Table 2.
Laboratory measures and medication course for patients with macrophage activation syndrome (MAS).
| Patient | FSTL-1 at Onset of sJIA, ng/ml | FSTL-1 at MAS, ng/ml | ESR, mm/h | Ferritin, ng/ml | sIL2-Rα, pg/ml | Medication Course |
|---|---|---|---|---|---|---|
| 1* | NA | 117 | 16 | 10,000 | 4369.6 | MPSL pulses |
| 2* | NA | 215 | 19 | 1740 | 5985.6 | MPSL pulses |
| 3* | NA | 185 | 22 | NA | 6027.3 | MPSL pulses, cyclosporine |
| 4 | 311 | 289 | 30 | 2416 | 16670 | MPSL pulses, cyclosporine |
| 5 | 175 | 190 | 59 | 8420 | 9063.3 | Anakinra, prednisone oral |
| 6 | † | 389 | 20 | 5424 | 5890 | MPSL pulses |
| 7 | † | 251 | 16 | 2352 | 8349 | MPSL pulses, anakinra |
| 8 | 307 | NA | NA | NA | NA | MPSL pulses, cyclosporine |
| 9 | NA | NA | NA | NA | NA | MPSL pulses, cyclosporine |
*
Three separate episodes of MAS separated by periods of resolution.
†
Patient’s initial presentation was with MAS. FSTL-1: Follistatin-like protein 1; ESR: erythrocyte sedimentation rate; sIL-2Rα: soluble interleukin 2 receptor-α; MPSL: methylprednisolone; NA: not available; sJIA: systemic juvenile idiopathic arthritis.