The efficacy of tolizumab in neuromyelitis optica spectrum disorders combined with connective tissue disease survey
Objective To evaluate the efficacy and safety of tocilizumab(TCZ)for the treatment of neuromyelitis optica spectrum disorders(NMOSD)with anti-aquaporin 4-immunoglobulin(AQP4-IgG)positivity in combination with connective tissue disease(CTD).Methods Six serum AQP4-IgG-positive NMOSD patients with at least one episode of NMOSD in the 12 months prior to enrollment and comorbid with CTD,including four patients with systemic lupus erythematosus(SLE),two patients with primary Sjogren's syndrome(pSS),were treated with intravenous infusion of TCZ at a dose of 8 mg/kg of body weight once a month for at least 7 months.The annual relapse rate(ARR)of NMOSD,expanded disability status scale(EDSS)score,serum AQP4-IgG titer,peripheral blood B cell level,and CTD activity were observed in each patient during the 7-month period of TCZ treatment.Results None of the 6 patients had NMOSD relapse during TCZ treatment,ARR decreased from median(quartile)0.3(0,1.2)times/year to 0 times/year after treatment,but the difference was not statistically significant(Z=-1.826,P=0.068).EDSS score significantly decreased[from median(quartile)3.5(2.5,4.7)points to 2.0(0.8,3.1)points,Z=-2.220,P=0.026],serum AQP4-IgG titer significantly decreased[from median(quartiles)1∶100(1∶83,1∶320)to 1∶21(0,1∶49),Z=-2.201,P=0.028],and peripheral blood B cell counts significantly decreased[from a median(quartile)of 116(75,201)cells/L to 37(19,97)cells/L,Z=-2.201,P=0.028].CTD maintained clinically stable during the treatment.Leukocyte and neutrophil counts were observed severely reduced in one patient,which recovered after treatment with medications.All patients did not experience adverse reactions such as infections.Conclusions TCZ has a good efficacy and safety in treatment with NMOSD with CTD.
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