Biochemistry, Genetics and Molecular Biology (miscellaneous)RheumatologyImmunology and AllergyImmunology
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Annals of the Rheumatic Diseases (ARD) is an international peer review journal committed to promoting the highest standards of scientific exchange and education. It covers all aspects of rheumatology, which includes the spectrum of musculoskeletal conditions, arthritic disease, and connective tissue disorders. ARD publishes basic, clinical, and translational scientific research. Concise scientific communication is encouraged and peer reviewed proceedings of international meetings are featured. Educational papers include state of the art reviews, "how to" articles and educational cases that focus on problems faced in clinical practice. The journal was first published in 1939 and has an authorative global Editorial Board and a growing international readership.
Marie Pouletty, Charlotte Borocco, Naïm Ouldali, Marion Caseris, Romain Basmaci, Noémie Lachaume, Philippe Bensaïd, Samia Pichard, Hanane Kouider, Guillaume Morelle, Irina Craiu, Corinne Pondarré, Anna Dehò, A. Maroni, Mehdi Oualha, Zahir Amoura, Julien Haroche, Juliette Chommeloux, Fanny Bajolle, Constance Beyler, Stéphane Bonacorsi, Guislaine Carcelain, Isabelle Koné‐Paut, Brigitte Bader‐Meunier, Albert Faye, Ulrich Meinzer, Caroline Galeotti, Isabelle Melki
BackgroundCurrent data suggest that COVID-19 is less frequent in children, with a milder course. However, over the past weeks, an increase in the number of children presenting to hospitals in the greater Paris region with a phenotype resembling Kawasaki disease (KD) has led to an alert by the French national health authorities.MethodsMulticentre compilation of patients with KD in Paris region since April 2020, associated with the detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (‘Kawa-COVID-19’). A historical cohort of ‘classical’ KD served as a comparator.ResultsSixteen patients were included (sex ratio=1, median age 10 years IQR (4·7 to 12.5)). SARS-CoV-2 was detected in 12 cases (69%), while a further three cases had documented recent contact with a quantitative PCR-positive individual (19%). Cardiac involvement included myocarditis in 44% (n=7). Factors prognostic for the development of severe disease (ie, requiring intensive care, n=7) were age over 5 years and ferritinaemia >1400 µg/L. Only five patients (31%) were successfully treated with a single intravenous immunoglobulin (IVIg) infusion, while 10 patients (62%) required a second line of treatment. The Kawa-COVID-19 cohort differed from a comparator group of ‘classical’ KD by older age at onset 10 vs 2 years (p<0.0001), lower platelet count (188 vs 383 G/L (p<0.0001)), a higher rate of myocarditis 7/16 vs 3/220 (p=0.0001) and resistance to first IVIg treatment 10/16 vs 45/220 (p=0.004).ConclusionKawa-COVID-19 likely represents a new systemic inflammatory syndrome temporally associated with SARS-CoV-2 infection in children. Further prospective international studies are necessary to confirm these findings and better understand the pathophysiology of Kawa-COVID-19.Trial registration numberNCT02377245
Ingrid E. Lundberg, Anna Tjärnlund, Matteo Bottai, Victoria P. Werth, Clarissa Pilkington, Marianne de Visser, Lars Alfredsson, Anthony A. Amato, Richard J. Barohn, Matthew H. Liang, Jasvinder A. Singh, Rohit Aggarwal, Snjólaug Arnardottir, Hector Chinoy, Robert G. Cooper, Katalin Dankó, Mazen M. Dimachkie, Brian M. Feldman, Ignacio García‐De La Torre, Patrick Gordon, Taichi Hayashi, James D. Katz, Hitoshi Kohsaka, Peter A. Lachenbruch, Bianca Lang, Yuhui Li, Chester V. Oddis, Marzena Olesińska, Ann M. Reed, Lidia Rutkowska‐Sak, Helga Sanner, Albert Selva-O’Callaghan, Yeong Wook Song, Jiří Vencovský, Steven R. Ytterberg, Frederick Miller, Lisa G. Rider
ObjectiveTo develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups.MethodsCandidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology and paediatric clinics worldwide. Several statistical methods were used to derive the classification criteria.ResultsBased on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived. Each item is assigned a weighted score. The total score corresponds to a probability of having IIM. Subclassification is performed using a classification tree. A probability cut-off of 55%, corresponding to a score of 5.5 (6.7 with muscle biopsy) ‘probable IIM’, had best sensitivity/specificity (87%/82% without biopsies, 93%/88% with biopsies) and is recommended as a minimum to classify a patient as having IIM. A probability of ≥90%, corresponding to a score of ≥7.5 (≥8.7 with muscle biopsy), corresponds to ‘definite IIM’. A probability of <50%, corresponding to a score of <5.3 (<6.5 with muscle biopsy), rules out IIM, leaving a probability of ≥50 to <55% as ‘possible IIM’.ConclusionsThe European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology and paediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of ‘definite’, ‘probable’ and ‘possible’ IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria.