Case Report

Successful Tocilizumab Therapy for Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease: A Case-Based Review

Table 2

Case reports of biologic-associated macrophage activation syndrome after administration of biologics for the induction treatment of AOSD.

PtReferenceAge (years)SexDisease duration of AOSDDisease manifestationsTherapy before MAS developing (mg)Biologics caused MASTherapy after biologic-associated MAS (mg)Adverse events after biologics

1Gianella et al. [17]20F2 monthsF-R-A-LEU-L-HSMIbuprofenETNPulse, PSL (100)MAS, death

2Kaneko et al. [18]44M<7 monthsF-A-R-LEUPSL (50–30)ETNPulse: twice, PSL (50), PE: twice, IVIG, AZAMAS

3Kobayashi et al. [19]57F3 weeksF-A-R-LEU-S-L-ESTPSL (80)TCZDXP (0.2 mg/kg), CyA (2 mg/kg) CMV infection, MAS, CDI

4Banse et al. [20]49F6 yearsF-A-R-LEU-S-LMTX (0.3 mg/kg), IFX, ANK, TCZCanakinumabIVIG, PSL (1.5 mg/kg)MAS

MAS, macrophage activation syndrome; AOSD, adult-onset Still’s disease; Pt, patient number; F, fever; A, arthritis; R, skin rash; LEU, leukocytosis; S, sore throat; L, lymphadenopathies; HSM, hepatosplenomegaly; EST, elevated serum transaminases; DIC, disseminated intravascular coagulation; PSL, prednisolone; Pulse, methylprednisolone pulse therapy; MTX, methotrexate; IFX, infliximab; ANK, anakinra; TCZ, tocilizumab; CyA, cyclosporine A; PE, plasma exchange; DXP, dexamethasone palmitate; IVIG, intravenous immunoglobulin; AZA, azathioprine; CMV, cytomegalovirus; CDI, Clostridium difficile infection.