Table 1

Main characteristics of the included studies in SR1: prevalence of manifestations

First author, year, countryStudy design, number of centresReferral centre(s)Number of sJIA/AOSD/total patientsNumber of women with sJIA/AOSD/total patients (%)Diagnostic criteria met, sJIA, %Diagnostic criteria met, AOSD, %Disease coursePouchot systemic scoreRoB: score, overall risk
Uppal, 1995,
India30
LOB, retrospective,
NR
NRsJIA: 23
AOSD: 31
Total: 54
sJIA: 10 (43.4)
AOSD: 14 (45.1)
Total: 24 (44.4)
ACR 1972: 100Yamaguchi: 100
Medsger and Christy: 100
Monocyclic: 17.4%
sJIA—
22.6% AOSD
Polycyclic: 17.4% sJIA—19.4% AOSD
Chronic: 52.2% sJIA—51.6% AOSD
Unclassified: 13.0% sJIA—6.4% AOSD
Mean, original: 3.7 for both sJIA and AOSD4
Lin, 2000, Taiwan32 LOB, retrospective, 1NRsJIA: 24
AOSD: 21
Total: 45
sJIA: 14 (58.3)
AOSD: 12 (57.1)
Total: 26 (57.7)
ACR 1972: 100Yamaguchi: 100Monocyclic: 66.7% sJIA—57.1% AOSD
Polycyclic: 33.3% sJIA—42.9% AOSD
NR5
Pay, 2006,
Turkey31
LOB, retrospective, 6YessJIA: 25
AOSD: 95
Total: 120
sJIA: 13 (52.0)
AOSD: 50 (52.6)
Total: 63 (52.5)
ILAR criteria (Durban first revision): 100Yamaguchi: 100Monocyclic: 21.1% sJIA—21% AOSD
Polycyclic: 41.7% sJIA—16.8% AOSD
Chronic: 29.2% sJIA—41.1% AOSD
Unclassified: 8.3% sJIA—21.1% AOSD
NR4
Inoue, 2016,
Japan12
LOB, prospective, 1NRsJIA: 77
AOSD: 33
Total: 110
sJIA: 32 (41.5)
AOSD: 21 (63.6)
Total: 53 (48.2)
ILAR: 100Yamaguchi: 100NRNR5
Kudela, 2019,
Germany28
Cross-sectional, retrospective, NRNRsJIA: 20
AOSD: 30
Total: 50
sJIA: 9 (45.0)
AOSD: (20.0)
Total: 15 (30.0)
ILAR: 55.5
By physicians: 100
ILAR: 16.7
Yamaguchi: 66.7
By physicians: 100
NRMean, modified:
3.5±1.1 for sJIA (n=15)
4.0±1.4 for AOSD (n=19)
6
Ruscitti, 2017, Italy65 LOB, retrospective, 1NRsJIA: 21
AOSD: 29
Total: 50
sJIA: 15 (71.4)
AOSD: 6 (20.7)
Total: 21 (42.0)
ILAR: 100Yamaguchi: 100NRMean, original:
4.620±2.110 for sJIA
5.591±2.041 for AOSD
6
Ruscitti, 2022,
Italy66
LOB, retrospective, 11YessJIA: 166
AOSD: 194
Total: 360
sJIA: 87 (52.4)
AOSD: 92 (47.4)
Total:179 (49.7)
ILAR: 100Yamaguchi: 100Monocyclic: 24.7% sJIA—34.5% AOSD
Polycyclic: 45.2% sJIA—44.8% AOSD
Chronic: 21.7% sJIA—12.9% AOSD
Median, original:
3 (2–5) for sJIA
5 (4–7) for AOSD
5
Neau, 2022,
France29
LOB, retrospective, 10NRsJIA: 86
AOSD: 152
Total: 238
sJIA: 48 (55.8)
AOSD: 88 (57.9)
Total: 136 (57.1)
ILAR: 50.7
PRINTO: 55.5
By physicians: 100
Yamaguchi: 60.8
Fautrel: 65.7
By physicians: 100
Chronic: 25.5% sJIA—21.7% AOSD
Unclassified: 9.3% sJIA—10.5% AOSD
NR5
  • The articles are sorted by year of publication.

  • 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). Disease pattern (systemic vs chronic articular) is not provided in the table because only 2 studies provided information on this issue: Lin et al 32 (with 100% systemic pattern for both sJIA and AOSD) and Neau et al 29 (65.1% and 67.7% systemic pattern for sJIA and AOSD, respectively).

  • Pouchot systemic score, original22 or modified by Rau et al.23 Data are mean±SD or median (IQR).

  • Risk of bias, adapted from Hoy scale. Summary of the overall risk of study bias: low risk (0–3), moderate risk (4–5), high risk (6–8). Green colour, low risk (no study had low risk in this table). Orange colour, moderate risk. Red colour, high risk.

  • All studies using the ILAR criteria were based on the second 2001 Edmonton revision,15 except Pay et al 31 which used the first 1997 Durban revision.17

  • For Inoue et al 12: the number of patients is 77 for sJIA, 33 for AOSD, 110 for total patients. However, for many clinical or biological features, the authors provide for each feature the number (n) reported on the number (N) of active patients, which is 66 for sJIA, 27 for AOSD, 93 in total.

  • For Kudela et al,28 the diagnosis was established by at least two experienced physicians.

  • For Neau et al, 29 the diagnosis was retained if it was confirmed by the referring physician at the last follow-up.

  • This table does not include the age of patients because data were too heterogeneous: 3 studies gave the current age at time of study, 2 the mean age at onset and 4 the median age at onset or diagnosis. Similarly, it does not include the mean delay in diagnosis (because it was only provided in 2 studies), nor the mean follow-up (because it was only reported in 2 studies).

  • ACR 1972, American College of Rheumatology 1972 criteria; AOSD, adult-onset Still’s disease; ILAR, International League of Associations for Rheumatology; LOB, longitudinal observational; NR, not reported or not clearly reported; PRINTO, Paediatric Rheumatology INternational Trials Organisation; sJIA, systemic juvenile idiopathic arthritis.