Table 3 Clinical characteristics.
Patient 1 | Patient 2 | Patient 3 | Patient 4 | |
|---|---|---|---|---|
Sex | Female | Male | Female | Female |
Age at onset of MAS | 9 years | 5 years | 15 years | 3 years |
Underlying disease | SoJIA | SoJIA | SLE | SoJIA |
Ongoing treatment at onset of MAS | Tocilizumab, Mtx s.c. | Corticosteroids, anakinra, CsA | Hydroxychloroquine | Corticosteroids, tocilizumab, Mtx s.c. |
Verified infections at MAS onset | EBV | None | None | VZV |
Diagnostic criteria PRES | Yes | Yes | Yes | Yes |
Diagnostic criteria HLH-2004 | Yes | Yes | Yes | Yes |
Neurological symptoms | Severe | No | Severe | Severe |
First-line MAS therapy | MP-pulses | MP-pulses | MP-pulses | MP-pulses |
Etoposide | 90–120 mg/m2/dose for 7 wks | 50–100 mg/m2/dose for 6 wks | 50–75 mg/m2/dose for 5 wks | 70 mg/m2/dose for 4 wks |
Additional treatment | ICU care, corticosteroids, CsA, rituximab | ICU care, corticosteroids | ICU care, corticosteroids, CsA | ICU care, corticosteroids |
Clinical response | Complete | Complete | Severe CNS sequele | Partial CNS sequele |