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Table 4 Reports describing common features shared by AOSD and SJIA or differences between the two conditions

From: Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts

StudyPatientsSimilaritiesDifferences
Clinical features
 Bywaters [2]AOSD, n = 14
SJIA, NA
Adult patients with the same clinical features of SJIA: “Still disease” of the adults?
 Cabane et al. [32] (retrospective chart review)AOSD, n = 8
SJIA, n = 10
No significant differences in initial systemic manifestations or in joint lesionsAmyloidosis restricted to AOSD (n = 3)
 Tanaka et al. [44] (in Japanese; abstract in English)AOSD, n = 19
SJIA, n = 26
Fever and arthritis in all patients
Polyarthritis, 77% SJIA versus 53% AOSD
Sore throat more frequent in AOSD (AOSD 68% versus SJIA 19%)
 Ohta and Yamaguchi [41] (in Japanese; abstract in English)AOSD, n = 92
SJIA, n = 11
91–93% clinical items statistically not different between SJIA and AOSD
 Uppal et al. [4] (retrospective analysis)AOSD, n = 31
SJIA, n = 23
Similar incidence of fever, sore throat, lymphadenopathy, and splenomegalyRash and hepatomegaly more common in SJIA
Polyarthritis more common in AOSD
 Luthi et al. [37] (retrospective review)AOSD, n = 10
SJIA, n = 9
Similar presentation at onset (systemic and articular manifestations)
 Lin et al. [36] (retrospective analysis)AOSD, n = 21
SJIA, n = 24
Similar presentation at onset (systemic and articular manifestations)Sore throat more frequent in AOSD
 Pay et al. [38] (multicenter retrospective review)AOSD, n = 95
SJIA, n = 25
No differences in the pattern of fever and localization of skin rashHigher frequency of fever, rash, myalgia, weight loss, and sore throat in AOSD
Response to treatment/clinical outcome
 Cabane et al. [32]AOSD, n = 8
SJIA, n = 10
The same treatments
Poor articular prognosis in both conditions
 Tanaka et al. [44]AOSD, n = 19
SJIA, n = 26
The same articular prognosis with the same treatments
 Uppal et al. [4]AOSD, n = 31
SJIA, n = 23
Similar clinical outcomes
 Luthi et al. [37]AOSD, n = 10
SJIA, n = 9
Similar clinical outcomes
 Lin et al. [36]AOSD, n = 21
SJIA, n = 24
Better articular outcome in AOSD than SJIA
 Pay et al. [38]AOSD, n = 95
SJIA, n = 25
The same treatments in both conditions
 Feist et al. [33] (subgroup analysis of pooled data from 4 SJIA studies with canakinumab)AOSD, n = 29
SJIA, n = 272
The same response to treatment with canakinumab
Laboratory parameters
 Tanaka et al. [44]AOSD, n = 19
SJIA, n = 26
Similar initial laboratory dataSerum iron levels more elevated in AOSD than SJIA
 Uppal et al. [4]AOSD, n = 31
SJIA, n = 23
Similar laboratory profiles
 Luthi et al. [37]AOSD, n = 10
SJIA, n = 9
Similar laboratory profiles
Ferritin levels elevated both in SJIA and AOSD
 Lin et al. [36]AOSD, n = 21
SJIA, n = 24
Similar laboratory profilesFerritin levels more elevated in AOSD than in SJIA
 Pay et al. [38]AOSD, n = 95
SJIA, n = 25
Similar laboratory profilesLiver dysfunction and neutrophilia more common in AOSD than in SJIA
 Hashimoto et al. [39] (in Japanese; abstract in English)AOSD, n = 7
SJIA, n = 4
IL-2 receptor elevated in sera of AOSD and SJIA patients compared to controls
 Bae et al. [29]AOSD, n = 37
SJIA, NA
S100A12 (calcium-binding protein) elevation in sera of AOSD compared to controls (as described in SJIA)
 Kudela et al. [47]
(abstract)
AOSD, n = 30
SJIA, n = 20
Highly elevated IL-18 serum levels in both active AOSD and SJIA
Genetic profile
 Miller et al. [48]AOSD, n = 16
SJIA, n = 35
HLA-DR4 more involved in SJIA
HLA-DR7 more involved in AOSD
 Cush et al. [50]
(ACR-ARHP abstract)
AOSD, n = 21
SJIA, NA
Genomic profiling in AOSD patients similar to studies in SJIA patients
 Nirmala et al. [49]AOSD, n = 17
SJIA, NA
Similar genes upregulated/downregulated by treatment with IL-1 inhibitors in AOSD compared to studies in SJIA patients
Classification criteria
 Talesnik et al. [43] (retrospective analysis in Spanish; abstract in English)AOSD, n = 7
SJIA, n = 14
The same proportion of patients in each form of disease course (monocyclic systemic form, polycyclic systemic form, monocyclic chronic joint form, polycyclic chronic joint form)
 El Hamshary et al. [54] (retrospective cross-sectional study)AOSD, NA
SJIA, n = 30
23/30 SJIA patients fulfilled Yamaguchi criteria and 20/30 ILAR criteria
 Kumar et al. [52] (retrospective chart review)AOSD, NA
SJIA, n = 31
23/31 SJIA patients fulfilled Yamaguchi criteria and 18/31 ILAR criteria
 Oliveira Ramos et al. [55] (analysis of Portuguese rheumatic disease registry)AOSD, NA
SJIA, n = 66
35/66 SJIA patients fulfilled criteria for AOSD
 Yang et al. [53] (retrospective review)AOSD, n = 169
SJIA, NA
ILAR criteria for SJIA can identify AOSD patients at risk of relapse, MAS, and ICU
 Debach et al. [56] (abstract)AOSD, NA
SJIA, n = 17
42% of patients with SJIA fulfilled Yamaguchi criteria for AOSD
  1. AOSD adult-onset Still’s disease, ICU intensive care unit, IL interleukin, ILAR International Leagues of Associations of Rheumatology, MAS macrophage activation syndrome, SJIA systemic juvenile idiopathic arthritis