Table 2

Demographic characteristics of the samples, patterns and courses, and criteria fulfilled, by group (SR1: prevalence of manifestations)

Articles (n)Total number of patientsPatients with sJIA (n)Patients with AOSD (n)sJIAAOSD
Weighted average descriptor
% women8101043157953%50%
Age (years)3193107868.546.5
Age at onset (years)*29947525.127.2
Diagnostic delay (years)29947525.24.4
Follow-up (months)299475225.719.7
Weighted % descriptor
Systemic pattern†‡228311017373%72%
Articular pattern§‡12388615226%22%
Monocyclic course¶457923834128%31%
Polycyclic course¶457923834141%35%
Chronic course¶477230047226%24%
Unclassified course¶335811124710%14%
Weighted % criteria
ILAR (2001 Edmonton revision)**53723423087%17%
ILAR (1997 Durban revision)**12525100%
PRINTO1727256%
Yamaguchi857057088%
Fautrel114314366%
ACR 197224747100%
Medsger and Christy13131100%
  • Results are presented in weighted percentages or means.

  • *Ruscitti et al 65 do not specify whether age is current age or age at onset or at diagnosis; however, the other parameters were collected at diagnosis, so we considered age to be age at diagnosis. Nevertheless, it could be current age, because 11 and 52 years seem too old for sJIA and AOSD onset, respectively.

  • †In Lin et al,32 100% of patients had initial systemic onset, but 46% of patients with sJIA and 38% of patients with AOSD exhibited chronic arthritis during follow-up.

  • ‡Only 2 studies provided information on the disease pattern (systemic vs articular): Lin et al 32 (with 100% systemic pattern at onset for both patients with sJIA and AOSD) and Neau et al 29 (65.1% and 67.7% systemic pattern for sJIA and AOSD, respectively).

  • §In Neau et al 29, the ‘articular pattern’ is defined as ‘chronic pattern’ and corresponded to the ‘chronic course’.

  • ¶The table reports for each study how the authors classified the course of the disease, although the strict definition of monocyclic/polycyclic/chronic and unclassified course may vary among studies (for the exact definition, please refer to each study).

  • **All studies using the ILAR criteria were based on the second Edmonton revision,15 except Pay et al 31 which used the first Durban revision.17

  • ACR 1972, American College of Rheumatology 1972 criteria; AOSD, adult-onset Still’s disease; ILAR, International League of Associations for Rheumatology; PRINTO, Paediatric Rheumatology INternational Trials Organisation; sJIA, systemic juvenile idiopathic arthritis.