Background/Purpose: Takayasu arteritis (TAK) is a large vessel vasculitis involving the aorta and its major branches in patients younger than 40 years. Glucocorticoids (GCs) are the mainstay of treatment for TAK, but relapses and GC dependence are seen in more than two-thirds of patients. Increasing evidence supports a role for B cells in the pathogenesis of TAK. Circulating plasmablasts and memory B cells are increased, while naive B cells are decreased in patients with active TAK as compared with inactive and control patients [1]. These findings suggest a potential role for B cell depleting therapy in TAK. Our aim was to assess the efficacy of Rituximab (RTX) in a single center series of patients with TAK.

Methods: We conducted a prospective, single center, open-label study on 5 TAK patients treated with RTX. All patients satisfied the American college of Rheumatology classification criteria for TAK. Four of the 5 patients had a refractory disease and had received high dose GCs and synthetic and/or biological immunosuppressive (IS) agents before RTX. One new diagnosed, treatment naïve TAK patient refused GCs and received RTX in monotherapy. RTX was administered according to rheumatoid arthritis scheme (2 infusions of 1.000 mg, 15 days apart). Clinical evaluation, laboratory tests (full blood count, ESR, CRP) and imaging modalities (CTA or MRA, and PET/CT) were performed at first RTX administration and every 6 months thereafter. Disease activity was assessed using Kerr index. Radiographic disease progression was defined as new or worsening lesions at follow-up CTA or MRA. PET/CT was considered positive for active disease if two or more large vessels showed grade 2 FDG uptake or higher.

Results: Five patients (4 female) were included in the study. Mean (SD) age was 30.4 (17.4) years. At first RTX administration, all patientshad active disease according to Kerr index (≥2), and had evidence of active disease at PET/CT. Table 1 summarizes the main results of our study. Despite RTX treatment, 4 of the 5 patients had evidence of persistent disease activity and/or radiographic disease progression at follow-up CTA or MRA. Only one patient experienced long-term remission (30 months to date) after two courses of RTX.

Conclusion: Our data do not support a role for RTX in refractory TAK patients. References: [1] Hoyer BF et al. Takayasu arteritis is characterised by disturbances of B cell homeostasis and responds to B cell depletion therapy with rituximab. Ann Rheum Dis 2012;71:75–9. Table 1.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/rituximab-in-patients-with-takayasu-arteritis-a-single-center-experience-on-five-patients/