Benralizumab with corticosteroids for treating relapsing or refractory eosinophilic granulomatosis with polyangiitis [ID6266]

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune disease that causes inflammation of the blood vessels.

It is characterised by high levels of white blood cells called eosinophils and mainly affects small to medium-sized blood vessels. Asthma is one of the key features of EGPA. Asthma may begin many years before any other symptoms. Most people with EGPA also have upper airway involvement.

Later symptoms may include rashes joint pain and swelling peripheral neuropathy abdominal pain diarrhoea shortness of breath arrhythmia presence of red blood cells in urine chest pain and heart failure.EGPA affects around 1 in 22000 people in the UK.(1) Based on this estimated prevalence there are around 2600 people in England with EGPA.(2) In the first 12 months following an EGPA diagnosis around 19% of people had an inpatient stay in hospital related to the condition.(1) Over 50% of people experience a relapse after treatment within 5-years.(3)The aim of treatment is initially to induce remission then to maintain remission and treat relapse when necessary.

Without treatment life-threatening complications may develop and the condition can be fatal.

Clinical guidelines recommend that the Five Factor Score which predicts the risk of disease progression and mortality in people with EGPA is used to inform treatment decisions.(4) Corticosteroids are the mainstay of treatment.(4) Initially high-dose oral corticosteroid with or without intravenous corticosteroid is used to induce remission.

In addition inhaled and nasal corticosteroids are used for asthma and nasal symptoms. Corticosteroids can be slowly tapered over several months. In some people cyclophosphamide can be used to induce remission. Rituximab may be used to induce remission when cyclophosphamide is unsuitable. Maintenance treatments include oral corticosteroids and immunosuppressive agents such as methotrexate and azathioprine.

There is limited evidence for the efficacy of these immunosuppressive treatments in people with EGPA and they are not specifically licensed in this indication.References:1. Hwee J. Harper L. Fu Q. Nirantharakumar K. Mu G. & Jakes R. W. (2024). Prevalence incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the United Kingdom.

ERJ Open Research.2.

Office for National Statistics (ONS) released 23 November 2023 ONS website statistical bulletin?Population estimates for England and Wales: mid-20223. Smith R. M. et al. (2020). Rituximab as therapy to induce remission after relapse in ANCA associated vasculitis. Annals of the rheumatic diseases 79(9) 1243-1249.4 Emmi G. et al (2023).

Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nature reviews Rheumatology 19(6) 378-393.