Background/Purpose:

Takayasu arteritis (TA) is a rare chronic-relapsing vasculitis involving primarily the aorta and its major branches.TA is associated with considerable morbidity and mortality. Therapy is based on corticosteroids (CS) but steroid-sparing immunosuppressive drugs are required in most patients to minimize CS adverse events and to control progressive vascular disease. However, about 25% of patients relapse when CS are tapered. In this setting, previous works showed that tocilizumab (TCZ), an humanized anti-IL6 receptor antibody may be useful.

Objectives

To evaluate the safety and efficacy of TCZ in the treatment of refractory TA.

Methods:

We retrospectively studied 7 TA patients (pts) treated with TCZ (8 mg/kg monthly) between 2010 and 2012 at a single academic Italian center. All pts satisfied ACR criteria for TA classification and had active refractory TA. Treatment efficacy was evaluated as: i) reduction of signs and symptoms of active disease, ii) steroid sparing activity (assessed as reduction in the average daily dose measured within the 12 month period preceding each medical evaluation, iii) angio-MRI assessment of vascular lesions evolution, iv) decrease in CRP and ESR.

Results:

All 7 pts were female, with a median age at the beginning of TCZ therapy of  35 years (range 32-46), median duration of disease 66 months (range 17-101). Before TCZ therapy, they were taking a median of 4 (range 1-8) immunosuppressive agents. Four pts had been previously treated with anti-TNF agents. Median FU on TCZ therapy was 14 months (range 9-24). Mean duration of CS therapy before TCZ  was 37 months. Two pts did not show signs or symptoms of active disease during FU while 3 pts satisfied NIH criteria of active disease. During FU, average prednisone daily dose decreased from a median value of 8.3 mg (range 5.9–29) to 8.0 mg (range 5.0-16): however, the dose could be reduced more than 3 mg/day in 4 patients. The median number of vascular lesions was unchanged (8, range 4-12) at baseline and at the end of FU. In one pt vascular lesions improved during FU and in another did not progress, while in the other 5 pts there was worsening of at least one vascular lesion. Median values of ESR and CRP decreased from 34 (range 8.0-76) to 4.0 (range 2.0-45) mm/h and from 13 (range 10-35) to 2.0 (range 1.0-44) mg/l, respectively. TCZ was stopped in 4 pts because of suboptimal disease control. During FU one pt had severe pneumonia, requiring TCZ interruption, another had relapsing upper respiratory infections and a third developed pytiriasis rosea, that subsided after TCZ interruption.

Conclusion:

In this study of refractory TA, TCZ showed efficacy only in a minority of pts. Our data do not confirm the positive results of TCZ therapy reported in previous studies . However, our pts may have had more severe disease, as suggested by the higher number of immunosuppressive agents at baseline compared to previous reports. Finally, it should be noted that ESR and CRP do not appear to correlate reliably with disease activity during TCZ therapy. Further studies are necessary to  better define the role of TCZ in TA therapy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/tocilizumab-in-refractory-takayasus-arteritis-7-patients-followed-at-a-single-italian-centre/