| | Pt | Reference | Age (years) | Sex | Disease duration of AOSD | Disease manifestations before initiation of biologics | Therapy before biologics (mg) | First biologics/response | Second biologics/response | Maintenance therapy after biologics (mg) | Adverse events after biologics |
| | 1 | Maeshima et al. [22] | 16 | F | N.D. | F-A-R-LEU-EST-MAS | Pulse, PSL (60), etoposide, CyA (200), TAC (3), MTX (4–8) | IFX/ineffective | ETN/effective | MTX (12), PSL | PCP |
| | 2 | Loh et al. [23] | 20 | M | 2 weeks | F-R-LEU-S-L-HSM-MAS | Pulse‖, hydrocortisone | ANK/effective | | CyA (100), PSL, MTX (10) | N.D. |
| | 3 | Komiya et al. [24] | 35 | F | <2 months | F-R-LEU-S-L-HSM-EST-MAS | Pulse: 3 times, PSL (80–50), CyA (200), TAC (3), PE | TCZ/effective | | PSL, TAC (3–1) | N.D. |
| | 4 | de Boysson et al. [25]
(2 patients) | 45 | F | 2 weeks | F-A-R-LEU-S-HSM-MAS | Pulse, PSL (1 mg/kg), IVIG: 3 times, MTX (10) | TCZ/effective | | PSL, MTX | N.D. |
| | 5 | de Boysson et al. [25]
(2 patients) | 24 | F | >2 weeks | F-A-R-LEU-S-EST-MAS | PSL (1 mg/kg), MTX (10), IVIG | TCZ/effective | | PSL, MTX | N.D. |
| | 6 | Savage et al. [26] | 32 | M | 4 weeks | F-A-R-LEU-S-fulminant myocarditis-MAS | PSL (40), pulse, IVIG, CyA (3 mg/kg) | ANK/ineffective | TCZ/effective | N.D. | Cardiac arrest after TCZ initiation |
| | 7 | Kobayashi et al. [27] | 61 | F | N.D. | F-A-R-LEU-S-L-HSM-EST-MAS | Pulse: twice, PSL (40), CyA (150), PE | TCZ/effective | | PSL (40–6), CyA (100), MTX (4–6) | N.D. |
| | 8 | Our patient | 71 | F | 4 weeks | F-A-R-LEU-S-L-HSM-EST-MAS | Pulse: twice, PSL (60) | TCZ/effective | | PSL (60) | PCP, CMV infection |
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AOSD, adult-onset Still’s disease; MAS, macrophage activation syndrome; Pt, patient number; N.D., no data; F, fever; A, arthritis; R, skin rash; LEU, leukocytosis; S, sore throat; L, lymphadenopathies; HSM, hepatosplenomegaly; EST, elevated serum transaminases; Pulse, methylprednisolone pulse therapy; PSL, prednisolone; CyA, cyclosporine A; TAC, tacrolimus; MTX, methotrexate; PE, plasma exchange; IVIG, intravenous immunoglobulin; INF, infliximab; ANK, anakinra; TCZ, tocilizumab; ETN, etanercept; PCP, Pneumocystis jirovecii pneumonia; CMV, cytomegalovirus; used after first biologics; this patient continues to do well on TCZ as monotherapy after recovering from cardiac arrest.
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