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. 2023 Dec 1;9(4):e003578.
doi: 10.1136/rmdopen-2023-003578.

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

Antonio Vitale #  1   2 Valeria Caggiano #  3   2 Giuseppe Lopalco  4 Henrique A Mayrink Giardini  5 Francesco Ciccia  6 Ibrahim A Almaghlouth  7   8 Piero Ruscitti  9 Petros P Sfikakis  10 Abdurrahman Tufan  11 Lorenzo Dagna  12   13 Roberto Giacomelli  14   15 Andrea Hinojosa-Azaola  16 Gafaar Ragab  17   18 Haner Direskeneli  19 Lampros Fotis  20 Jurgen Sota  3   2 Florenzo Iannone  4 Maria Morrone  4 Isabele Parente de Brito Antonelli  5 Marilia Ambiel Dagostin  5 Daniela Iacono  6 Martina Patrone  6 Kazi Asfina  8 Fehaid Alanazi  7 Ilenia Di Cola  9 Carla Gaggiano  3   2 Maria G Tektonidou  10 Riza Can Kardas  11 Hamit Kucuk  11 Corrado Campochiaro  12   13 Alessandro Tomelleri  12   13 Luca Navarini  14   15 Onorina Berardicurti  14   15 Eduardo Martín-Nares  16 Jiram Torres-Ruiz  16 Ayman Abdel-Monem Ahmed Mahmoud  17 Fatma Alibaz-Oner  19 Katerina Kourtesi  20 Maria Tarsia  2   21 Paolo Sfriso  22 Joanna Makowska  23 Marcello Govoni  24 Francesco La Torre  25 Maria Cristina Maggio  26 Sara Monti  27   28 Emanuela Del Giudice  29 Giacomo Emmi  30   31 Elena Bartoloni  32 José Hernández-Rodríguez  33 Verónica Gómez-Caverzaschi  33 Armin Maier  34 Gabriele Simonini  35 Annamaria Iagnocco  36 Giovanni Conti  37 Alma Nunzia Olivieri  38 Amato De Paulis  39   40 Alberto Lo Gullo  41 Ombretta Viapiana  42 Ewa Wiesik-Szewczyk  43 Sukran Erten  44 Benson Ogunjimi  45   46   47   48 Francesco Carubbi  49 Samar Tharwat  50   51 Katerina Laskari  52 Stefania Costi  53 Paola Triggianese  54   55 Anastasios Karamanakos  56 Alessandro Conforti  57 Micol Frassi  58 Gian Domenico Sebastiani  59 Antonio Gidaro  60 Angela Mauro  61 Alberto Balistreri  62 Claudia Fabiani  2   63 Bruno Frediani  3   2 Luca Cantarini  1   2
Affiliations

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

Antonio Vitale et al. RMD Open. .

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.

Keywords: Child; Epidemiology; Still's Disease, Adult-Onset.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Flow diagram showcasing the distribution of the enrolled AIDA patients into the three study groups (pediatric-onset, adult-onset and elderly-onset patients) and the fulfilment of classification criteria used before and after the age of 16 (ILAR criteria, PRINTO criteria, Yamaguchi criteria, Fautrel criteria14). AIDA, AutoInflammatory Disease Alliance, ILAR, International League of Associations for Rheumatology; PRINTO, Pediatric Rheumatology International Trials Organization.
Figure 2
Figure 2
Frequency of use of the different conventional disease-modifying antirheumatic drugs (cDMARDs) in the different groups (pediatric-onset, adult-onset and elderly-onset patients).
Figure 3
Figure 3
Kaplan-Meier curves with p values obtained with the Log-rank test. The drug survival was assessed in the three age groups (pediatric, adult and elderly onset) for anakinra (A), canakinumab (B), tocilizumab (C), and tumour necrosis factor (TNF) inhibitors.

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