Still’s disease continuum from childhood to elderly: data from the international AIDA Network Still’s disease registry
Authors: Vitale Antonio , Caggiano Valeria , Lopalco Giuseppe , Mayrink Giardini Henrique A , Ciccia Francesco , Almaghlouth Ibrahim A , Ruscitti Piero , Sfikakis Petros P , Tufan Abdurrahman , Dagna Lorenzo , Giacomelli Roberto , Hinojosa-Azaola Andrea , Ragab Gafaar , Direskeneli Haner , Fotis Lampros , Sota Jurgen , Iannone Florenzo , Morrone Maria , de Brito Antonelli Isabele Parente , Dagostin Marilia Ambiel , Iacono Daniela , Patrone Martina , Asfina Kazi , Alanazi Fehaid , Di Cola Ilenia , Gaggiano Carla , Tektonidou Maria G , Kardas Riza Can , Kucuk Hamit , Campochiaro Corrado , Tomelleri Alessandro , Navarini Luca , Berardicurti Onorina , Martín-Nares Eduardo , Torres-Ruiz Jiram , Mahmoud Ayman Abdel-Monem Ahmed , Alibaz-Oner Fatma , Kourtesi Katerina , Tarsia Maria , Sfriso Paolo , Makowska Joanna , Govoni Marcello , La Torre Francesco , Maggio Maria Cristina , Monti Sara , Del Giudice Emanuela , Emmi Giacomo , Bartoloni Elena , Hernández-Rodríguez José , Gómez-Caverzaschi Verónica , Maier Armin , Simonini Gabriele , Iagnocco Annamaria , Conti Giovanni , Olivieri Alma Nunzia , De Paulis Amato , Lo Gullo Alberto , Viapiana Ombretta , Wiesik-Szewczyk Ewa , Erten Sukran , Ogunjimi Benson , Carubbi Francesco , Tharwat Samar , Laskari Katerina , Costi Stefania , Triggianese Paola , Karamanakos Anastasios , Conforti Alessandro , Frassi Micol , Sebastiani Gian Domenico , Gidaro Antonio , Mauro Angela , Balistreri Alberto , Fabiani Claudia , Frediani Bruno , Cantarini Luca 
Published: December 2023

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.