학술논문
The Systemic Score May Identify Life-Threatening Evolution in Still Disease: Data from the GIRRCS AOSD-Study Group and the AIDA Network Still Disease Registry.
Document Type
Academic Journal
Author
Ruscitti P; Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.; Masedu F; Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.; Vitale A; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Italy.; Caggiano V; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Italy.; Di Cola I; Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.; Cipriani P; Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.; Valenti M; Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.; Mayrink Giardini HA; Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.; de Brito Antonelli IP; Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.; Dagostin MA; Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.; Lopalco G; Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.; Iannone F; Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.; Maria M; Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.; Almaghlouth IA; Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.; College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.; Asfina KN; College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.; Ali HH; College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.; Ciccia F; Department of Precision Medicine, University of Campania 'Luigi Vanvitelli', Naples, Italy.; Iacono D; Department of Precision Medicine, University of Campania 'Luigi Vanvitelli', Naples, Italy.; Pantano I; Department of Precision Medicine, University of Campania 'Luigi Vanvitelli', Naples, Italy.; Mauro D; Department of Precision Medicine, University of Campania 'Luigi Vanvitelli', Naples, Italy.; Sfikakis PP; Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.; Tektonidou M; Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.; Laskari K; Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.; Berardicurti O; Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Via Álvaro del Portillo 200, Rome, Italy.; Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome 'Campus Biomedico' School of Medicine, Rome, Italy.; Dagna L; Division of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy.; Tomelleri A; Division of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy.; Tufan A; Department of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Turkey.; Can Kardas R; Department of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Turkey.; Hinojosa-Azaola A; Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.; Martín-Nares E; Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.; Kawakami-Campos PA; Department of Ophthalmology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán Mexico City, Mexico.; Ragab G; Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Egypt.; Faculty of Medicine, Newgiza University (NGU), Egypt.; Hegazy MT; Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Egypt.; Faculty of Medicine, Newgiza University (NGU), Egypt.; Direskeneli H; Department of Internal Medicine, Division of Rheumatology, Marmara University, Faculty of Medicine, Istanbul, Turkey.; Alibaz-Oner F; Department of Internal Medicine, Division of Rheumatology, Marmara University, Faculty of Medicine, Istanbul, Turkey.; Fotis L; Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Greece.; Sfriso P; Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy.; Govoni M; Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna-Ferrara, University of Ferrara, Ferrara, Italy.; La Torre F; Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.; Cristina Maggio M; University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) 'G.D'Alessandro', University of Palermo, Palermo, Italy.; Montecucco C; Department of Internal Medicine and Therapeutics, University of Pavia, Italy.; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.; De Stefano L; Department of Internal Medicine and Therapeutics, University of Pavia, Italy.; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.; Bugatti S; Department of Internal Medicine and Therapeutics, University of Pavia, Italy.; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.; Rossi S; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.; Makowska J; Department of Rheumatology, Medical University of Lodz, Zeromskiego 113, Lodz, Poland.; Del Giudice E; Pediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Latina, Italy.; Emmi G; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.; Centre for Inflammatory Diseases, Department of Medicine, Monash Medical Centre, Monash University, Clayton, VIC, Australia.; Bartoloni E; Rheumatology Unit, Department of Medicine and Surgery, University of Perugia, Perugia, Italy.; Hernández-Rodríguez J; Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain.; Conti G; Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria (AOU), 'G. Martino' Messina, Italy.; Nunzia Olivieri A; Department of Woman, Child and of General and Specialized Surgery, University of Campania 'Luigi Vanvitelli', Naples, Italy.; Lo Gullo A; UOSD Reumatologia, ARNAS Garibaldi, Catania, Italy.; Simonini G; NEUROFARBA Department, Rheumatology Unit, MeyerChildren's Hospital IRCCS, University of Florence, Florence, Italy.; Viapiana O; Rheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Italy.; Wiesik-Szewczyk E; Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, National Research Institute, Warsaw, Poland.; Erten S; Department of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yıldırım Beyazıt University, Ankara, Turkey.; Carubbi F; Department of Life, Health & Environmental Sciences and Internal Medicine and Nephrology Unit, Department of Medicine, University of L'Aquila and ASL Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.; De Paulis A; Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.; Maier A; Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.; Tharwat S; Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.; Department of Internal Medicine, Faculty of Medicine, Horus University, New Damietta, Egypt.; Costi S; Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Milan, Italy.; Iagnocco A; Academic Rheumatology Centre, Ospedale Mauriziano - Dipartimento Scienze Cliniche e Biologiche, Università degli Studi di Torino, Turin, Italy.; Sebastiani GD; UOC di Reumatologia, Azienda Ospedaliera San Camillo Forlanini, Roma, Italy.; Gidaro A; Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.; Brucato AL; Pediatric Unit, Fatebenefratelli Hospital, Milan, Italy.; Karamanakos A; Joint Academic Rheumatology Program, First Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece.; Akkoç N; Division of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey.; Caso F; Rheumatology Unit, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.; Costa L; Rheumatology Unit, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.; Prete M; Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine (DIM), University of Bari Medical School, Italy.; Perosa F; Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine (DIM), University of Bari Medical School, Italy.; Atzeni F; Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Messina, Italy.; Guggino G; Rheumatology Section, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University Hospital P. Giaccone, University of Palermo, Palermo, Italy.; Fabiani C; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Italy.; Frediani B; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Italy.; Giacomelli R; Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Via Álvaro del Portillo 200, Rome, Italy.; Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome 'Campus Biomedico' School of Medicine, Rome, Italy.; Cantarini L; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Italy.
Source
Publisher: Wiley Country of Publication: United States NLM ID: 101623795 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2326-5205 (Electronic) Linking ISSN: 23265191 NLM ISO Abbreviation: Arthritis Rheumatol Subsets: MEDLINE
Subject
Language
English
Abstract
Objective: We aimed to evaluate the clinical usefulness of the systemic score in the prediction of life-threatening evolution in Still disease. We also aimed to assess the clinical relevance of each component of the systemic score in predicting life-threatening evolution and to derive patient subsets accordingly.
Methods: A multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult-Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life-threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis.
Results: A total of 597 patients with Still disease were assessed (mean ± SD age 36.6 ± 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life-threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07-1.42; P = 0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life-threatening evolution (OR 3.36; 95% CI 1.81-6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48-2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14-4.49; P = 0.042) both significantly predicted life-threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations.
Conclusion: The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted.
(© 2024 American College of Rheumatology.)
Methods: A multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult-Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life-threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis.
Results: A total of 597 patients with Still disease were assessed (mean ± SD age 36.6 ± 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life-threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07-1.42; P = 0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life-threatening evolution (OR 3.36; 95% CI 1.81-6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48-2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14-4.49; P = 0.042) both significantly predicted life-threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations.
Conclusion: The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted.
(© 2024 American College of Rheumatology.)