish, Roberta A. Berard, Gaelle Chedeville, Jean-Philippe Proulx-Gauthier, Dax G. Rumsey, Lori B. Tucker, Stephanie Wong, Jaime Guzman
Summary: Canadian pediatric rheumatology practices were generally in line with the 2019 JIA guideline recommendations, except for the frequent use of oral MTX and infrequent direct escalation from NSAIDs to bDMARDs in sacroiliitis and enthesitis.
Article
Rheumatology
Molly J. Dushnicky, Claire Fine, Tania Cellucci, Stephanie Garner, Liane Heale, Mark Matsos, Karen A. Beattie, Michelle Batthish
Summary: This study aims to identify gaps in transition care for adolescent patients with juvenile idiopathic arthritis or systemic lupus erythematosus and their parents. The results show that there are discrepancies between the current care and the ideal experience, with patients focusing on process issues and parents focusing on management of the environment.
JCR-JOURNAL OF CLINICAL RHEUMATOLOGY
(2023)
Review
Economics
Gillian R. Currie, Brittany Gerber, Diane Lorenzetti, Karen MacDonald, Susanne M. Benseler, Francois P. Bernier, Kym M. Boycott, K. Vanessa Carias, Bettina Hamelin, Robin Z. Hayeems, Claire LeBlanc, Marinka Twilt, Gijs van Rooijen, Durhane Wong-Rieger, Rae S. M. Yeung, Deborah A. Marshall
Summary: Rare diseases impose a significant burden on various aspects. Limited evidence exists on the socioeconomic burden of rare diseases, particularly for those without available treatments. This study developed a framework to guide future research on the cost elements of rare diseases.
Meeting Abstract
Rheumatology
Simran Heera, Karen Beattie, Julie Herrington, Lauren Heesels, Tania Cellucci, Stephanie Garner, Liane Heale, Mark Matsos, Michelle Batthish
JOURNAL OF RHEUMATOLOGY
(2023)
Meeting Abstract
Rheumatology
Christina Ma, Habeba Talaat, Alessana Carmona, Julie Herrington, Tania Cellucci, Stephanie Garner, Mark Matsos, Karen Beattie, Michelle Batthish
JOURNAL OF RHEUMATOLOGY
(2022)
Meeting Abstract
Rheumatology
Claire Fine, Karen Beattie, Tania Cellucci, Stephanie Garner, Liane Heale, Mark Matsos, Michelle Batthish
JOURNAL OF RHEUMATOLOGY
(2022)
Meeting Abstract
Rheumatology
Jessica Scott, Tania Cellucci, Evelyn Rozenblyum, Rahul Chanchlani, Karen Beattie
JOURNAL OF RHEUMATOLOGY
(2022)
Meeting Abstract
Rheumatology
Teresa Semalulu, Karen Beattie, Alessana Carmona, Tania Cellucci, Liane Heale, Stephanie Garner, Jan Gorter, Mark Matsos, Michelle Batthish
JOURNAL OF RHEUMATOLOGY
(2022)
Meeting Abstract
Rheumatology
Jason An, Madeline Couse, Daniela Dominguez, Michelle Batthish, Roberta Berard, Tania Cellucci, Dilan Dissanayake, Liane Heale, Ronald Laxer, Christian Marshall, Johannes Roth, Robert Rottapel, Linda Hiraki
JOURNAL OF RHEUMATOLOGY
(2022)