Still’s disease continuum from childhood to elderly: data from the international AIDA Network Still’s disease registry
Antonio Vitale, Valeria Caggiano, Giuseppe Lopalco, Henrique Ayres Mayrink Giardini, Francesco Ciccia, Ibrahim Almaghlouth, Piero Ruscitti, Petros P. Sfikakis, Abdurrahman Tufan, Lorenzo Dagna, Roberto Giacomelli, Andrea Hinojosa‐Azaola, Gafaar Ragab, Haner Di̇reskeneli̇, Lampros Fotis, Jurgen Sota, Florenzo Iannone, Maria Morrone, Isabele Parente de Brito Antonelli, Marilia Ambiel Dagostin, Daniela Iacono, Martina Patrone, Kazi Nur Asfina, Fehaid Alanazi, Ilenia Di Cola, Carla Gaggiano, Maria G. Tektonidou, Rıza Can Kardaş, Hamit Küçük, Corrado Campochiaro, Alessandro Tomelleri, Luca Navarini, Onorina Berardicurti, Eduardo Martín‐Nares, Jiram Torres‐Ruiz, Ayman Abdel-Monem Ahmed Mahmoud, Fatma Alıbaz-Öner, Katerina Kourtesi, Maria Tarsia, Paolo Sfriso, Joanna Makowska, Marcello Govoni, Francesco La Torre, Maria Cristina Maggio, Sara Monti, Emanuela Del Giudice, Giacomo Emmi, Elena Bartoloni, José Hernández‐Rodríguez, Verónica Gómez‐Caverzaschi, Armin Maier, Gabriele Simonini, Annamaria Iagnocco, Giovanni Conti, Alma Nunzia Olivieri, Amato de Paulis, Alberto Lo Gullo, Ombretta Viapiana, Ewa Więsik–Szewczyk, Şükran Erten, Benson Ogunjimi, Francesco Carubbi, Samar Tharwat, Katerina Laskari, Stefania Costi, Paola Triggianese, Αναστάσιος Καραμανάκος, Alessandro Conforti, Micol Frassi, Gian Domenico Sebastiani, Antonio Gidaro, Angela Mauro, Alberto Balistreri, Claudia Fabiani, Bruno Frediani, Luca Cantarini
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.