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4. Prévision de la résistance aux immunoglobulines dans la maladie de Kawasaki dans des populations multiethniques en Europe : étude de cohorte multicentrique

Author

Ouldali, N., primary, Dellepiane, R.M., additional, Torreggiani, S., additional, Mauri, L., additional, Beaujour, G., additional, Beyler, C., additional, Cucchetti, M., additional, Dumaine, C., additional, Lavecchia, A., additional, Melki, I., additional, Stracquadaino, R., additional, Vinit, C., additional, Meinzer, U., additional, and Cimaz, R., additional

Published
2022
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5. Canakinumab in systemic juvenile idiopathic arthritis: real-world data from a retrospective Italian cohort

Author

De Matteis A, Bracaglia C, Pires Marafon D, Piscitelli AL, Alessio M, Naddei R, Orlando F, Filocamo G, Minoia F, Ravelli A, Tibaldi J, Cimaz R, Marino A, Simonini G, Mastrolia MV, La Torre F, Tricarico I, Licciardi F, Montin D, Maggio MC, Alizzi C, Martini G, Civino A, Gallizzi R, Olivieri AN, Ardenti Morini F, Conti G, De Benedetti F, Pardeo M., De Matteis, A, Bracaglia, C, Pires Marafon, D, Piscitelli, Al, Alessio, M, Naddei, R, Orlando, F, Filocamo, G, Minoia, F, Ravelli, A, Tibaldi, J, Cimaz, R, Marino, A, Simonini, G, Mastrolia, Mv, La Torre, F, Tricarico, I, Licciardi, F, Montin, D, Maggio, Mc, Alizzi, C, Martini, G, Civino, A, Gallizzi, R, Olivieri, An, Ardenti Morini, F, Conti, G, De Benedetti, F, and Pardeo, M.

Published
2022

6. Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey

Author

Cattalini M, Della Paolera S, Zunica F, Bracaglia C, Giangreco M, Verdoni L, Meini A, Sottile R, Caorsi R, Zuccotti G, Fabi M, Montin D, Meneghel A, Consolaro A, Dellepiane RM, Maggio MC, La Torre F, Marchesi A, Simonini G, Villani A, Cimaz R, Ravelli A, Taddio A Maria Concetta Alberelli: UOC Pediatria, Marche-Nord, Clotilde Alizzi: Department of Health Promotion Sciences Maternal and Infantile Care, Internal Medicine and Medical Specialities 'G. D’Alessandro', University of Palermo, Palermo Italy, Patrizia Barone: Unità Operativa Complessa di Broncopneumologia Pediatrica AOU 'Policlinico - Vittorio Emanuele Via Santa Sofia 78 Catania, Lucia Augusta Baselli: Pediatric Intermediate Care Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan Italy, Veronica Bennato: U. O. Pediatria, Ospedale A, Manzoni Lecco, Francesca Biscaro: UOC Pediatria, Ospedale Ca’ Foncello, Treviso, Grazia Bossi: UOC Pediatria, Fondazione IRCCS Policlinico San Matteo, Pavia Italy, Andrea Campana: Bambino Gesù Children’s Hospital, Rome Italy, Maurizio Carone: UO Malattie Infettive, Ospedale Pediatrico ‘Giovanni XXIII’, Bari Italy, Adele Civino: U. O. C. Pediatria P. O. Vito Fazzi, Lecce, Giovanni Conti: Nefrologia e Reumatologia Pediatrica con Dialisi, Azienda Ospedaliero-Universitario 'G. Martino', Eleonora Dei Rossi: University of Trieste, Trieste Italy, Emanuela Del Giudice: Department of Maternal Infantile and Urological Sciences, Sapeinza University of Rome, Polo Pontini, Alice Dell’Anna: U. O. C. Pediatria P. O. Vito Fazzi Lecce, Maia De Luca: Bambino Gesù Children’s Hospital, Piazza S. Onofrio n. 4, 00165 Rome, Italy, Enrico Felici: Pediatric and Pediatric Emergency Unit, The Children Hospital, AO SS Antonio e Biago e C. Arrigo, Alessandria Italy, Giovanni Filocamo: Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Milano, Ilenia Floretta: Pediatria, Ospedale Santa Chiara, Trento Italy, Maria Loreta Foschini: SC Pediatria, PO SAN MICHELE AOBrotzu, Cagliari Italy, Marcello Lanari: Department of Pediatrics, University of Bologna, IRCCS S. Orsola-Malpighi Hospital, Bologna Italy, Bianca Lattanzi: SOD Pediatria, Ospedali Riuniti, Ancona Italy, Alessandra Lazzerotti: Clinica Pediatrica, Università Milano Bicocca, Fondazione MBBM - onlus c/o Ospedale San Gerardo, Monza Italy, Francesco Licciardi: Department of Pediatrics and Public Health, University of Turin, Turin Italy, Alessandra Manerba: Child Cardiology, ASST Spedali Civili di Brescia and University of Brescia, Brescia Italy, Savina Mannarino: Division of Cardiology, Children’s Hospital V Buzzi, ASST FBF Sacco, Achille Marino: Department of Pediatrics, Desio Hospital, ASST Monza, Desio Italy, Agostina Marolda: Pediatrics and Neonatology Dipartment, ASST Ovest Milanese, 'G. Fornaroli' Hospital, Magenta Milan, Laura Martelli: Paediatric Department, Hospital Papa Giovanni XXIII, Bergamo Italy, Giorgia Martini, Department of Woman’s and Child’s Health, University of Padova, Padua Italy, Angela Mauro: Department of Paediatrics, Emergency Department, Santobono-Pausilipon Children’s Hospital, Naples, Italy. Maria Vincenza Mastrolia: Pediatric Rheumatology Unit, AOU Meyer, University of Florence, Florence, Italy. Angelo Mazza: Paediatric Department, Angela Miniaci: Clinica Pediatrica, Reumatologia, Azienda Ospedaliero-Universitaria di Bologna, Francesca Minoia: Fondazione IRCCS Cà Granda, Alma Olivieri: Dipartimento della donna, del bambino e di chirurgia generale e specialistica, Università della Campania, 'L Vanvitelli, Napoli, Guido Pennoni: Dipartimento Materno-Infantile, Gubbio-Gualdo Tadino, Italy, Rossana Pignataro: UOC Pediatria e Neonatologia, ASST Lodi, Lodi, Francesca Ricci, Clinica Pediatrica, ASST Spedali Civili di Brescia e Università degli Studi di Brescia, Donato Rigante: Department of Pediatrics, Univarsità Cattolica Sacro Cuore, Matilde Rossi: UOC di Pediatrai e Neonatologia, Ospedale di Macerata, Macerata, Claudia Santagati: Dipartimento di Pediatria, Ospedale di Rovigo, Rovigo, Martina Soliani: Pediatria ASST Cremona, Italy, Silvia Sonego: University of Trieste, Domenico Sperlì: UOC di Pediatria, S. O. 'Annunziata' - A. O. di Cosenza, Sara Stucchi: Maternal and Child Health, Division of Paediatrics, ASST Grande Ospedale Metropolitano Niguarda, Milano Italy, Barbara Teruzzi: Maternal and Child Health, Elpidio Tierno: UOC di Pediatria, Dipartimento della Salute della Donna e del Bambin, AORN 'Sant’Anna e San Sebastiano'- Caserta, Tatiana Utytatnikova: Dipartimento Materno-Infantile, Pediatria, ASST Bergamo-EST, Seriate Bergamo, Piero Valentini, Department of Pediatrics, Gianluca Vergine, UOC Pediatria Rimini, Ospedale Infermi, ASL Romagna, Rimini Italy., Cattalini, Marco, Della Paolera, Sara, Zunica, Fiammetta, Bracaglia, Claudia, Giangreco, Manuela, Verdoni, Lucio, Meini, Antonella, Sottile, Rita, Caorsi, Roberta, Zuccotti, Gianvincenzo, Fabi, Marianna, Montin, Davide, Meneghel, Alessandra, Consolaro, Alessandro, Dellepiane, Rosa Maria, Maggio, Maria Cristina, La Torre, Francesco, Marchesi, Alessandra, Simonini, Gabriele, Villani, Alberto, Cimaz, Rolando, Ravelli, Angelo, Taddio, Andrea, Cattalini, M, Della Paolera, S, Zunica, F, Bracaglia, C, Giangreco, M, Verdoni, L, Meini, A, Sottile, R, Caorsi, R, Zuccotti, G, Fabi, M, Montin, D, Meneghel, A, Consolaro, A, Dellepiane, Rm, Maggio, Mc, La Torre, F, Marchesi, A, Simonini, G, Villani, A, Cimaz, R, Ravelli, A, Taddio, A Maria Concetta Alberelli: UOC Pediatria, Marche-Nord, Clotilde Alizzi: Department of Health Promotion Sciences Maternal and Infantile, Care, Internal Medicine and Medical Specialities 'G., D’Alessandro', University of, Palermo, Palermo, Italy, Patrizia Barone: Unità Operativa Complessa di Broncopneumologia Pediatrica AOU 'Policlinico, - Vittorio Emanuele Via Santa Sofia 78 Catania, Lucia Augusta Baselli: Pediatric Intermediate Care, Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore, Policlinico, Milan, Italy, Veronica Bennato: U. O., Pediatria, Ospedale, A, Manzoni, Lecco, Francesca Biscaro: UOC, Pediatria, Ospedale Ca’, Foncello, Treviso, Grazia Bossi: UOC, Pediatria, Fondazione IRCCS Policlinico San, Matteo, Pavia, Italy, Andrea Campana: Bambino Gesù Children’s, Hospital, Rome, Italy, Maurizio Carone: UO Malattie, Infettive, Ospedale Pediatrico ‘Giovanni, Xxiii’, Bari, Italy, Adele Civino: U. O. C. Pediatria P. O. Vito Fazzi, Lecce, Giovanni Conti: Nefrologia e Reumatologia Pediatrica con Dialisi, Azienda Ospedaliero-Universitario 'G. Martino', Eleonora Dei Rossi: University of, Trieste, Trieste, Italy, Emanuela Del Giudice: Department of Maternal Infantile and Urological, Science, Sapeinza University of, Rome, Polo, Pontini, Alice Dell’Anna: U. O. C. Pediatria P. O., Vito Fazzi Lecce, Maia De Luca: Bambino Gesù Children’s, Hospital, Piazza, S. Onofrio n. 4., 00165, Rome, Italy, Enrico Felici: Pediatric and Pediatric Emergency, Unit, The Children, Hospital, AO SS Antonio e Biago e C., Arrigo, Alessandria, Italy, Giovanni Filocamo: Fondazione IRCCS Cà, Granda, Ospedale Maggiore, Policlinico, Milano, Ilenia Floretta:, Pediatria, Ospedale Santa, Chiara, Trento, Italy, Maria Loreta Foschini: SC, Pediatria, PO SAN MICHELE, Aobrotzu, Cagliari, Italy, Marcello Lanari: Department of, Pediatric, University of, Bologna, IRCCS S., Orsola-Malpighi Hospital, Bologna, Italy, Bianca Lattanzi: SOD, Pediatria, Ospedali, Riuniti, Ancona, Italy, Alessandra Lazzerotti: Clinica, Pediatrica, Università Milano, Bicocca, Fondazione MBBM, - onlus c/o Ospedale San Gerardo, Monza, Italy, Francesco Licciardi: Department of Pediatrics and Public, Health, University of, Turin, Turin, Italy, Alessandra Manerba: Child, Cardiology, ASST Spedali Civili di Brescia and University of, Brescia, Brescia, Italy, Savina Mannarino: Division of, Cardiology, Children’s Hospital, V Buzzi, ASST FBF, Sacco, Achille Marino: Department of, Pediatric, Desio, Hospital, Asst, Monza, Desio, Italy, Agostina Marolda: Pediatrics and Neonatology, Dipartment, ASST Ovest, Milanese, 'G., Fornaroli' Hospital, Magenta, Milan, Laura Martelli: Paediatric, Department, Hospital Papa Giovanni, Xxiii, Bergamo, Italy, Giorgia, Martini, Department of Woman’s and Child’s, Health, University of, Padova, Padua, Italy, Angela Mauro: Department of, Paediatric, Emergency, Department, Santobono-Pausilipon Children’s, Hospital, Naples, Italy., Maria Vincenza Mastrolia: Pediatric Rheumatology Unit, Aou, Meyer, University of, Florence, Florence, Italy., Angelo Mazza: Paediatric Department, Angela Miniaci: Clinica, Pediatrica, Reumatologia, Azienda Ospedaliero-Universitaria di, Bologna, Francesca Minoia: Fondazione IRCCS Cà, Granda, Alma Olivieri: Dipartimento della, Donna, del bambino e di chirurgia generale, e specialistica, Università della, Campania, 'L, Vanvitelli, Napoli, Claudio, Guido Pennoni: Dipartimento, Materno-Infantile, Gubbio-Gualdo Tadino, Italy, Rossana Pignataro: UOC Pediatria, e Neonatologia, Asst, Lodi, Lodi, Francesca, Ricci, Clinica, Pediatrica, ASST Spedali Civili di Brescia, e Università degli Studi di Brescia, Donato Rigante: Department of, Pediatric, Univarsità Cattolica Sacro, Cuore, Matilde Rossi: UOC di Pediatrai, e Neonatologia, Ospedale di, Macerata, Macerata, Claudia Santagati: Dipartimento di, Pediatria, Ospedale di, Rovigo, Rovigo, Martina Soliani: Pediatria ASST Cremona, Italy, Silvia Sonego: University of, Trieste, Domenico Sperlì: UOC di Pediatria, S. O. 'Annunziata' - A. O. di Cosenza, Sara Stucchi: Maternal and Child, Health, Division of, Paediatric, ASST Grande Ospedale Metropolitano, Niguarda, Milano, Italy, Barbara Teruzzi: Maternal and Child, Health, Elpidio Tierno: UOC di, Pediatria, Dipartimento della Salute della Donna, e del Bambin, AORN 'Sant’Anna, e San Sebastiano'- Caserta, Tatiana Utytatnikova: Dipartimento, Materno-Infantile, Pediatria, Asst, Bergamo-EST, Seriate, Bergamo, Piero, Valentini, Department of, Pediatric, Gianluca, Vergine, UOC Pediatria, Rimini, Ospedale, Infermi, Asl, Romagna, Rimini, Italy., Cattalini M., Della Paolera S., Zunica F., Bracaglia C., Giangreco M., Verdoni L., Meini A., Sottile R., Caorsi R., Zuccotti G., Fabi M., Montin D., Meneghel A., Consolaro A., Dellepiane R.M., Maggio M.C., La Torre F., Marchesi A., Simonini G., Villani A., Cimaz R., Ravelli A., Taddio A., Adamoli P., Alberelli M.C., Alizzi C., Barone P., Bennato V., Biscaro F., Bossi G., Campana A., Carone M., Civino A., Conti G., Rossi E.D., Del Giudice E., Dell'Anna A., De Luca M., Felici E., Filocamo G., Floretta I., Foschini M.L., Lanari M., Lattanzi B., Lazzerotti A., Licciardi F., Manerba A., Mannarino S., Marino A., Marolda A., Martelli L., Martini G., Mauro A., Mastrolia M.V., Mazza A., Miniaci A., Minoia F., Olivieri A., Pennoni G., Pignataro R., Ricci F., Rigante D., Rossi M., Santagati C., Soliani M., Sonego S., Sperli D., Stucchi S., Teruzzi B., Tierno E., Utytatnikova T., Valentini P., and Vergine G.

Subjects
Coronary artery abnormalities, Hypotension, Kawasaki disease, Multisystem inflammatory syndrome associated with coronavirus disease, Myocarditis, Pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection, SARS-CoV-2, Age Distribution, Antirheumatic Agents, Aspirin, C-Reactive Protein, COVID-19, Child, Child, Preschool, Coronary Artery Disease, Cough, Diarrhea, Dyspnea, Female, Glucocorticoids, Heart Failure, Humans, Hyperferritinemia, Immunoglobulins, Intravenous, Immunologic Factors, Infant, Intensive Care Units, Pediatric, Interleukin 1 Receptor Antagonist Protein, Italy, Lymphopenia, Male, Mucocutaneous Lymph Node Syndrome, Platelet Aggregation Inhibitors, Shock, Systemic Inflammatory Response Syndrome, Tachypnea, Troponin T, Vomiting, lcsh:Diseases of the musculoskeletal system, coronary artery abnormalities, hypotension, kawasaki disease, multisystem inflammatory syndrome associated with coronavirus disease, myocarditis, pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection, age distribution, antirheumatic agents, aspirin, C-reactive protein, child, preschool, coronary artery disease, cough, diarrhea, yspnea, female, glucocorticoids, heart failure, humans, hyperferritinemia, immunoglobulins, intravenous, immunologic factors, infant, intensive care units, pediatric, interleukin 1 receptor antagonist protein, italy, lymphopenia, male, mucocutaneous lymph node syndrome, platelet aggregation inhibitors, shock, systemic inflammatory response syndrome, tachypnea, troponin T, vomiting, Myocarditi, 030204 cardiovascular system & hematology, SARS-CoV-2, Kawasaki disease, Pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection, Myocarditis, Hypotension, Multisystem inflammatory syndrome associated with coronavirus disease, Coronary artery abnormalities, Coronary artery disease, Settore MED/38 - Pediatria Generale E Specialistica, 0302 clinical medicine, Glucocorticoid, Immunologic Factor, Immunology and Allergy, Coronary artery abnormalitie, Fisher's exact test, Pediatric, biology, lcsh:RJ1-570, Antirheumatic Agent, Settore MED/38, Intensive Care Units, Cohort, symbols, Platelet aggregation inhibitor, Intravenous, Human, Research Article, medicine.medical_specialty, Immunoglobulins, 03 medical and health sciences, symbols.namesake, Rheumatology, Internal medicine, medicine, Preschool, 030203 arthritis & rheumatology, business.industry, Platelet Aggregation Inhibitor, lcsh:Pediatrics, medicine.disease, Systemic inflammatory response syndrome, Immunoglobulins, Intravenou, Pediatrics, Perinatology and Child Health, biology.protein, lcsh:RC925-935, business
Abstract

Background There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities. Methods The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group – KDG) or KD-like (Kawacovid Group - KCG) disease between February 1st 2020, and May 31st 2020. Demographic, clinical, laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAP®). Relationship between clinical presentation and SARS-CoV-2 infection was also taken into account. Moreover, clinical characteristics of KDG during SARS-CoV-2 epidemic (KDG-CoV2) were compared to Kawasaki Disease patients (KDG-Historical) seen in three different Italian tertiary pediatric hospitals (Institute for Maternal and Child Health, IRCCS “Burlo Garofolo”, Trieste; AOU Meyer, Florence; IRCCS Istituto Giannina Gaslini, Genoa) from January 1st 2000 to December 31st 2019. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groups. Results One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis. 37,8% of patients among KCG presented hypotension/non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated ferritin and troponin-T characterized KCG. KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p = 0.04 and 71,9% vs 43,4%; p = 0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p Conclusion Our study suggests that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD and PIMS-TS. Older age at onset and clinical peculiarities like the occurrence of myocarditis characterize this multi-inflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.

Published
2020

7. POS0172 DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT AND HAVE MORE SEVERE DISEASE IN THE LARGEST jSSc COHORT OF THE WORLD. RESULTS FROM THE THE JUVENILE SCLERODERMA INCEPTION COHORT. www.juvenile-scleroderma.com

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Sztajnbok, F. R., additional, Stanevicha, V., additional, Anton, J., additional, Johnson, S., additional, Khubchandani, R., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Malcova, H., additional, Marrani, E., additional, Pain, C., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Costa Reis, P., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Abu Al Saoud, S., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2022
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8. AB1236 CLINICAL CHARACTERISTICS OF JUVENILE ONSET SYSTEMIC SCLEROSIS PATIENTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT COMPARED TO ADULT AGE JUVENILE-ONSET PATIENTS FROM EUSTAR. ARE THESE DIFFERENCES SUGGESTING RISK FOR MORTALITY?

Author

Foeldvari, I., primary, Klotsche, J., additional, Carreira, P., additional, Kasapcopur, O., additional, Torok, K., additional, Airò, P., additional, Iannone, F., additional, Allanore, Y., additional, Balbir-Gurman, A., additional, Schmeiser, T., additional, Sztajnbok, F. R., additional, Terreri, M. T., additional, Stanevicha, V., additional, Anton, J., additional, Feldman, B., additional, Khubchandani, R., additional, Alexeeva, E., additional, Johnson, S., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Campochiaro, C., additional, De Vries-Bouwstra, J., additional, Kostik, M., additional, Lehman, T., additional, Marrani, E., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Janarthanan, M., additional, Malcova, H., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Seskute, G., additional, Truchetet, M. E., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Costa Reis, P., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, Veale, D., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, and Distler, O., additional

Published
2022
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9. POS1302 PATIENT AND PHYSICIAN REPORTED OUTCOMES OF JUVENILE SYSTEMIC SCLEROSIS PATIENTS SIGNIFICANTLY IMPROVE OVER 12 MONTHS OBSERVATION PERIOD IN THE JUVENILE SYSTEMIC SCLERODERMA INCEPTION COHORT. www.juvenile-scleroderma.com

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Anton, J., additional, Katsikas, M., additional, Stanevicha, V., additional, Sztajnbok, F. R., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Marrani, E., additional, Sifuentes-Giraldo, W. A., additional, Johnson, S., additional, Khubchandani, R., additional, Nemcova, D., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Janarthanan, M., additional, Kallinich, T., additional, Minden, K., additional, Moll, M., additional, Nielsen, S., additional, Patwardhan, A., additional, Schonenberg, D., additional, Smith, V., additional, and Helmus, N., additional

Published
2022
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10. POS1299 JUVENILE SYSTEMIC SCLEROSIS TREATMENT PRACTICES IN AN INTERNATIONAL COHORT AND COMPARISON TO RECENT SHARE CONSENSUS GUIDELINES.

Author

Foeldvari, I., primary, Torok, K., additional, Kasapcopur, O., additional, Adrovic, A., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Anton, J., additional, Sztajnbok, F. R., additional, Stanevicha, V., additional, Appenzeller, S., additional, Avcin, T., additional, Johnson, S., additional, Khubchandani, R., additional, Kostik, M., additional, Marrani, E., additional, Sifuentes-Giraldo, W. A., additional, Nemcova, D., additional, Santos, M. J., additional, Schonenberg, D., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Janarthanan, M., additional, Kallinich, T., additional, Lehman, T., additional, Moll, M., additional, Nuruzzaman, F., additional, Patwardhan, A., additional, Smith, V., additional, and Helmus, N., additional

Published
2022
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12. Anti-SSA/Ro positivity and congenital heart block: obstetric and foetal outcome in a cohort of anti-SSA/Ro positive pregnant patients with and without autoimmune diseases

Author

Fredi, M, Argolini, Lm, Angeli, F, Trespidi, L, Ramoni, V, Zatti, S, Vojinovic, T, Donzelli, D, Gazzola, Fg, Xoxi, B, Andreoli, L, Lojacono, A, Ferrazzi, E, Montecucco, C, Chighizola, Cb, Meroni, Pl, Franceschini, F, Cimaz, R, Caporali, R, Tincani, A, and Gerosa, M

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

Neonatal lupus (NL) is an acquired disease caused by the transplacental passage of anti-SSA/Ro antibodies. The rate of congenital heart block (CHB), its most serious manifestation, ranges from 1 to 5%. The aim of this study was to retrospectively assess the prevalence of CHB in anti-SSA/Ro positive pregnant women with or without systemic autoimmune diseases from 2010 to 2020.Patients underwent monthly visit and a shared follow-up programme of weekly (16th-24th week) foetal heart rate assessment by obstetric ultrasound.322 pregnancies in 258 anti-SSA/Ro patients were included; 314 were followed from the beginning of pregnancy because of the known presence of anti-SSA/Ro autoantibodies and 1 case of CHB occurred in an anti-SSA/Ro+ asymptomatic subject (0.3%). In the same period, 8 additional patients were referred to our clinics after in utero CHB diagnosis and subsequent discovery of anti-SSA/Ro without a disease diagnosis. Globally, 9 cases of congenital CHB (2.8%) occurred: 7 complete, 1 II-III degree and 1 rst degree CHB. Anti-SSB/La positivity was associated with a higher risk of CHB (7.8% vs. 1.2%; p=0.0071). No differences in maternal or foetal outcomes were found in comparison with a large cohort of unselected pregnancies except for caesarian section. Hydroxychloroquine (HCQ) was used in 58.3% pregnancies, with a different prevalence according with maternal diagnosis.Our data suggest that anti-SSA/Ro positive patents with a de ned systemic autoimmune disease undergoing a strict follow-up since positive pregnancy test display a low risk of pregnancy complications, including but not limited to NL.

Published
2022

13. Development and implementation of the AIDA International Registry for patients with Behçet's disease

Author

Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ös, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L, Autoinflammatory Diseases Alliance (AIDA) Network, Vitale, Antonio, Della Casa, Francesca, Ragab, Gaafar, Almaghlouth, Ibrahim A, Lopalco, Giuseppe, Pereira, Rosa Maria, Guerriero, Silvana, Govoni, Marcello, Sfikakis, Petros P, Giacomelli, Roberto, Ciccia, Francesco, Monti, Sara, Ruscitti, Piero, Piga, Matteo, Lomater, Claudia, Tufan, Abdurrahman, Opris-Belinski, Daniela, Emmi, Giacomo, Hernández-Rodríguez, José, Şahin, Ali, Sebastiani, Gian Domenico, Bartoloni, Elena, Akkoç, Nurullah, Gündüz, Özgül Soysal, Cattalini, Marco, Conti, Giovanni, Hatemi, Gulen, Maier, Armin, Parronchi, Paola, Del Giudice, Emanuela, Erten, Sukran, Insalaco, Antonella, Li Gobbi, Francesca, Maggio, Maria Cristina, Shahram, Farhad, Caggiano, Valeria, Hegazy, Mohamed Tharwat, Asfina, Kazi Nur, Morrone, Maria, Prado, Leandro L, Dammacco, Rosanna, Ruffilli, Francesca, Arida, Aikaterini, Navarini, Luca, Pantano, Ilenia, Cavagna, Lorenzo, Conforti, Alessandro, Cauli, Alberto, Marucco, Elena Maria, Kucuk, Hamit, Ionescu, Ruxandra, Mattioli, Irene, Espinosa, Gerard, Araújo, Olga, Karkaş, Burak, Canofari, Claudia, Sota, Jurgen, Laymouna, Ahmed Hatem, Bedaiwi, Asma A, Colella, Sergio, Giardini, Henrique Ayres M, Albano, Valeria, Lo Monaco, Andrea, Fragoulis, George E, Kardas, Riza Can, Berlengiero, Virginia, Hussein, Mohamed A, Ricci, Francesca, La Torre, Francesco, Rigante, Donato, Więsik-Szewczyk, Ewa, Frassi, Micol, Gentileschi, Stefano, Tosi, Gian Marco, Dagostin, Marilia Ambiel, Mahmoud, Ayman Abdel-Monem Ahmed, Tarsia, Maria, Alessio, Giovanni, Cimaz, Rolando, Giani, Teresa, Gaggiano, Carla, Iannone, Florenzo, Cipriani, Paola, Mourabi, Mariam, Spedicato, Veronica, Barneschi, Sara, Aragona, Emma, Balistreri, Alberto, Frediani, Bruno, Fabiani, Claudia, and Cantarini, Luca

Subjects
Adult, Registrie, Autoinflammatory disease, Registry, Settore MED/16 - REUMATOLOGIA, precision medicine, behçet’s disease, Settore MED/38 - Pediatria Generale E Specialistica, Retrospective Studie, Internal Medicine, Humans, Prospective Studies, Registries, Child, international registry, Retrospective Studies, Behçet's disease, autoinflammatory diseases, rare diseases, uveitis, Behcet Syndrome, Prospective Studie, Uveiti, Emergency Medicine, Rare disease, Human
Abstract

Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). The Registry is a clinical physician-driven non-population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Starting from January 31st, 2021, to February 7th, 2022, 110 centres from 23 countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5993 fields organised into 16 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.Trial registration NCT05200715 in 21/01/2022.

Published
2022

14. Treatment of Multisystem Inflammatory Syndrome in Children

Author

McArdle A. J., Vito O., Patel H., Seaby E. G., Shah P., Wilson C., Broderick C., Nijman R., Tremoulet A. H., Munblit D., Ulloa-Gutierrez R., Carter M. J., De T., Hoggart C., Whittaker E., Herberg J. A., Kaforou M., Cunnington A. J., Levin M., Vazquez J. A., Carmona R., Perez L., Rubinos M., Veliz N., Yori S., Haerynck F., Hoste L., Leal I. A., Da Silva A. R. A., Silva A. E. A., Barchik A., Barreiro S. T. A., Cochrane N., Teixeira C. H., Arauj J. M., Ossa R. A. P. -D. L., Vieira C. S., Dimitrova A., Ganeva M., Stefanov S., Telcharova-Mihaylovska A., Biggs C. M., Scuccimarri R., Withington D., Raul B. B., Ampuero C., Aravena J., Casanova D., Cruces P., Diaz F., Garcia-Salum T., Godoy L., Medina R. A., Galaz G. V., Avila-Aguero M. L., Brenes-Chacon H., Ivankovich-Escoto G., Yock-Corrales A., Badib A., Badreldin K., Elkhashab Y., Heshmat H., Heinonen S., Angoulvant F., Belot A., Ouldali N., Beske F., Heep A., Masjosthusmann K., Reiter K., Heuvel I. V. D., Both U. V., Agrafiotou A., Antachopoulos C., Eleftheriou I., Farmaki E., Fotis L., Kafetzis D., Lampidi S., Liakopoulou T., Maritsi D., Michailidou E., Milioudi M., Mparmpounaki I., Papadimitriou E., Papaevangelou V., Roilides E., Tsiatsiou O., Tsolas G., Tsolia M., Vantsi P., Pineda L. Y. B., Aguilar K. L. B., Quintero E. M. C., Ip P., Kwan M. Y. W., Kwok J., Lau Y. L., To K., Wong J. S. C., David M., Farkas D., Kalcakosz S., Szekeres K., Zsigmond B., Aslam N., Andreozzi L., Bianco F., Bucciarelli V., Buonsenso D., Cimaz R., D'Argenio P., Dellepiane R. M., Fabi M., Mastrolia M. V., Mauro A., Mazza A., Romani L., Simonini G., Tipo V., Valentini P., Verdoni L., Reel B., Pace D., Torpiano P., Flores M. F., Dominguez M. G., Vargas A. L. G., Hernandez L. L., Figueroa R. P. M., Gaxiola G. P., Valadez J., Klevberg S., Knudsen P. K., Maseide P. H., Carrera J. M., Castano E. G., Timana C. A. D., Leon T. D., Estripeaut D., Levy J., Norero X., Record J., Rojas-Bonilla M., Iramain R., Hernandez R., Huaman G., Munaico M., Peralta C., Seminario D., Yarleque E. H. Z., Gadzinska J., Mandziuk J., Okarska-Napierala M., Alacheva Z. A., Alexeeva E., Ananin P. V., Antsupova M., Bakradze M. D., Bobkova P., Borzakova S., Chashchina I. L., Fisenko A. P., Gautier M. S., Glazyrina A., Kondrikova E., Korobyants E., Korsunskiy A. A., Kovygina K., Krasnaya E., Kurbanova S., Kurdup M. K., Mamutova A. V., Mazankova L., Mitushin I. L., Nargizyan A., Orlova Y. O., Osmanov I. M., Polyakova A. S., Romanova O., Samitova E., Sologub A., Spiridonova E., Tepaev R. F., Tkacheva A. A., Yusupova V., Zholobova E., Grasa C. D., Segura N. L., Martinon-Torres F., Melendo S., Echevarria A. M., Guzman J. M. M., Argueta J. R. P., Rivero-Calle I., Riviere J., Rodriguez-Gonzalez M., Rojo P., Manubens J. S., Soler-Palacin P., Soriano-Arandes A., Tagarro A., Villaverde S., Altman M., Brodin P., Horne A., Palmblad K., Brotschi B., Sauteur P. M., Schmid J. P., Prader S., Relly C., Schlapbach L. J., Seiler M., Truck J., Wutz D., Ketharanathan N., Vermont C., Ozkan E. A., Erdeniz E. H., Borisova G., Boychenko L., Diudenko N., Kasiyan O., Katerynych K., Melnyk K., Miagka N., Teslenko M., Trykosh M., Volokha A., Akomolafe T., Al-Abadi E., Alders N., Avram P., Bamford A., Bank M., Roy R. B., Beattie T., Boleti O., Broad J., Carrol E. D., Chandran A., Cooper H., Davies P., Emonts M., Evans C., Fidler K., Foster C., Gong C., Gongrun B., Gonzalez C., Grandjean L., Grant K., Hacohen Y., Hall J., Hassell J., Hesketh C., Hewlett J., Hnieno A., Holt-Davis H., Hossain A., Hudson L. D., Johnson M., Johnson S., Jyothish D., Kampmann B., Kavirayani A., Kelly D., Kucera F., Langer D., Lillie J., Longbottom K., Lyall H., MacKdermott N., Maltby S., McLelland T., McMahon A. -M., Miller D., Morrison Z., Mosha K., Muller J., Myttaraki E., Nadel S., Osaghae D., Osman F., Ostrzewska A., Panthula M., Papachatzi E., Papadopoulou C., Penner J., Polandi S., Prendergast A. J., Ramnarayan P., Rhys-Evans S., Riordan A., Rodrigues C. M. C., Romaine S., Seddon J., Shingadia D., Srivastava A., Struik S., Taylor A., Tran S., Tudor-Williams G., Van Der Velden F., Ventilacion L., Wellman P. A., Yanney M. P., Yeung S., Badheka A., Badran S., Bailey D. M., Burch A. K., Burns J. C., Cichon C., Cirks B., Dallman M. D., Delany D. R., Fairchok M., Friedman S., Geracht J., Langs-Barlow A., Mann K., Padhye A., Quade A., Ramirez K. A., Rockett J., Sayed I. A., Shahin A. A., Umaru S., Widener R., Angela M. H., Kandawasvika G., McArdle A.J., Vito O., Patel H., Seaby E.G., Shah P., Wilson C., Broderick C., Nijman R., Tremoulet A.H., Munblit D., Ulloa-Gutierrez R., Carter M.J., De T., Hoggart C., Whittaker E., Herberg J.A., Kaforou M., Cunnington A.J., Levin M., Vazquez J.A., Carmona R., Perez L., Rubinos M., Veliz N., Yori S., Haerynck F., Hoste L., Leal I.A., Da Silva A.R.A., Silva A.E.A., Barchik A., Barreiro S.T.A., Cochrane N., Teixeira C.H., Arauj J.M., Ossa R.A.P.-D.L., Vieira C.S., Dimitrova A., Ganeva M., Stefanov S., Telcharova-Mihaylovska A., Biggs C.M., Scuccimarri R., Withington D., Raul B.B., Ampuero C., Aravena J., Casanova D., Cruces P., Diaz F., Garcia-Salum T., Godoy L., Medina R.A., Galaz G.V., Avila-Aguero M.L., Brenes-Chacon H., Ivankovich-Escoto G., Yock-Corrales A., Badib A., Badreldin K., Elkhashab Y., Heshmat H., Heinonen S., Angoulvant F., Belot A., Ouldali N., Beske F., Heep A., Masjosthusmann K., Reiter K., Heuvel I.V.D., Both U.V., Agrafiotou A., Antachopoulos C., Eleftheriou I., Farmaki E., Fotis L., Kafetzis D., Lampidi S., Liakopoulou T., Maritsi D., Michailidou E., Milioudi M., Mparmpounaki I., Papadimitriou E., Papaevangelou V., Roilides E., Tsiatsiou O., Tsolas G., Tsolia M., Vantsi P., Pineda L.Y.B., Aguilar K.L.B., Quintero E.M.C., Ip P., Kwan M.Y.W., Kwok J., Lau Y.L., To K., Wong J.S.C., David M., Farkas D., Kalcakosz S., Szekeres K., Zsigmond B., Aslam N., Andreozzi L., Bianco F., Bucciarelli V., Buonsenso D., Cimaz R., D'Argenio P., Dellepiane R.M., Fabi M., Mastrolia M.V., Mauro A., Mazza A., Romani L., Simonini G., Tipo V., Valentini P., Verdoni L., Reel B., Pace D., Torpiano P., Flores M.F., Dominguez M.G., Vargas A.L.G., Hernandez L.L., Figueroa R.P.M., Gaxiola G.P., Valadez J., Klevberg S., Knudsen P.K., Maseide P.H., Carrera J.M., Castano E.G., Timana C.A.D., Leon T.D., Estripeaut D., Levy J., Norero X., Record J., Rojas-Bonilla M., Iramain R., Hernandez R., Huaman G., Munaico M., Peralta C., Seminario D., Yarleque E.H.Z., Gadzinska J., Mandziuk J., Okarska-Napierala M., Alacheva Z.A., Alexeeva E., Ananin P.V., Antsupova M., Bakradze M.D., Bobkova P., Borzakova S., Chashchina I.L., Fisenko A.P., Gautier M.S., Glazyrina A., Kondrikova E., Korobyants E., Korsunskiy A.A., Kovygina K., Krasnaya E., Kurbanova S., Kurdup M.K., Mamutova A.V., Mazankova L., Mitushin I.L., Nargizyan A., Orlova Y.O., Osmanov I.M., Polyakova A.S., Romanova O., Samitova E., Sologub A., Spiridonova E., Tepaev R.F., Tkacheva A.A., Yusupova V., Zholobova E., Grasa C.D., Segura N.L., Martinon-Torres F., Melendo S., Echevarria A.M., Guzman J.M.M., Argueta J.R.P., Rivero-Calle I., Riviere J., Rodriguez-Gonzalez M., Rojo P., Manubens J.S., Soler-Palacin P., Soriano-Arandes A., Tagarro A., Villaverde S., Altman M., Brodin P., Horne A., Palmblad K., Brotschi B., Sauteur P.M., Schmid J.P., Prader S., Relly C., Schlapbach L.J., Seiler M., Truck J., Wutz D., Ketharanathan N., Vermont C., Ozkan E.A., Erdeniz E.H., Borisova G., Boychenko L., Diudenko N., Kasiyan O., Katerynych K., Melnyk K., Miagka N., Teslenko M., Trykosh M., Volokha A., Akomolafe T., Al-Abadi E., Alders N., Avram P., Bamford A., Bank M., Roy R.B., Beattie T., Boleti O., Broad J., Carrol E.D., Chandran A., Cooper H., Davies P., Emonts M., Evans C., Fidler K., Foster C., Gong C., Gongrun B., Gonzalez C., Grandjean L., Grant K., Hacohen Y., Hall J., Hassell J., Hesketh C., Hewlett J., Hnieno A., Holt-Davis H., Hossain A., Hudson L.D., Johnson M., Johnson S., Jyothish D., Kampmann B., Kavirayani A., Kelly D., Kucera F., Langer D., Lillie J., Longbottom K., Lyall H., MacKdermott N., Maltby S., McLelland T., McMahon A.-M., Miller D., Morrison Z., Mosha K., Muller J., Myttaraki E., Nadel S., Osaghae D., Osman F., Ostrzewska A., Panthula M., Papachatzi E., Papadopoulou C., Penner J., Polandi S., Prendergast A.J., Ramnarayan P., Rhys-Evans S., Riordan A., Rodrigues C.M.C., Romaine S., Seddon J., Shingadia D., Srivastava A., Struik S., Taylor A., Tran S., Tudor-Williams G., Van Der Velden F., Ventilacion L., Wellman P.A., Yanney M.P., Yeung S., Badheka A., Badran S., Bailey D.M., Burch A.K., Burns J.C., Cichon C., Cirks B., Dallman M.D., Delany D.R., Fairchok M., Friedman S., Geracht J., Langs-Barlow A., Mann K., Padhye A., Quade A., Ramirez K.A., Rockett J., Sayed I.A., Shahin A.A., Umaru S., Widener R., Angela M.H., Kandawasvika G., Pediatric Surgery, Pediatrics, University of Zurich, National Institute of Health and Medical Research, Wellcome Trust, Medical Research Foundation, Shah, Priyen [0000-0001-9164-8862], Ulloa-Gutierrez, Rolando [0000-0002-9157-9227], Herberg, Jethro A [0000-0001-6941-6491], Cunnington, Aubrey J [0000-0002-1305-3529], Levin, Michael [0000-0003-2767-6919], and Apollo - University of Cambridge Repository

Subjects
Inotrope, Male, medicine.medical_treatment, 2700 General Medicine, 030204 cardiovascular system & hematology, Antibodies, Viral, Medical and Health Sciences, Cohort Studies, 0302 clinical medicine, Glucocorticoid, hemic and lymphatic diseases, Medicine and Health Sciences, 030212 general & internal medicine, Viral, Child, 11 Medical and Health Sciences, OUTCOMES, Respiration, Immunoglobulins, Intravenous, General Medicine, Systemic Inflammatory Response Syndrome, 3. Good health, Hospitalization, Treatment Outcome, Child, Preschool, Combination, Artificial, Regression Analysis, Drug Therapy, Combination, Female, Original Article, Intravenous, Life Sciences & Biomedicine, Cohort study, Human, medicine.medical_specialty, BATS Consortium, Adolescent, Immunoglobulins, 610 Medicine & health, Regression Analysi, Antibodies, Immunomodulation, 03 medical and health sciences, Medicine, General & Internal, Pharmacotherapy, Drug Therapy, Clinical Research, Internal medicine, General & Internal Medicine, medicine, MANAGEMENT, Confidence Intervals, Humans, Preschool, Propensity Score, Glucocorticoids, Mechanical ventilation, Science & Technology, business.industry, SARS-CoV-2, Inflammatory and immune system, COVID-19, Odds ratio, medicine.disease, Respiration, Artificial, Confidence interval, KAWASAKI-LIKE DISEASE, COVID-19 Drug Treatment, Systemic inflammatory response syndrome, 10036 Medical Clinic, Immunoglobulins, Intravenou, Propensity score matching, Cohort Studie, business, ACUTE RESPIRATORY SYNDROME, Confidence Interval, TOXIC-SHOCK-SYNDROME
Abstract

BackgroundEvidence is urgently needed to support treatment decisions for children with multisystem inflammatory syndrome (MIS-C) associated with severe acute respiratory syndrome coronavirus 2.MethodsWe performed an international observational cohort study of clinical and outcome data regarding suspected MIS-C that had been uploaded by physicians onto a Web-based database. We used inverse-probability weighting and generalized linear models to evaluate intravenous immune globulin (IVIG) as a reference, as compared with IVIG plus glucocorticoids and glucocorticoids alone. There were two primary outcomes: the first was a composite of inotropic support or mechanical ventilation by day 2 or later or death; the second was a reduction in disease severity on an ordinal scale by day 2. Secondary outcomes included treatment escalation and the time until a reduction in organ failure and inflammation.ResultsData were available regarding the course of treatment for 614 children from 32 countries from June 2020 through February 2021; 490 met the World Health Organization criteria for MIS-C. Of the 614 children with suspected MIS-C, 246 received primary treatment with IVIG alone, 208 with IVIG plus glucocorticoids, and 99 with glucocorticoids alone; 22 children received other treatment combinations, including biologic agents, and 39 received no immunomodulatory therapy. Receipt of inotropic or ventilatory support or death occurred in 56 patients who received IVIG plus glucocorticoids (adjusted odds ratio for the comparison with IVIG alone, 0.77; 95% confidence interval [CI], 0.33 to 1.82) and in 17 patients who received glucocorticoids alone (adjusted odds ratio, 0.54; 95% CI, 0.22 to 1.33). The adjusted odds ratios for a reduction in disease severity were similar in the two groups, as compared with IVIG alone (0.90 for IVIG plus glucocorticoids and 0.93 for glucocorticoids alone). The time until a reduction in disease severity was similar in the three groups.ConclusionsWe found no evidence that recovery from MIS-C differed after primary treatment with IVIG alone, IVIG plus glucocorticoids, or glucocorticoids alone, although significant differences may emerge as more data accrue. (Funded by the European Union's Horizon 2020 Program and others; BATS ISRCTN number, ISRCTN69546370.).

Published
2021

19. Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: analysis by the Pharmachild Safety Adjudication Committee

Author

Giancane G1, Swart JF2, Castagnola E3, Groll AH4, Horneff G5, 6, Huppertz HI7, Lovell DJ8, Wolfs T2, Herlin T9, Dolezalova P10, Sanner H11, Susic G13, Sztajnbok F14, Maritsi D15, Constantin T16, Vargova V17, Sawhney S18, Rygg M19, K Oliveira S21, Cattalini M22, Bovis F1, Bagnasco F1, Pistorio A23, Martini A24, Wulffraat N2, Ruperto N25, Paediatric Rheumatology International Trials Organisation (PRINTO). Cuttica R, Garay SM, Brunner J, Emminger W, Appenzeller S, Len C, Saad Magalhaes C, Telcharova-Mihaylovska A, Harjacek M, Jelusic M, Estmann A, Nielsen S, Herrera Mora C, Gervais E, Koné-Paut I, Quartier P, Foeldvari I, Horneff G, Lutz T, Minden K, Tzaribachev N, Trachana M, Tsitsami E, Vougiouka O, Orban I, Harel L, Hashkes P, Uziel Y, Cimaz R, Civino A, Consolini R, D'Angelo G, De Benedetti F, Filocamo G, Fueri E, Gallizzi R, Maggio MC, Magnolia MG, Miniaci A, Montin D, Olivieri A. N., Pastore S, Rigante D, Zulian F, Rumba-Rozenfelde I, Stanevicha V, Panaviene V, Rodriguez Lozano AL, Rubio-Perez N, Vega Cornejo G, Hoppenreijs E, Kamphuis S, Flato B, Nordal EB, Abdwani R, Miraval T, Paz Gastanaga ME, Smolewska E, Ailioaie C, Cochino AV, Laday M, Lazar C, Alexeeva E, Chasnyk V, Keltsev V, Suwairi WMS, Vijatov-Djuric G, Vojinovic J, Arkachaisri T, Koskova E, Avcin T, Ally M, Van Rensburg CJ, Louw I, Lopez JA, Boteanu AL, Calvo Penades I, De Inocencio J, Mesa-Del-Castillo P, Moreno E, Remesal A, Hofer M, Gok F, Ozen S, Ramanan A, Pallotti C, Villa L., Giancane, G1, Swart, Jf2, Castagnola, E3, Groll, Ah4, Horneff, G5, Huppertz, Hi7, Lovell, Dj8, Wolfs, T2, Herlin, T9, Dolezalova, P10, Sanner, H11, Susic, G13, Sztajnbok, F14, Maritsi, D15, Constantin, T16, Vargova, V17, Sawhney, S18, Rygg, M19, K Oliveira, S21, Cattalini, M22, Bovis, F1, Bagnasco, F1, Pistorio, A23, Martini, A24, Wulffraat, N2, Ruperto, N25, Paediatric Rheumatology International Trials Organisation (PRINTO)., Cuttica R, Garay, Sm, Brunner, J, Emminger, W, Appenzeller, S, Len, C, Saad Magalhaes, C, Telcharova-Mihaylovska, A, Harjacek, M, Jelusic, M, Estmann, A, Nielsen, S, Herrera Mora, C, Gervais, E, Koné-Paut, I, Quartier, P, Foeldvari, I, Horneff, G, Lutz, T, Minden, K, Tzaribachev, N, Trachana, M, Tsitsami, E, Vougiouka, O, Orban, I, Harel, L, Hashkes, P, Uziel, Y, Cimaz, R, Civino, A, Consolini, R, D'Angelo, G, De Benedetti, F, Filocamo, G, Fueri, E, Gallizzi, R, Maggio, Mc, Magnolia, Mg, Miniaci, A, Montin, D, Olivieri, A. N., Pastore, S, Rigante, D, Zulian, F, Rumba-Rozenfelde, I, Stanevicha, V, Panaviene, V, Rodriguez Lozano, Al, Rubio-Perez, N, Vega Cornejo, G, Hoppenreijs, E, Kamphuis, S, Flato, B, Nordal, Eb, Abdwani, R, Miraval, T, Paz Gastanaga, Me, Smolewska, E, Ailioaie, C, Cochino, Av, Laday, M, Lazar, C, Alexeeva, E, Chasnyk, V, Keltsev, V, Suwairi, Wm, Vijatov-Djuric, G, Vojinovic, J, Arkachaisri, T, Koskova, E, Avcin, T, Ally, M, Van Rensburg, Cj, Louw, I, Lopez, Ja, Boteanu, Al, Calvo Penades, I, De Inocencio, J, Mesa-Del-Castillo, P, Moreno, E, Remesal, A, Hofer, M, Gok, F, Ozen, S, Ramanan, A, Pallotti, C, Villa, L., Giancane, Gabriella, Swart, Joost F, Castagnola, Elio, Groll, Andreas H, Horneff, Gerd, Huppertz, Hans-Iko, Lovell, Daniel J, Wolfs, Tom, Herlin, Troel, Dolezalova, Pavla, Sanner, Helga, Susic, Gordana, Sztajnbok, Flavio, Maritsi, Despoina, Constantin, Tama, Vargova, Veronika, Sawhney, Sujata, Rygg, Marite, K Oliveira, Sheila, Cattalini, Marco, Bovis, Francesca, Bagnasco, Francesca, Pistorio, Angela, Martini, Alberto, Wulffraat, Nico, Ruperto, Nicolino, Cuttica, Ruben, Garay, Stella Mari, Brunner, Jurgen, Emminger, Wolfgang, Appenzeller, Simone, Len, Claudio, Saad Magalhaes, Claudia, Telcharova-Mihaylovska, Albena, Harjacek, Miroslav, Jelusic, Marija, Estmann, Anne, Nielsen, Susan, Herrera Mora, Cristina, Gervais, Elisabeth, Koné-Paut, Isabelle, Quartier, Pierre, Foeldvari, Ivan, Lutz, Thoma, Minden, Kirsten, Tzaribachev, Nikolay, Trachana, Maria, Tsitsami, Elena, Vougiouka, Olga, Orban, Ilonka, Harel, Liora, Hashkes, Philip, Uziel, Yosef, Cimaz, Rolando, Civino, Adele, Consolini, Rita, D'Angelo, Gianfranco, De Benedetti, Fabrizio, Filocamo, Giovanni, Fueri, Elena, Gallizzi, Romina, Maggio, Maria Cristina, Magnolia, Maria Greca, Miniaci, Angela, Montin, Davide, Olivieri, Alma Nunzia, Pastore, Serena, Rigante, Donato, Zulian, Francesco, Rumba-Rozenfelde, Ingrida, Stanevicha, Valda, Panaviene, Violeta, Rodriguez Lozano, Ana Luisa, Rubio-Perez, Nadina, Vega Cornejo, Gabriel, Hoppenreijs, Esther, Kamphuis, Sylvia, Flato, Berit, Nordal, Ellen Berit, Abdwani, Reem, Miraval, Tatiana, Paz Gastanaga, Maria Eliana, Smolewska, Elzbieta, Ailioaie, Constantin, Cochino, Alexis-Virgil, Laday, Matilda, Lazar, Calin, Alexeeva, Ekaterina, Chasnyk, Vyacheslav, Keltsev, Vladimir, Suwairi, Wafaa Mohammed Saad, Vijatov-Djuric, Gordana, Vojinovic, Jelena, Arkachaisri, Thaschawee, Koskova, Elena, Avcin, Tadej, Ally, Mahmood, Van Rensburg, Christa Janse, Louw, Ingrid, Lopez, Jordi Anton, Boteanu, Alina Lucica, Calvo Penades, Inmaculada, De Inocencio, Jaime, Mesa-Del-Castillo, Pablo, Moreno, Estefania, Remesal, Agustin, Hofer, Michael, Gok, Faysal, Ozen, Seza, Ramanan, Athimalaipet, Pallotti, Chiara, Villa, Luca, and Pediatrics

Subjects
Male, lcsh:Diseases of the musculoskeletal system, Biologic, Paediatrics: 760 [VDP], Artritis infecciosa, MedDRA, Infants malalts, Arthritis, Severity of Illness Index, Hospital patients, Cohort Studies, Pharmacovigilance, 0302 clinical medicine, 030212 general & internal medicine, Registries, Child, Biologics, Immunosuppressive therapy, Infections, Juvenile idiopathic arthritis, Opportunistic, biologics, immunosuppressive therapy, infections, juvenile idiopathic arthritis, opportunistic, Barneleddgikt, 3. Good health, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Antirheumatic Agents, Child, Preschool, Cohort, Pediatric Infectious Disease, Female, Infection, Research Article, medicine.medical_specialty, Tuberculosis, juvenil idiopathic arthritis, Opportunistic Infections, Herpes Zoster, 03 medical and health sciences, Immunocompromised Host, Juvenile idiopathic arthriti, Internal medicine, medicine, Humans, book, 030203 arthritis & rheumatology, Malalts hospitalitzats, Immunosupressió, business.industry, Sick children, medicine.disease, Rheumatology, Arthritis, Juvenile, Infectious arthritis, Pediatri: 760 [VDP], Orthopedic surgery, Opportunistiske infeksjoner, book.journal, lcsh:RC925-935, business, Infeccions oportunistes, Immunosuppression
Abstract

Background: To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC). Methods: The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists. A final evidence-based list of OI resulted by matching the adjudicated infections with the provisional list of OI. Results: A total of 772 infectious events in 572 eligible patients, of which 335 serious/severe/very severe non-OI and 437 OI (any intensity/severity), according to the provisional list, were retrieved. Six hundred eighty-two of 772 (88.3%) were adjudicated as infections, of them 603/682 (88.4%) as common and 119/682 (17.4%) as OI by the SAC. Matching these 119 opportunistic events with the provisional list, 106 were confirmed by the SAC as OI, and among them infections by herpes viruses were the most frequent (68%), followed by tuberculosis (27.4%). The remaining events were divided in the groups of non-OI and possible/patient and/or pathogen-related OI. Conclusions: We found a significant number of OI in JIA patients on immunosuppressive therapy. The proposed list of OI, created by consensus and validated in the Pharmachild cohort, could facilitate comparison among future pharmacovigilance studies. © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Published
2020

20. Subcutaneous Abatacept in Patients With Polyarticular-Course Juvenile Idiopathic Arthritis

Author

Brunner H. I., Tzaribachev N., Vega-Cornejo G., Louw I., Berman A., Calvo Penades I., Anton J., Avila-Zapata F., Cuttica R., Horneff G., Foeldvari I., Keltsev V., Kingsbury D. J., Viola D. O., Joos R., Lauwerys B., Paz Gastanaga M. E., Rama M. E., Wouters C., Bohnsack J., Breedt J., Fischbach M., Lutz T., Minden K., Miraval T., Ally M. M. T. M., Rubio-Perez N., Solau Gervais E., van Zyl R., Li X., Nys M., Wong R., Banerjee S., Lovell D. J., Martini A., Ruperto N., Becker M. L., Ilowite N. T., Dare J. A., Morris P. K., Beukelman T. G., Wagner-Weiner L., Zemel L., Quartier P., Kone-Paut I., Belot A., Gerloni V., Ferrandiz M., Van Rensburg D. J., Scheibel I. M., Goldstein-Schainberg C., Silva C., Terreri M. T. S. E. L. A., Gamir M., Burgos Vargas R., Faugier Fuentes E., Cimaz R., Alessio M., Espada G., Brunner, H. I., Tzaribachev, N., Vega-Cornejo, G., Louw, I., Berman, A., Calvo Penades, I., Anton, J., Avila-Zapata, F., Cuttica, R., Horneff, G., Foeldvari, I., Keltsev, V., Kingsbury, D. J., Viola, D. O., Joos, R., Lauwerys, B., Paz Gastanaga, M. E., Rama, M. E., Wouters, C., Bohnsack, J., Breedt, J., Fischbach, M., Lutz, T., Minden, K., Miraval, T., Ally, M. M. T. M., Rubio-Perez, N., Solau Gervais, E., van Zyl, R., Li, X., Nys, M., Wong, R., Banerjee, S., Lovell, D. J., Martini, A., Ruperto, N., Becker, M. L., Ilowite, N. T., Dare, J. A., Morris, P. K., Beukelman, T. G., Wagner-Weiner, L., Zemel, L., Quartier, P., Kone-Paut, I., Belot, A., Gerloni, V., Ferrandiz, M., Van Rensburg, D. J., Scheibel, I. M., Goldstein-Schainberg, C., Silva, C., Terreri, M. T. S. E. L. A., Gamir, M., Burgos Vargas, R., Faugier Fuentes, E., Cimaz, R., Alessio, M., and Espada, G.

Subjects
Male, musculoskeletal diseases, 0301 basic medicine, medicine.medical_specialty, Adolescent, genetic structures, Immunology, Juvenile, Arthritis, Injections, Subcutaneou, Abatacept, Arthritis, Juvenile, Child, Child, Preschool, Cohort Studies, Female, Humans, Immunosuppressive Agents, Injections, Subcutaneous, Treatment Outcome, Injections, Immunosuppressive Agent, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Pharmacokinetics, Open label study, medicine, Immunology and Allergy, In patient, Preschool, skin and connective tissue diseases, 030203 arthritis & rheumatology, business.industry, Subcutaneous, medicine.disease, Dermatology, Clinical trial, 030104 developmental biology, Methotrexate, Cohort Studie, business, Human, medicine.drug
Abstract

Objective: To investigate the pharmacokinetics, effectiveness, and safety of subcutaneous (SC) abatacept treatment over 24 months in patients with polyarticular-course juvenile idiopathic arthritis (JIA). Methods: In this phase III, open-label, international, multicenter, single-arm study, patients with polyarticular JIA (cohort 1, ages 6–17 years and cohort 2, ages 2–5 years) in whom treatment with ≥1 disease-modifying antirheumatic drug was unsuccessful received weight-tiered SC abatacept weekly: 10 to

Published
2018

22. O.51 - Abatacept sous-cutané chez des patients présentant une arthrite juvénile idiopathique polyarticulaire et une réponse inadéquate aux DMARDs conventionnels ou biologiques : pharmacocinétique, efficacité et sécurité

Author

Solau-Gervais, E., Lovell, D., Ruperto, N., Tzaribachev, N., Vega-Cornejo, G., Louw, I., Berman, A., Calvo, I., Cuttica, R., Horneff, G., Avila-Zapata, F., Anton, J., Cimaz, R., Joos, R., Espada, G., Li, X., Nys, M., Wong, R., Banerjee, S., Brunner, H., and Martini, A.

Published
2016
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23. Treatment of Multisystem Inflammatory Syndrome in Children

Author

McArdle, A.J. Vito, O. Patel, H. Seaby, E.G. Shah, P. Wilson, C. Broderick, C. Nijman, R. Tremoulet, A.H. Munblit, D. Ulloa-Gutierrez, R. Carter, M.J. De, T. Hoggart, C. Whittaker, E. Herberg, J.A. Kaforou, M. Cunnington, A.J. Levin, M. Vazquez, J.A. Carmona, R. Perez, L. Rubinos, M. Veliz, N. Yori, S. Haerynck, F. Hoste, L. Leal, I.A. Da Silva, A.R.A. Silva, A.E.A. Barchik, A. Barreiro, S.T.A. Cochrane, N. Teixeira, C.H. Arauj, J.M. Ossa, R.A.P.-D.L. Vieira, C.S. Dimitrova, A. Ganeva, M. Stefanov, S. Telcharova-Mihaylovska, A. Biggs, C.M. Scuccimarri, R. Withington, D. Raul, B.B. Ampuero, C. Aravena, J. Casanova, D. Cruces, P. Diaz, F. Garcia-Salum, T. Godoy, L. Medina, R.A. Galaz, G.V. Avila-Aguero, M.L. Brenes-Chacon, H. Ivankovich-Escoto, G. Yock-Corrales, A. Badib, A. Badreldin, K. Elkhashab, Y. Heshmat, H. Heinonen, S. Angoulvant, F. Belot, A. Ouldali, N. Beske, F. Heep, A. Masjosthusmann, K. Reiter, K. Heuvel, I.V.D. Both, U.V. Agrafiotou, A. Antachopoulos, C. Eleftheriou, I. Farmaki, E. Fotis, L. Kafetzis, D. Lampidi, S. Liakopoulou, T. Maritsi, D. Michailidou, E. Milioudi, M. Mparmpounaki, I. Papadimitriou, E. Papaevangelou, V. Roilides, E. Tsiatsiou, O. Tsolas, G. Tsolia, M. Vantsi, P. Pineda, L.Y.B. Aguilar, K.L.B. Quintero, E.M.C. Ip, P. Kwan, M.Y.W. Kwok, J. Lau, Y.L. To, K. Wong, J.S.C. David, M. Farkas, D. Kalcakosz, S. Szekeres, K. Zsigmond, B. Aslam, N. Andreozzi, L. Bianco, F. Bucciarelli, V. Buonsenso, D. Cimaz, R. D'Argenio, P. Dellepiane, R.M. Fabi, M. Mastrolia, M.V. Mauro, A. Mazza, A. Romani, L. Simonini, G. Tipo, V. Valentini, P. Verdoni, L. Reel, B. Pace, D. Torpiano, P. Flores, M.F. Domínguez, M.G. Vargas, A.L.G. Hernandez, L.L. Figueroa, R.P.M. Gaxiola, G.P. Valadez, J. Klevberg, S. Knudsen, P.K. Maseide, P.H. Carrera, J.M. Castano, E.G. Timana, C.A.D. Leon, T.D. Estripeaut, D. Levy, J. Norero, X. Record, J. Rojas-Bonilla, M. Iramain, R. Hernandez, R. Huaman, G. Munaico, M. Peralta, C. Seminario, D. Yarleque, E.H.Z. Gadzinska, J. Mandziuk, J. Okarska-Napierała, M. Alacheva, Z.A. Alexeeva, E. Ananin, P.V. Antsupova, M. Bakradze, M.D. Bobkova, P. Borzakova, S. Chashchina, I.L. Fisenko, A.P. Gautier, M.S. Glazyrina, A. Kondrikova, E. Korobyants, E. Korsunskiy, A.A. Kovygina, K. Krasnaya, E. Kurbanova, S. Kurdup, M.K. Mamutova, A.V. Mazankova, L. Mitushin, I.L. Nargizyan, A. Orlova, Y.O. Osmanov, I.M. Polyakova, A.S. Romanova, O. Samitova, E. Sologub, A. Spiridonova, E. Tepaev, R.F. Tkacheva, A.A. Yusupova, V. Zholobova, E. Grasa, C.D. Segura, N.L. Martinon-Torres, F. Melendo, S. Echevarria, A.M. Guzman, J.M.M. Argueta, J.R.P. Rivero-Calle, I. Riviere, J. Rodriguez-Gonzalez, M. Rojo, P. Manubens, J.S. Soler-Palacin, P. Soriano-Arandes, A. Tagarro, A. Villaverde, S. Altman, M. Brodin, P. Horne, A. Palmblad, K. Brotschi, B. Sauteur, P.M. Schmid, J.P. Prader, S. Relly, C. Schlapbach, L.J. Seiler, M. Truck, J. Wutz, D. Ketharanathan, N. Vermont, C. Ozkan, E.A. Erdeniz, E.H. Borisova, G. Boychenko, L. Diudenko, N. Kasiyan, O. Katerynych, K. Melnyk, K. Miagka, N. Teslenko, M. Trykosh, M. Volokha, A. Akomolafe, T. Al-Abadi, E. Alders, N. Avram, P. Bamford, A. Bank, M. Roy, R.B. Beattie, T. Boleti, O. Broad, J. Carrol, E.D. Chandran, A. Cooper, H. Davies, P. Emonts, M. Evans, C. Fidler, K. Foster, C. Gong, C. Gongrun, B. Gonzalez, C. Grandjean, L. Grant, K. Hacohen, Y. Hall, J. Hassell, J. Hesketh, C. Hewlett, J. Hnieno, A. Holt-Davis, H. Hossain, A. Hudson, L.D. Johnson, M. Johnson, S. Jyothish, D. Kampmann, B. Kavirayani, A. Kelly, D. Kucera, F. Langer, D. Lillie, J. Longbottom, K. Lyall, H. MacKdermott, N. Maltby, S. McLelland, T. McMahon, A.-M. Miller, D. Morrison, Z. Mosha, K. Muller, J. Myttaraki, E. Nadel, S. Osaghae, D. Osman, F. Ostrzewska, A. Panthula, M. Papachatzi, E. Papadopoulou, C. Penner, J. Polandi, S. Prendergast, A.J. Ramnarayan, P. Rhys-Evans, S. Riordan, A. Rodrigues, C.M.C. Romaine, S. Seddon, J. Shingadia, D. Srivastava, A. Struik, S. Taylor, A. Taylor, A. Taylor, A. Tran, S. Tudor-Williams, G. Van Der Velden, F. Ventilacion, L. Wellman, P.A. Yanney, M.P. Yeung, S. Badheka, A. Badran, S. Bailey, D.M. Burch, A.K. Burns, J.C. Cichon, C. Cirks, B. Dallman, M.D. Delany, D.R. Fairchok, M. Friedman, S. Geracht, J. Langs-Barlow, A. Mann, K. Padhye, A. Quade, A. Ramirez, K.A. Rockett, J. Sayed, I.A. Shahin, A.A. Umaru, S. Widener, R. Angela, M.H. Kandawasvika, G. BATS Consortium

Subjects
hemic and lymphatic diseases
Abstract

BACKGROUND Evidence is urgently needed to support treatment decisions for children with multisystem inflammatory syndrome (MIS-C) associated with severe acute respiratory syndrome coronavirus 2. METHODS We performed an international observational cohort study of clinical and outcome data regarding suspected MIS-C that had been uploaded by physicians onto a Web-based database. We used inverse-probability weighting and generalized linear models to evaluate intravenous immune globulin (IVIG) as a reference, as compared with IVIG plus glucocorticoids and glucocorticoids alone. There were two primary outcomes: the first was a composite of inotropic support or mechanical ventilation by day 2 or later or death; the second was a reduction in disease severity on an ordinal scale by day 2. Secondary outcomes included treatment escalation and the time until a reduction in organ failure and inflammation. RESULTS Data were available regarding the course of treatment for 614 children from 32 countries from June 2020 through February 2021; 490 met the World Health Organization criteria for MIS-C. Of the 614 children with suspected MIS-C, 246 received primary treatment with IVIG alone, 208 with IVIG plus glucocorticoids, and 99 with glucocorticoids alone; 22 children received other treatment combinations, including biologic agents, and 39 received no immunomodulatory therapy. Receipt of inotropic or ventilatory support or death occurred in 56 patients who received IVIG plus glucocorticoids (adjusted odds ratio for the comparison with IVIG alone, 0.77; 95% confidence interval [CI], 0.33 to 1.82) and in 17 patients who received glucocorticoids alone (adjusted odds ratio, 0.54; 95% CI, 0.22 to 1.33). The adjusted odds ratios for a reduction in disease severity were similar in the two groups, as compared with IVIG alone (0.90 for IVIG plus glucocorticoids and 0.93 for glucocorticoids alone). The time until a reduction in disease severity was similar in the three groups. CONCLUSIONS We found no evidence that recovery from MIS-C differed after primary treatment with IVIG alone, IVIG plus glucocorticoids, or glucocorticoids alone, although significant differences may emerge as more data accrue. Copyright © 2021 Massachusetts Medical Society.

Published
2021

24. Patients with Juvenile Systemic Sclerosis Have a Distinct Pattern of Organ Involvement: Results from the Juvenile Systemic Sclerosis Inception Cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, M, Sakamoto, AP, Sztajnbok, F, Feldman, B, Stanevicha, V, Anton-Lopez J, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, MM, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Marrani, E, Schonenberg-Meinema, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Battagliotti, C, Berntson, L, Bica, BERG, Brunner, J, Cimaz, R, Reis, PC, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Hetlevik, SO, Uziel, Y, and Helmus, N

Published
2021

25. Development of a New International Antiphospholipid Syndrome Classification Criteria Phase I/II Report: Generation and Reduction of Candidate Criteria

Author

Barbhaiya, M., Zuily, S., Ahmadzadeh, Y., Amigo, M. -C., Avcin, T., Bertolaccini, M., Branch, D. W., de Jesus, G., Devreese, K. M. J., Frances, C., Garcia, D., Guillemin, F., Levine, S. R., Levy, R. A., Lockshin, M. D., Ortel, T., Seshan, S. V., Tektonidou, M., Wahl, D., Willis, R., Naden, R., Costenbader, K., Erkan, D., Agmon-Levin, N., Aguilar, C., Alba, P., Alpan, O., Ambrozic, A., Amoura, Z., Andrade, D., Andrade, L., Appenzeller, S., Esen, B. A., Atsumi, T., Berkun, Y., Cabral, A., Canaud, G., Cervera, R., Chen, P., Chighizola, C., Cimaz, R., Cohen, H., Costedoat-Chalumeau, N., Crowther, M., Cuadrado, M. J., de Groot, P. G., de Moerloose, P., Derksen, R., Diz-Kucukkaya, R., Dorner, T., Fortin, P., Giannakopoulos, B., Gomez-Puerta, J. A., Gonzalez, E. B., Inanc, M., Kenet, G., Khamashta, M., Kriegel, M., Krilis, S., Laskin, C., Massicotte, P., Mccarty, G., Meroni, P. L., Mikdashi, J., Myones, B., Pengo, V., Petri, M., Roubey, R., Sammaritano, L., Sanna, G., Sciascia, S., Signorelli, F., Soybilgic, A., Tincani, A., Woller, S., and Yelnik, C.

Subjects
Antiphospholipid Syndrome, Consensus, Delphi Technique, Humans, Predictive Value of Tests, Rheumatology, Severity of Illness Index, CONSENSUS STATEMENT, Potential candidate, AMERICAN-COLLEGE, Article, DISEASE, Reduction (complexity), 03 medical and health sciences, 0302 clinical medicine, Antiphospholipid syndrome, RHEUMATOLOGY/EUROPEAN LEAGUE, Nominal group technique, Medicine and Health Sciences, Hierarchical organization, Medicine, CLINICAL-SIGNIFICANCE, computer.programming_language, 030203 arthritis & rheumatology, RISK, VENOUS THROMBOEMBOLISM, Information retrieval, business.industry, SYSTEMIC-SCLEROSIS, medicine.disease, Phase i ii, MYOCARDIAL-INFARCTION, ANTIBODIES, Report generation, business, computer, Delphi
Abstract

Objective : An international multidisciplinary initiative, jointly supported by the American College of Rheumatology and European Alliance of Associations for Rheumatology, is underway to develop new rigorous classification criteria to identify patients with high likelihood of antiphospholipid syndrome (APS) for research purposes. The present study was undertaken to apply an evidence- and consensus-based approach to identify candidate criteria and develop a hierarchical organization of criteria within domains. Methods : During phase I, the APS classification criteria steering committee used systematic literature reviews and surveys of international APS physician scientists to generate a comprehensive list of items related to APS. In phase II, we reviewed the literature, administered surveys, formed domain subcommittees, and used Delphi exercises and nominal group technique to reduce potential APS candidate criteria. Candidate criteria were hierarchically organized into clinical and laboratory domains. Results : Phase I generated 152 candidate criteria, expanded to 261 items with the addition of subgroups and candidate criteria with potential negative weights. Using iterative item reduction techniques in phase II, we initially reduced these items to 64 potential candidate criteria organized into 10 clinical and laboratory domains. Subsequent item reduction methods resulted in 27 candidate criteria, hierarchically organized into 6 additive domains (laboratory, macrovascular, microvascular, obstetric, cardiac, and hematologic) for APS classification. Conclusion : Using data- and consensus-driven methodology, we identified 27 APS candidate criteria in 6 clinical or laboratory domains. In the next phase, the proposed candidate criteria will be used for real-world case collection and further refined, organized, and weighted to determine an aggregate score and threshold for APS classification.

Published
2021

26. Male Juvenile Systemic Sclerosis Patients Have More Severe Disease: Results from the International Juvenile Scleroderma Inception Cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, M, Sakamoto, AP, Sztajnbok, F, Feldman, B, Stanevicha, V, Anton-Lopez J, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, MM, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Marrani, E, Schonenberg-Meinema, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Battagliotti, C, Berntson, L, Bica, BERG, Brunner, J, Cimaz, R, Reis, PC, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Hetlevik, SO, Uziel, Y, and Helmus, N

Published
2021

27. The phenotype of TNF receptor-associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the Eurofever/EUROTRAPS international registry

Author

Lachmann, H J, Papa, R, Gerhold, K, Obici, L, Touitou, I, Cantarini, L, Frenkel, J, Anton, J, Kone-Paut, I, Cattalini, M, Bader-Meunier, B, Insalaco, A, Hentgen, V, Merino, R, Modesto, C, Toplak, N, Berendes, R, Ozen, S, Cimaz, R, Jansson, A, Brogan, P A, Hawkins, P N, Ruperto, N, Martini, A, Woo, P, and Gattorno, M

Published
2014
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29. POS1304 JUVENILE SYSTEMIC SCLEROSIS (JSSC) PATIENTS WITH OVERLAP CHARACTERISTICS DO NOT HAVE MILD DISEASE. RESULTS FROM THE JSSC INCEPTION COHORT. WWW.JUVENILESCLERODERMA.COM

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Stanevicha, V., additional, Anton, J., additional, Sztajnbok, F. R., additional, Khubchandani, R., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Marrani, E., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Costa Reis, P., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Johnson, S., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2021
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33. POS0751 COMORBIDITY AND LONG-TERM OUTCOME IN PATIENTS WITH CONGENITAL HEART BLOCK: PRELIMINARY DATA OF THE ITALIAN REGISTRY ON THE IMMUNE-MEDIATED CONGENITAL HEART BLOCK

Author

Fredi, M., primary, Rizzo, G., additional, Andreoli, L., additional, Bacco, B., additional, Bertero, T., additional, Bortoluzzi, A., additional, Ceccarelli, F., additional, Cimaz, R., additional, Conigliaro, P., additional, Corradi, F., additional, De Vita, S., additional, DI Poi, E., additional, Elefante, E., additional, Emmi, G., additional, Gerosa, M., additional, Govoni, M., additional, Hoxha, A., additional, Lojacono, A., additional, Marrani, E., additional, Marozio, L., additional, Mathieu, A., additional, Mosca, M., additional, Melissa, P., additional, Picchi, C., additional, Piga, M., additional, Priori, R., additional, Ramoni, V., additional, Ruffatti, A., additional, Simonini, G., additional, Tani, C., additional, Tonello, M., additional, Trespidi, L., additional, Urban, M. L., additional, Vezzoli, M., additional, Zatti, S., additional, Calza, S., additional, Brucato, A., additional, Franceschini, F., additional, and Tincani, A., additional

Published
2021
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36. OP0093 RETENTION RATE OF IL-1 INHIBITORS IN PATIENTS WITH SCHNITZLER’S SYNDROME

Author

Crisafulli, F., primary, Vitale, A., additional, Gaggiano, C., additional, Dagna, L., additional, Cavalli, G., additional, Cimaz, R., additional, Viapiana, O., additional, Iannone, F., additional, Lopalco, G., additional, Bortolotti, R., additional, Abdel Jaber, M., additional, Montecucco, C., additional, Monti, S., additional, Balduzzi, S., additional, Emmi, G., additional, Airò, P., additional, Franceschini, F., additional, Cantarini, L., additional, and Frassi, M., additional

Published
2021
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38. POS0079 PATIENTS WITH JUVENILE SYSTEMIC SCLEROSIS HAVE A DISTINCT PATTERN OF ORGAN INVOLVEMENT.RESULTS FROM THE JUVENILE SYSTEMIC SCLEROSIS INCEPTION COHORT. WWW.JUVENILE-SCLERODERMA.COM

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Stanevicha, V., additional, Anton, J., additional, Sztajnbok, F. R., additional, Khubchandani, R., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Marrani, E., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Costa Reis, P., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Johnson, S., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2021
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40. Relapse risk factors in anti-N-methyl-D-aspartate receptor encephalitis

Author

Nosadini, Margherita, Granata, Tiziana, Matricardi, Sara, Freri, Elena, Ragona, Francesca, Papetti, Laura, Suppiej, Agnese, Valeriani, Massimiliano, Sartori, Stefano, Italian Working Group on Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis, Bonuccelli, A, Beccaria, F, Buechner, S, Buratti, S, Cantalupo, G, Cappellari, A, Casellato, S, Cesaroni, E, Cimaz, R, Cordelli, Dm, Costa, P, Dell'Avvento, S, Dilena, R, Falsaperla, R, Foiadelli, T, Frigo, Ac, Fusco, L, Giacobbe, A, Giannotta, M, Grazian, L, Maggio, Mc, Mancardi, Mm, Melis, M, Natali Sora MG, Orsini, A, Petruzzellis, A, Pini, A, Pruna, D, Santangelo, G, Savasta, S, Scaduto, Mc, Serino, D, Simula, D, Solazzi, R, Sotgiu, S, Splendiani, A, Toldo, I, Vigevano, F, Viri, M, Visconti, P, Zamponi, N, Zanus, C, Zoccarato, M, Zuliani, L, Nosadini M., Granata T., Matricardi S., Freri E., Ragona F., Papetti L., Suppiej A., Valeriani M., Sartori S., Bonuccelli A., Beccaria F., Buechner S., Buratti S., Cantalupo G., Cappellari A., Casellato S., Cesaroni E., Cimaz R., Cordelli D.M., Costa P., Dell'Avvento S., Dilena R., Falsaperla R., Foiadelli T., Frigo A.C., Fusco L., Giacobbe A., Giannotta M., Grazian L., Maggio M.C., Mancardi M.M., Melis M., Natali Sora M.G., Orsini A., Petruzzellis A., Pini A., Pruna D., Santangelo G., Savasta S., Scaduto M.C., Serino D., Simula D., Solazzi R., Sotgiu S., Splendiani A., Toldo I., Vigevano F., Viri M., Visconti P., Zamponi N., Zanus C., Zoccarato M., and Zuliani L.

Subjects
Male, 030506 rehabilitation, Gastroenterology, Cohort Studies, 0302 clinical medicine, Retrospective Studie, Modified Rankin Scale, Recurrence, Risk Factors, Child, relapse, Anti-N-Methyl-D-Aspartate Receptor Encephalitis, Hazard ratio, Italy, Child, Preschool, Cohort, anti‐N‐methyl‐D‐aspartate receptor encephalitis, Female, 0305 other medical science, Encephalitis, Human, Cohort study, medicine.medical_specialty, Adolescent, Socio-culturale, anti-NMDAR antibodies, 03 medical and health sciences, anti-NMDAR, Developmental Neuroscience, Internal medicine, medicine, Humans, Infant, Retrospective Studies, Preschool, Survival analysis, Autoimmune encephalitis, business.industry, Retrospective cohort study, medicine.disease, anti-NMDAR antibodies, autoimmune encephalitis, anti‐N‐methyl‐D‐aspartate receptor encephalitis, autoimmune encephalitis, Anti-N-methyl-D-aspartate receptor encephalitis, anti-NMDAR, autoimmune encephalitis, relapse, Anti-N-Methyl-D-Aspartate Receptor Encephaliti, Pediatrics, Perinatology and Child Health, Neurology (clinical), Cohort Studie, business, 030217 neurology & neurosurgery
Abstract

Aim: To identify factors that may predict and affect the risk of relapse in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Method: This was a retrospective study of an Italian cohort of patients with paediatric (≤18y) onset anti-NMDAR encephalitis. Results: Of the 62 children included (39 females; median age at onset 9y 10mo, range 1y 2mo–18y; onset between 2005 and 2018), 21 per cent relapsed (median two total events per relapsing patient, range 2–4). Time to first relapse was median 31.5months (range 7–89mo). Severity at first relapse was lower than onset (median modified Rankin Scale [mRS] 3, range 2–4, vs median mRS 5, range 3–5; admission to intensive care unit: 0/10 vs 3/10). At the survival analysis, the risk of relapsing was significantly lower in patients who received three or more different immune therapies at first disease event (hazard ratio 0.208, 95% confidence interval 0.046–0.941; p=0.042). Neurological outcome at follow-up did not differ significantly between patients with relapsing and monophasic disease (mRS 0–1 in 39/49 vs 12/13; p=0.431), although follow-up duration was significantly longer in relapsing (median 84mo, range 14–137mo) than in monophasic patients (median 32mo, range 4–108mo; p=0.002). Interpretation: Relapses may occur in about one-fifth of children with anti-NMDAR encephalitis, are generally milder than at onset, and may span over a long period, although they do not seem to be associated with severity in the acute phase or with outcome at follow-up. Aggressive immune therapy at onset may reduce risk of relapse. What this paper adds: Relapses of anti-N-methyl-D-aspartate receptor encephalitis may span over a long period. Relapses were not associated with severity in the acute phase or outcome at follow-up. Aggressive immune therapy at onset appears to decrease risk of relapse.

Published
2019

41. Maintenance of antibody response to diphtheria/tetanus vaccine in patients aged 2-5 years with polyarticular-course juvenile idiopathic arthritis receiving subcutaneous abatacept

Author

Brunner HI, Tzaribachev N, Cornejo GV, Joos R, Gervais E, Cimaz R, Calvo Penadés I, Cuttica R, Lutz T, Quartier P, Gandhi Y, Nys M, Wong R, Martini A, Lovell DJ, Ruperto N, and Pediatric Rheumatology Collaborative Study Group and the Paediatric Rheumatology

Subjects
Abatacept, Biologic DMARDs, Juvenile idiopathic arthritis, Vaccination
Abstract

Patients with polyarticular-course juvenile idiopathic arthritis (pJIA), receiving disease-modifying anti-rheumatic drugs with immunosuppressive effects, may be at increased risk of vaccine-preventable infections. This substudy assessed protective antibody responses to diphtheria and tetanus vaccination given prior to study enrolment in patients with pJIA.

Published
2020

42. Prevalence of cranial involvement in a cohort of Italian patients with chronic non-bacterial osteomyelitis

Author

Ferrara, G., Insalaco, A., Pardeo, M., Cattalini, M., La Torre, F., Finetti, M., Ricci, F., Alizzi, C., Teruzzi, B., Simonini, G., Messia, V., Pastore, S., Morra, L., Cimaz, R., Marco Gattorno, Taddio, A., Ferrara, Giovanna, Insalaco, Antonella, Pardeo, Manuela, Cattalini, Marco, La Torre, Francesco, Finetti, Martina, Ricci, Francesca, Alizzi, Clotilde, Teruzzi, Barbara, Simonini, Gabriele, Messia, Virginia, Pastore, Serena, Morra, Laura, Cimaz, Rolando, Gattorno, Marco, and Taddio, Andrea

Subjects
Male, Chronic Non-Bacterial Osteomyeliti, Brain, Cephalometry, Child, Chronic Disease, Cohort Studies, Female, Humans, Italy, Prevalence, Retrospective Studies, Skull, Osteomyelitis, Chronic Non-Bacterial Osteomyelitis, children, Immunology, Rheumatology, Immunology and Allergy
Abstract

Chronic non-bacterial osteomyelitis (CNO) is a non-infectious inflammatory disease characterised by uni- or multi-focal bone lytic lesions. CNO mainly affects metaphysis of long bones, pelvis and shoulder girdle. Neurocranium lesions are extremely rare. The objective of the study is to describe the prevalence and clinical manifestations of CNO patients with neurocranium involvement in an Italian cohort of CNO patients.This is a retrospective study. Medical records of patients with CNO admitted to eight paediatric rheumatology centres were reviewed.Among 86 patients with CNO enrolled in the study, three of them were female and presented neurocranium involvement - multifocal lesions. Two out of the 3 patients were completely asymptomatic for cranial involvement, while one of the 3 complained of cranial bossing. Cranial involvement was detected with bone scintigraphy and then confirmed by magnetic resonance imaging and/or computed tomography. Two patients presented fever and two with skin manifestations. Laboratory inflammatory markers were increased in two of them. All patients underwent bone biopsy confirming the diagnosis. They all received NSAIDs. Two patients received corticosteroids and then methotrexate and achieved clinical remission, while one patient received pamidronate.This is the first report of neurocranium involvement in a cohort of patients affected by CNO. In our cohort no patient showed significant signs attributable to cranial involvement.

Published
2020

43. Is there a difference in presentation of female and male patients with juvenile systemic scleroderma?

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Stanevicha, V, Sztajnbok, F, Sakamoto, AP, Alexeeva, E, Anton, J, Katsicas, M, Smith, V, Avcin, T, Cimaz, R, Kostik, M, Lehman, T, Sifuentes-Giraldo, WA, Appenzeller, S, Janarthanan, M, Moll, M, Nemcova, D, Jose Santos, M, Nassar, A, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Costa Reis, P, Eleftheriou, D, Harel, L, Horneff, G, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Patwardhan, A, Uziel, Y, and Helmus, N

Subjects
ddc: 610, integumentary system, 610 Medical sciences, Medicine
Abstract

Background: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. There are limited data published regarding the differences in clinical presentation of male and female patients with jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is[for full text, please go to the a.m. URL], 47. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 33. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 29. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Published
2019
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44. Update from the juvenile scleroderma Inception Cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Stanevicha, V, Sztajnbok, F, Terreri, MT, Alexeeva, E, Anton, J, Katsicas, M, Smith, V, Avcin, T, Cimaz, R, Kostik, M, Lehman, T, Sifuentes-Giraldo, WA, Appenzeller, S, Janarthanan, M, Moll, M, Nemcova, D, Jose Santos, M, Schonenberg, D, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Costa Reis, P, Eleftheriou, D, Harel, L, Horneff, G, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Patwardhan, A, Uziel, Y, and Helmus, N

Subjects
ddc: 610, integumentary system, 610 Medical sciences, Medicine
Abstract

Background: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. There are limited data regarding the clinical presentation of jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is a multinational registry that prospectively collects information[for full text, please go to the a.m. URL], 47. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 33. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 29. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Published
2019
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50. International cohort study for pediatric Behçet’s Disease: update 2011

Author

Faye A, Cantarini L, Khalil S, Benamour S, Kümmerle-Deschner J, Tutkum I, Anton J, Ozen S, Hofer M, Bouayed K, Chkirate B, Cimaz R, Gattorno M, Shahram F, Bello M, Koné-Paut I, Letierce A, Tran TA, Davatchi F, Kasapcopur O, Al Mayouf A, Pajot C, Nielsen S, Lepore L, and Bono W

Subjects
Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Published
2011
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