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

Author:

Vitale Antonio,Caggiano Valeria,Lopalco Giuseppe,Mayrink Giardini Henrique AORCID,Ciccia FrancescoORCID,Almaghlouth Ibrahim A,Ruscitti PieroORCID,Sfikakis Petros P,Tufan AbdurrahmanORCID,Dagna LorenzoORCID,Giacomelli Roberto,Hinojosa-Azaola Andrea,Ragab Gafaar,Direskeneli HanerORCID,Fotis Lampros,Sota Jurgen,Iannone Florenzo,Morrone Maria,de Brito Antonelli Isabele Parente,Dagostin Marilia Ambiel,Iacono Daniela,Patrone Martina,Asfina KaziORCID,Alanazi Fehaid,Di Cola Ilenia,Gaggiano CarlaORCID,Tektonidou Maria GORCID,Kardas Riza Can,Kucuk Hamit,Campochiaro CorradoORCID,Tomelleri AlessandroORCID,Navarini LucaORCID,Berardicurti Onorina,Martín-Nares EduardoORCID,Torres-Ruiz Jiram,Mahmoud Ayman Abdel-Monem Ahmed,Alibaz-Oner FatmaORCID,Kourtesi Katerina,Tarsia Maria,Sfriso PaoloORCID,Makowska Joanna,Govoni Marcello,La Torre Francesco,Maggio Maria Cristina,Monti SaraORCID,Del Giudice Emanuela,Emmi GiacomoORCID,Bartoloni ElenaORCID,Hernández-Rodríguez José,Gómez-Caverzaschi Verónica,Maier Armin,Simonini Gabriele,Iagnocco Annamaria,Conti Giovanni,Olivieri Alma Nunzia,De Paulis Amato,Lo Gullo AlbertoORCID,Viapiana OmbrettaORCID,Wiesik-Szewczyk Ewa,Erten Sukran,Ogunjimi Benson,Carubbi FrancescoORCID,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 LucaORCID

Abstract

ObjectiveStill’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.MethodsSubjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still’s disease.ResultsA 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.ConclusionsDespite 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.

Publisher

BMJ

Subject

Immunology,Immunology and Allergy,Rheumatology

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