Rituximab for Maintenance of Remission in ANCA-Associated Vasculitis

Expert Consensus Guidelines -- Executive Summary

Joanna Tieu; Rona Smith; Neil Basu; Paul Brogan; David D'Cruz; Neeraj Dhaun; Oliver Flossmann; Lorraine Harper; Rachel B. Jones; Peter C. Lanyon; Raashid A. Luqmani; Stephen P. McAdoo; Chetan Mukhtyar; Fiona A. Pearce; Charles D. Pusey; Joanna C. Robson; Alan D. Salama; Lucy Smyth; Richard A. Watts; Lisa C. Willcocks; David R. W. Jayne


Rheumatology. 2020;59(4):727-731. 

In This Article


Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Considerable improvements in therapy mean induction of remission occurs in most patients with AAV.[1–4] However, disease relapse continues to pose a burden to patients. Morbidity accrues with relapses through disease-related damage and adverse effects of therapies to manage these relapses, negatively impacting on quality of life.[5]

Rituximab (RTX), a monoclonal antibody targeting CD20, leads to peripheral B cell depletion. This has been successfully trialled, and is licensed, for the induction and maintenance of remission in AAV.[2,3] RTX is increasingly being used for the maintenance of remission in patients with AAV, to reduce the risk of relapse and its consequences.[6] Other commonly used agents that have been trialled for the maintenance of remission in AAV include azathioprine, methotrexate and mycophenolate.[7–9] The decision to select RTX for the maintenance of remission is multifactorial, including but not limited to, patient-related factors and preferences, previous treatment and response, consideration of the overall risk of relapse, and access to therapy. These guidelines have been developed by a group of physicians practising in the UK.

Whilst guidelines on the management of AAV have proposed RTX as a treatment option in remission maintenance, there has been limited guidance on how this should be used.[10,11] We present guidelines developed through a modified Delphi exercise on the use of RTX in the maintenance of remission in adult AAV patients, with additional focus on adjunct therapies, adverse effects and use of prophylaxis. These guidelines can be used to assist specialty physicians making treatment decisions in patients with AAV when RTX has been chosen for remission maintenance.