Table 4 Reports describing common features shared by AOSD and SJIA or differences between the two conditions
Study | Patients | Similarities | Differences |
|---|---|---|---|
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 lesions | Amyloidosis 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 splenomegaly | Rash 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 rash | Higher 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 data | Serum 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 profiles | Ferritin levels more elevated in AOSD than in SJIA |
Pay et al. [38] | AOSD, n = 95 SJIA, n = 25 | Similar laboratory profiles | Liver 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 | – |