Abstract

Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune disease affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens. We report a case of a 57-year-old female patient presenting with hemoptysis, sinusitis, and conjunctivitis. Based on lung biopsy, the diagnosis of antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) was established. She was put on rituximab as induction and maintenance therapy. She responded initially to rituximab as induction therapy but failed to respond in the maintenance course of the drug. Rituximab was stopped and mycophenolate mofetil was administered. She responded as laboratory c-ANCA titers turned negative and symptoms subsided. There are no randomized clinical trials addressing rituximab effect in induction and remission at the same time. This case report doubts the efficacy of the use of rituximab therapy for both induction and maintenance of remission at the same time, waiting for the results of the ongoing trials.

Highlights

  • Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune syndrome affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens [1]

  • We report a case of ANCA-associated vasculitis where rituximab was used for both induction and maintenance of remission

  • There are many studies that suggest the use of rituximab as an induction or maintenance therapy, there is a shortage of data about the use of the drug for both targets at the same time for the selected patients and many studies recommend further research

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Summary

Introduction

Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune syndrome affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens [1]. Cyclophosphamide and glucocorticoids have been the standard of remission induction therapy for severe AAV for 40 years [2]. These are associated with high morbidity rates and adverse effects such as infections, cancer, and infertility [1]. Its use allows reducing the exposure to cyclophosphamide and maintenance immunosuppression [1] It has been used in AAV for induction of remission as in RAVE and RITUXVAS trials, and in the maintenance as in MAINRITSAN trial [1, 3, 4]. We report a case of ANCA-associated vasculitis where rituximab was used for both induction and maintenance of remission

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