Background/Purpose:

Rheumatoid vasculitis (RV) is a rare systemic inflammatory process affecting small to medium sized blood vessels in patients with rheumatoid arthritis (RA). RV confers high morbidity and mortality, and evidence regarding safe, effective treatment options is lacking. Our study aims to characterize the indication, outcomes and adverse effects of rituximab treatment in a large single center cohort of RV patients.

Methods:

Patients evaluated with systemic RV from 2000 to 2017 who were treated with rituximab were retrospectively identified through direct medical chart review. Patients who received rituximab for an indication other than RV, were treated at an outside institution, or for whom follow-up information was unavailable were excluded. Clinical characteristics, treatment, outcomes and adverse effects were analyzed.

Results:

17 patients with RV who were treated with rituximab were identified. Mean age at RV diagnosis was 59.2 (range:38-77), 59% were female, 94% were Caucasian and 76% had positive rheumatoid factor (RF). At time of initiating rituximab therapy, the median BVAS/RA was 4.0 (IQR, 2.0-7.5). RV presented in the skin in 9 patients (53%, ulcers and/or leukocytoclastic vasculitis), as mononeuritis multiplex in 2 (12%), inflammatory ocular disease in 2 (12%), and affected multiple organ systems in 4 (24%).

Rituximab was used as first-line induction therapy in 8 (47%) patients, first-line for relapse in 4 (24%), second-line in 2 (12%), and third- or fourth-line or in combination with another agent in 3 (18%). 15 patients had follow-up information recorded at 3 and 6 months from rituximab use. At 3 months, 2 (13%) had achieved complete remission (CR), and 10 (67%) had achieved partial remission (PR). At 6 months, 6 patients (40%) had achieved CR, 8 (53%) had achieved PR, and 1 patient had no response (NR). 8 of 13 patients with available records (62%) had CR at 12 months, and 5 patients (38%) had PR.

There were no significant differences in age, sex, smoking status, BMI, duration of RA, baseline RA characteristics, DAS28-CRP(3) or BVAS-RA baseline scores, RA treatments prior to development of RV, or type of RV presentation when compared between groups who did and did not achieve complete remission at 6 months.

10 (59%) of 17 patients experienced no adverse effects, 4 (24%) of 17 experienced mild adverse effects (infusion reaction, rash, infection requiring oral antibiotics) and 3 (18%) of 17 required hospitalization; 2 with infection requiring IV antibiotics and 1 with pneumocystis pneumonia. There were no deaths as a result of rituximab use.

Conclusion:

Systemic RV is difficult to treat effectively. Complete remission of RV was achieved in 62% of patients and partial response in 38% within 12 months of rituximab use. Rituximab represents an effective treatment option for RV with an acceptable side effect profile. Further evidence is needed to inform treatment for patients with RV.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/rituximab-therapy-for-systemic-rheumatoid-vasculitis-indications-outcomes-and-adverse-events/