Tsvetelina Yoneva1, Yana Zdravkova1, Georgi Kotov1, Ekaterina Ivanova Todorova2, Georgi Vasilev2, Rasho Rashkov1, Ivan Sheytanov1

1Clinic of Rheumatology, University Hospital St. Ivan Rilski, Sofia, Bulgaria
2Laboratory of Clinical Immunology, University Hospital St. Ivan Rilski, Sofia, Bulgaria

Keywords: Antineutrophil cytoplasmic antibodies, rituximab, vasculitis.


Objectives: This study aimed to analyze a group of patients with severe and refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) managed with rituximab and to report on treatment outcomes.

Patients and methods: A total of 78 patients (41 females, 37 males; mean age: 50.1±13.4 years; range, 18 to 76 years) with AAV on rituximab treatment were included in the single-center, retrospective study conducted between 2009 and 2018. The diagnosis was established based on the 1990 classification criteria of the American College of Rheumatology and the definitions of vasculitis of Chapel Hill Consensus Conference. Laboratory and immunological tests were conducted. Disease activity was determined through the Birmingham Vasculitis Activity Score.

Results: Rituximab was preferred over cyclophosphamide in 37 patients and used as a second-line therapy after cyclophosphamide in 41 cases. Rituximab treatment showed favorable outcomes with regard to serum creatinine levels, proteinuria, and hematuria, as well as in cases of isolated lung involvement. Nearly half of patients with pulmonary renal syndrome also improved, with 22.2% achieving remission. ANCAs were positive in 85.9% of patients at the onset of rituximab treatment and became negative in 82% of the positive cases. Adverse events were rare and included infusion reactions (one case of reactivation of a herpes zoster infection and one case of allergic reaction).

Conclusion: Rituximab is an efficient and safe therapeutic option in patients with AAV who are difficult to treat, have insufficient response, or have not tolerated other treatments.

Citation: Yoneva T, Zdravkova Y, Kotov G, Todorova EI, Vasilev G, Rashkov R, et al. Analysis of treatment outcomes in patients with antineutrophil cytoplasmic antibody-associated vasculitis on rituximab therapy. Arch Rheumatol 2023;38(3):397-405.

Ethics Committee Approval

The study protocol was approved by the University Hospital ‘St. Ivan Rilski’ Ethics Committee (date 14.03.2019, no: 29). The study was conducted in accordance with the principles of the Declaration of Helsinki.

Author Contributions

Idea/concept: R.R.; Design: T.Y., R.R., I.S.; Control/supervision: I.S.; Data collection and/or processing: T.Y., Y.Z.; Analysis and/or interpretation: T.Y., Y.Z., E.I.T., G.V.; Literature review, writing the article: T.Y., G.K.; Critical Review: R.R., I.S.; References and fundings: T.Y.; Materials: T.Y., E.I.T., G.V.

Conflict of Interest

The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Financial Disclosure

The authors received no financial support for the research and/or authorship of this article.

Data Sharing Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.