Table 2 The clinical and laboratory characteristics of JDM patients at initial presentation of MAS
Patient number | Study (year) | Fever | Serum enzymes | Ferritin (ng/mL) | Blood cells | sIL-2R (U/ml) | HSM | HPC | Treatment and outcome |
|---|---|---|---|---|---|---|---|---|---|
1 | Kobayashi et al. (2000) | No | AST 309 U/L, LDH 752 U/L | 765 | WBC 3.4*109/L, Hb 12.2Â g/dL, PLT 7*109/L | 1120 | Yes | Yes | Oral PSL, CsA, IVIG; improvement |
2 | Sterba et al. (2004) | Yes | ND | ND | Severe pancytopenia | ND | Yes | No | CS, CsA; improvement |
3 | Kobayashi et al. (2006) | Yes | AST 145 U/L, LDH 941 U/L | 679 | WBC 2.7*109/L, Hb 14.6Â g/dL, PLT 119*109/L | 1610 | ND | Yes | IVMP, CsA; improvement |
4 | Yajima et al. (2008) | Yes | ND | 2796 | Neu 2.56*109/L, Hb 7.7Â g/dL, PLT 39*109/L | 882 | Yes | Yes | IVMP, ISAs, IVIG; Death |
5 | Bustos et al. (2012). | Yes | ALT 241 U/L, AST 515 U/L, LDH 1537 U/L | 1789 | Cytopenia | ND | Yes | ND | IVMP, CsA, IVIG, PE; improvement |
6 | Wang et al. (2013) | Yes | ALT 64 U/L, AST 145 U/L, LDH 707 U/L | > 1500 | WBC 2.02*109/L, Hb 12.5 g/dL, PLT 84*109/L | ND | Yes | Yes | IVMP/PSL, CYC; improvement |
7 | Yamashita et al. (2013) | Yes | AST 721 U/L, ALT 292 U/L, LDH 1069 U/L | 4172 | WBC 2.82*109/L, Hb 8.6Â g/dL, PLT 112*109/L | 2046 | Yes | ND | IVDEX, CSA, CYC; Death (diffuse alveolar damage) |
8 | Lilleby et al. (2014) | Yes | ND | 7437 | ND | ND | ND | Yes | IVMP/PSL, IVIG, CSA, ETO, anakinra; improvement |
9 | Ye et al. (2014) | Yes | ALT 268 U/L, AST 147 U/L, LDH 964 U/L | 8962 | WBC 7.84*109/L, Hb 14.2Â g/dL, PLT 122*109/L | ND | Yes | Yes | IVMP/DEX, CsA; improvement |
10 | Poddighe et al. (2014) | Yes | AST 50 U/L, ALT 14 U/L, LDH 890 U/L | 5899 | WBC 9.5*109/L, Hb 11.7Â g/dL, PLT 244*109/L | ND | Yes | Yes | IVMP/oral PSL, CSA; improvement |
11 | Önen et al. (2014) | Yes | AST 205 U/L, ALT 59 U/L, LDH 1085 U/L | 14,657 | WBC 6.92*109/L, Hb 8.4 g/dL, PLT 144*109/L | ND | Yes | Yes | IVMP, CSA, IVIG; improvement |
12 | Wakiguchi et al. (2015) | Yes | AST1154 U/L, ALT 596 U/L, LDH 2267 U/L | 8062 | WBC 2.56*109/L, Hb 13.4Â g/dL, PLT 119*109/L | ND | ND | Yes | IVMP/DEX, CsA, CYC; improvement |
13 | Teshigawara et al. (2016) | No | ND | 967.4 | WBC 3.9*109/L, Hb 13.4Â g/dL, PLT 124*109/L | ND | ND | Yes | IVMP/DEX/, CYC, CsA/TAC; improvement |
14 | Deschamps et al. (2018) | Yes | ALT 245 U/L, AST 302 U/L, LDH 578 U/L | 577 | WBC 3.9*109/L, Hb 10.7Â g/dL, PLT 124*109/L | ND | ND | Yes | IVMP/oral PSL, IVIG, CsA; improvement |
15 | Stewart JA et al. (2022) | Yes | ALT 157 U/L, AST 281 U/L, LDH 696 U/L | 1641 | Pancytopenia | ND | ND | ND | IVMP/DEX, Anakinra, IVIG, ETO, TOF; improvement |
16 | Mouri M et al. (2022) | No | ALT 795 U/L, AST 1608 U/L, LDH 925 U/L | 619 | WBC 2.8*109/L, Hb 13.3Â g/dL, PLT 69*109/L | ND | Yes | Yes | IVMP, TAC, IVIG, CsA, CYC, PE; improvement |
17 | Jagwani H et al. (2022) | No | ND | 4059 | Pancytopenia | ND | ND | ND | IVMP, IVIG, CsA; improvement |
18 | Our case | Yes | ALT 153 U/L, AST 199 U/L, LDH 780 U/L | 4322 | WBC 2.8*109/L, Hb 10.2Â g/dL, PLT 85*109/L | ND | Yes | Yes | IVMP, CsA; Death |