Still's disease continuum from childhood to elderly: data from the international AIDA Network Still's disease registry.

Elena Bartoloni, Alberto Lo Gullo, Amato De Paulis, Alma Nunzia Olivieri, Giovanni Conti, Annamaria Iagnocco, Gabriele Simonini, Armin Maier, Verónica Gómez-Caverzaschi, José Hernández-Rodríguez, Ombretta Viapiana, Giacomo Emmi, Emanuela Del Giudice, Sara Monti, Maria Cristina Maggio, Francesco La Torre, Marcello Govoni, Joanna Makowska, Paolo Sfriso, Anastasios Karamanakos, Luca Cantarini, Bruno Frediani, Claudia Fabiani, Alberto Balistreri, Angela Mauro, Antonio Gidaro, Gian Domenico Sebastiani, Micol Frassi, Alessandro Conforti, Maria Tarsia, Paola Triggianese, Stefania Costi, Katerina Laskari, Samar Tharwat, Francesco Carubbi, Benson Ogunjimi, Sukran Erten, Ewa Wiesik-Szewczyk, Lorenzo Dagna, Isabele Parente de Brito Antonelli, Maria Morrone, Florenzo Iannone, Jurgen Sota, Lampros Fotis, Haner Direskeneli, Gafaar Ragab, Andrea Hinojosa-Azaola, Roberto Giacomelli, Marilia Ambiel Dagostin, Abdurrahman Tufan, Petros P Sfikakis, Piero Ruscitti, Ibrahim A Almaghlouth, Francesco Ciccia, Henrique A Mayrink Giardini, Giuseppe Lopalco, Valeria Caggiano, Hamit Kucuk, Katerina Kourtesi, Fatma Alibaz-Oner, Ayman Abdel-Monem Ahmed Mahmoud, Jiram Torres-Ruiz, Eduardo Martín-Nares, Onorina Berardicurti, Luca Navarini, Alessandro Tomelleri, Corrado Campochiaro, Antonio Vitale, Riza Can Kardas, Maria G Tektonidou, Carla Gaggiano, Ilenia Di Cola, Fehaid Alanazi, Kazi Asfina, Martina Patrone, Daniela Iacono

Journal: RMD open 2023;9(4):e003578

PMID: 38053457

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.

© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

Address: Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy [email protected] [email protected].; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Azienda Ospedaliero-Universitaria Senese, Siena, Italy.; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.; Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de Sao Paulo, Sao Paulo, Brazil.; Department of Precision Medicine, Università degli Studi della Campania Luigi Vanvitelli, Napoli, Italy.; 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.; Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, Università degli Studi dell'Aquila, L'Aquila, Italy.; Joint Academic Rheumatology Program, Medical School, National and Kapodistrian, University of Athens, Athens, Greece.; Department of Internal Medicine, Division of Rheumatology, Gazi University, Ankara, Turkey.; 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.; Clinical and research section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Rome, Italy.; Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Biomedico" School of Medicine, Rome, Italy.; Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición, Mexico City, Mexico.; Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Cairo University, Giza, Egypt.; Faculty of Medicine, Newgiza University, 6th of October City, Egypt.; Department of Internal Medicine, Division of Rheumatology, Marmara University, School of Medicine, Istanbul, Turkey.; Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.; Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy.; Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy.; Department of Rheumatology, Medical University of Lodz, Lodz, Poland.; Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. 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