Table 1 Demographic characteristics, clinical and immunological parameters, therapeutic strategy, and response to the therapy
Simsek-Kiper et al., 2012 | Bader-Meunier et al., 2013 | Hanaya et al., 2017 | Uehara et al., 2018 | Currently presented patient | |
|---|---|---|---|---|---|
Gender, age at onset (years) | Male | Male, 13 | Male, 13 | Male, 24 | Male, 13 |
EULAR/ACR 2019 criteria | |||||
Fever | NA | NA | NA | NA | YES |
Hematologic | Thrombocytopenia | NO | NO | Leukopenia Thrombocytopenia | Leukopenia Lymphopenia Thrombocytopenia |
Neuropsychiatric | NA | NO | NO | NO | NO |
Mucocutaneous | NA | NO | NO | Malar rash | NO |
Pleuritis | NA | Pericarditis | Pericarditis | Pericarditis | Pleuritis and pericarditis |
Articular | NA | Polyarthritis | NO | NO | Polyarthritis |
Renal | NO | NO | NO | NO | Class III LN Zebra bodies |
ANA | NA | 1:800 | 1:640 | 1:2560 | 1:2560 |
Anti-ds-DNA | NA | 39 IU | 87 IU | > 1:400 | 1:640 |
Anti-Smith | NA | Negative | 149 U | Negative | Negative |
Low complement | NA | NO | YES | C3 42 mg/dL C4 5 mg/dL | C3 73 mg/dL C4 2 mg/dL |
aPL and/or LA | NA | NO | NO | YES | IgG aCL, IgG aB2GP, LA |
Treatment | NA | HCQ, ASA | GC | GC | HCQ, MMF, GC, ASA |
Response | NA | Satisfactory (tapered in 3 m) | Satisfactory (tapered in 20 m) | Satisfactory | Satisfactory |