RMD Open (Oct 2023)
Still’s disease continuum from childhood to elderly: data from the international AIDA Network Still’s disease registry
- Micol Frassi,
- Marcello Govoni,
- Annamaria Iagnocco,
- Florenzo Iannone,
- Paola Triggianese,
- Corrado Campochiaro,
- Sara Monti,
- Maria G Tektonidou,
- Eduardo Martin-Nares,
- Piero Ruscitti,
- Roberto Giacomelli,
- Luca Cantarini,
- Giuseppe Lopalco,
- Lorenzo Dagna,
- Francesco Carubbi,
- Alma Nunzia Olivieri,
- Antonio Vitale,
- Ombretta Viapiana,
- Fatma Alibaz-Öner,
- Haner Direskeneli,
- Petros P Sfikakis,
- Giacomo Emmi,
- Claudia Fabiani,
- Gabriele Simonini,
- Francesco Ciccia,
- Elena Bartoloni,
- Alessandro Tomelleri,
- Daniela Iacono,
- Riza Can Kardas,
- Bruno Frediani,
- Benson Ogunjimi,
- Amato de Paulis,
- Onorina Berardicurti,
- Alessandro Conforti,
- Ilenia Di Cola,
- Anastasios Karamanakos,
- Katerina Laskari,
- Abdurrahman Tufan,
- Stefania Costi,
- José Hernández-Rodríguez,
- Lampros Fotis,
- Jurgen Sota,
- Antonio Gidaro,
- Ewa Wiesik-Szewczyk,
- Gian Domenico Sebastiani,
- Jiram Torres-Ruiz,
- Paolo Sfriso,
- Giovanni Conti,
- Luca Navarini,
- Francesco La Torre,
- Samar Tharwat,
- Andrea Hinojosa-Azaola,
- Alberto Lo Gullo,
- Valeria Caggiano,
- Ibrahim A Almaghlouth,
- Kazi Asfina,
- Gafaar Ragab,
- Maria Cristina Maggio,
- Joanna Makowska,
- Emanuela Del Giudice,
- Armin Maier,
- Sukran Erten,
- Henrique A Mayrink Giardini,
- Maria Morrone,
- Isabele Parente de Brito Antonelli,
- Marilia Ambiel Dagostin,
- Martina Patrone,
- Fehaid Alanazi,
- Carla Gaggiano,
- Hamit Kucuk,
- Ayman Abdel-Monem Ahmed Mahmoud,
- Katerina Kourtesi,
- Maria Tarsia,
- Verónica Gómez-Caverzaschi,
- Angela Mauro,
- Alberto Balistreri
Affiliations
- Micol Frassi
- Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Brescia, Brescia, Italy
- Marcello Govoni
- Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna - Ferrara, University of Ferrara, Ferrara, Italy
- Annamaria Iagnocco
- Academic Rheumatology Centre, Ospedale Mauriziano - Dipartimento Scienze Cliniche e Biologiche, Università degli Studi di Torino, Turin, Italy
- Florenzo Iannone
- Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy
- Paola Triggianese
- Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy
- Corrado Campochiaro
- Division of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy
- Sara Monti
- Rheumatology Department, Istituto di ricovero e cura a carattere scientifico Policlinico S. Matteo Fondazione, University of Pavia, Pavia, Italy
- Maria G Tektonidou
- Cardiovascular Risk Research Laboratory, First Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens School of Medicine, Athens, Greece
- Eduardo Martin-Nares
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas Y Nutrición Salvador Zubirán, Mexico City, Mexico
- Piero Ruscitti
- Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L`Aquila, L`Aquila, Italy
- Roberto Giacomelli
- Rheumatology and Clinical Immunology, Department of Medicine, University of Rome “Campus Biomedico”, School of Medicine, Rome, Italy
- Luca Cantarini
- UOC Reumatologia, Azienda Ospedaliero-Universitaria Senese, ERN-RITA Center, Siena, Italy
- Giuseppe Lopalco
- Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy
- Lorenzo Dagna
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
- Francesco Carubbi
- 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
- Alma Nunzia Olivieri
- Department of Woman, Child and of General and Specialized Surgery, University of Campania Luigi Vanvitelli, Napoli, Italy
- Antonio Vitale
- UOC Reumatologia, Azienda Ospedaliero-Universitaria Senese, ERN-RITA Center, Siena, Italy
- Ombretta Viapiana
- Rheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy
- Fatma Alibaz-Öner
- Department of Internal Medicine, Division of Rheumatology, Marmara University, School of Medicine, Istanbul, Turkey
- Haner Direskeneli
- Department of Internal Medicine, Division of Rheumatology, Marmara University, School of Medicine, Istanbul, Turkey
- Petros P Sfikakis
- Cardiovascular Risk Research Laboratory, First Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens School of Medicine, Athens, Greece
- Giacomo Emmi
- Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
- Claudia Fabiani
- Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy
- Gabriele Simonini
- NEUROFARBA Department, Rheumatology Unit, Meyer Children`s University Hospital, University of Florence, Firenze, Italy
- Francesco Ciccia
- Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy
- Elena Bartoloni
- Department of Medicine and Surgery, MED/16- Rheumatology, Università degli studi di Perugia, P.zza Università, Perugia, Italy
- Alessandro Tomelleri
- Division of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy
- Daniela Iacono
- Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy
- Riza Can Kardas
- Department of Internal Medicine, Division of Rheumatology, Gazi University, Ankara, Turkey
- Bruno Frediani
- UOC Reumatologia, Azienda Ospedaliero-Universitaria Senese, ERN-RITA Center, Siena, Italy
- Benson Ogunjimi
- Antwerp Unit for Data Analysis and Computation in Immunology and Sequencing, University of Antwerp, Antwerp, Belgium
- Amato de Paulis
- Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Napoli, Italy
- Onorina Berardicurti
- Rheumatology and Clinical Immunology, Department of Medicine, University of Rome “Campus Biomedico”, School of Medicine, Rome, Italy
- Alessandro Conforti
- U.O.Medicina Generale, Ospedale San Paolo di Civitavecchia, ASL Roma 4, Civitavecchia, Rome, Italy
- Ilenia Di Cola
- Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L`Aquila, L`Aquila, Italy
- Anastasios Karamanakos
- Joint Academic Rheumatology Program, First Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece
- Katerina Laskari
- Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland
- Abdurrahman Tufan
- Division of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey
- Stefania Costi
- Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Milano, Italy
- José Hernández-Rodríguez
- Vasculitis Research Unit and Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, IDIBAPS, University of Barcelona, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Barcelona, Spain
- Lampros Fotis
- Third Department of Paediatrics, National and Kapodistrian University of Athens, General University Hospital Attikon, Athens, Greece
- Jurgen Sota
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet`s Disease Clinic, University of Siena, Siena, Italy
- Antonio Gidaro
- Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy
- Ewa Wiesik-Szewczyk
- Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Poland Ministry of Interior and Administration, Warszawa, Poland
- Gian Domenico Sebastiani
- UOC di Reumatologia, Azienda Ospedaliera San Camillo Forlanini, Roma, Italy
- Jiram Torres-Ruiz
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas Y Nutrición Salvador Zubirán, Mexico City, Mexico
- Paolo Sfriso
- Rheumatology Unit, Department of Medicine (DIMED), University of Padova, Padova, Italy
- Giovanni Conti
- Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria (AOU), G. Martino, Messina, Italy
- Luca Navarini
- Rheumatology and Clinical Immunology, Department of Medicine, University of Rome “Campus Biomedico”, School of Medicine, Rome, Italy
- Francesco La Torre
- Clinical Pediatrics, University of Bari, Bari, Italy
- Samar Tharwat
- Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt
- Andrea Hinojosa-Azaola
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas Y Nutrición Salvador Zubirán, Mexico City, Mexico
- Alberto Lo Gullo
- UOSD Reumatologia ARNAS Garibaldi, Catania, Italy
- Valeria Caggiano
- UOC Reumatologia, Azienda Ospedaliero-Universitaria Senese, ERN-RITA Center, Siena, Italy
- Ibrahim A Almaghlouth
- Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
- Kazi Asfina
- Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
- Gafaar Ragab
- Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt
- Maria Cristina Maggio
- University Department Pro.Sa.M.I. G. D`Alessandro, University of Palermo, Palermo, Italy
- Joanna Makowska
- Department of Rheumatology, Medical University of Lodz, Łódź, Poland
- Emanuela Del Giudice
- Pediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Sapienza, University of Rome, Polo Pontino, Latina, Italy
- Armin Maier
- Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy
- Sukran Erten
- Department of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yıldırım Beyazıt University, Ankara, Turkey
- Henrique A Mayrink Giardini
- Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de Sao Paulo, Sao Paulo, Brazil
- Maria Morrone
- Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy
- Isabele Parente de Brito Antonelli
- Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de Sao Paulo, Sao Paulo, Brazil
- Marilia Ambiel Dagostin
- Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de Sao Paulo, Sao Paulo, Brazil
- Martina Patrone
- Department of Precision Medicine, Università degli Studi della Campania Luigi Vanvitelli, Napoli, Italy
- Fehaid Alanazi
- Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
- Carla Gaggiano
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet`s Disease Clinic, University of Siena, Siena, Italy
- Hamit Kucuk
- Department of Internal Medicine, Division of Rheumatology, Gazi University, Ankara, Turkey
- Ayman Abdel-Monem Ahmed Mahmoud
- Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Cairo University, Giza, Egypt
- Katerina Kourtesi
- Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece
- Maria Tarsia
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Azienda Ospedaliero-Universitaria Senese, Siena, Italy
- Verónica Gómez-Caverzaschi
- 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
- Angela Mauro
- Pediatric Unit, Fatebenefratelli Hospital, Milan, Milan
- Alberto Balistreri
- Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy
- DOI
- https://doi.org/10.1136/rmdopen-2023-003578
- Journal volume & issue
-
Vol. 9,
no. 4
Abstract
Objective Still’s disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still’s disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still’s disease.Methods Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still’s disease.Results A total of 411 patients suffering from Still’s disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still’s disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p<0.0001) were significantly more frequent among elderly-onset patients compared with paediatric-onset subjects. Regarding laboratory data, thrombocytosis was significantly more frequent among paediatric patients onset compared with adult-onset subjects (p<0.0001), while thrombocytopenia was more frequent among elderly-onset patients although statistical significance was only bordered. No substantial differences were observed in the response to treatments.Conclusions Despite some minor difference between groups, overall, demographic, clinical, laboratory and treatments aspects of Still’s disease were similarly observed in patients at all ages. This supports that pediatric-onset, adult-onset and elderly-onset Still’s disease is the same clinical condition arising in different ages.
