New Treatment Strategies in Large-vessel Vasculitis

Sebastian Unizony; John H. Stone; James R. Stone


Curr Opin Rheumatol. 2013;25(1):3-9. 

In This Article

Rituximab and Tocilizumab With Other Large-vessel Vasculitides

IgG4-related disease (IgG4-RD) is a disorder characterized by elevated serum IgG4 levels and the infiltration of tissue by increased numbers of IgG4-expressing plasma cells along with fibrosis. Although almost any organ may be involved, IgG4-RD involvement of the aorta results in a lymphoplasmacytic aortitis or periaortitis.[54] Khosroshahi et al.[55] recently reported treatment with rituximab in 10 patients with IgG4-RD, including three patients with aortitis. All three patients showed a positive response to rituximab as evidenced by improvement in clinical signs and symptoms, and decreases in acute phase reactants and serum IgG4 levels. Prednisone treatment, which ranged from 10 to 60 mg/day before rituximab, could be discontinued in all three patients following treatment with rituximab. However, approximately 6 months following the initial course of rituximab, two of these patients experienced disease recurrences, characterized by worsening clinical symptoms and elevation of serum IgG4 levels, that responded to additional courses of BCDT.

Relapsing polychondritis is characterized by destructive inflammation targeting cartilaginous tissues, with aortic involvement in some cases. TNF-α blockers have been shown to have some benefit in a small number of these patients with refractory disease.[8] Narshi and Allard[56] described a patient with refractory relapsing polychondritis with aortitis who failed TNF-α blockade, but responded to tocilizumab administered at 8 mg/kg per month. As with GCA and TAK, administration of tocilizumab resulted in marked reduction in the CRP level and allowed a substantial decrease in the glucocorticoid dose. The effect of tocilizumab treatment on the aortic inflammation in this patient was not reported. Recently, Shibuya et al.[57] reported a patient with refractory Cogan's syndrome with involvement of the ascending aorta by PET-CT, who had failed TNF-α blockade, but responded to treatment with tocilizumab 8 mg/kg every 4 weeks. Six months of therapy resulted in clinical improvement, and (not surprisingly) normalization of CRP, but no decrease in aortic 18-fluordeoxyglucose uptake by PET-CT was seen.