Table 1 Clinical and laboratory findings of cases 1–3
Case 1 | Case 2 | Case 3 | |
|---|---|---|---|
Gender | Female | Female | Female |
Age at presentation | Neonate | 22 months | 16 years |
Failure to thrive | Yes | Unknown | Unknown |
Rash | No | Yes | Unknown |
ILD | Yes | Yes | Yes |
Arthritis | No | Polyarticular JIA | Polyarticular JIA |
CRP (mg/L) | 56 | < 5–46 | 3a |
Hb (g/L) | 7.1 (NR 11.4–14.1) | 11.1 (NR 11.5–16.5) | 12.1 (NR 11.5–16.5) |
C3 (g/L) | 1.47 (NR 0.9–1.8) | 1.65 (NR 0.75–1.65) | Unknown |
C4 (g/L) | 0.24 (NR 0.1–0.4) | 0.2 (NR 0.14–0.54) | Unknown |
IgG (g/L) | 11.49 (NR 2.4–8.8) | 26.0 (NR 6.0–16.0) | 10.10 (NR 6.0–16.0) |
IgA (g/L) | 0.66 (NR 0.1–0.5) | 3.5 (NR 0.5–2.4) | 3.06 (NR 0.8–2.8) |
IgM (g/L) | 1.36 (NR 0.2–1.0) | 2.1 (NR 0.5–1.8) | 1.26 (NR 0.5–1.9) |
Anti-smooth muscle | Positive | Negative | Negative |
ANA | Negative | 1:320 | 1:80/negativeb |
ENA | Negative | Negative | Negative |
Anti-dsDNA (mg/L) | Negative | Negative | 1.2 |
RF (IU/mL) | Negative | 123 | 797 |
Anti-CCP | Not performed | Negative | Not performed |