Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis
Introduction Eosinophilic granulomatosis with polyangiitis (EGPA) is a subset of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis with distinct pathophysiological mechanisms, clinical features and treatment responses. Rituximab is a licensed therapy for granulomatosis with polyangi...
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BMJ Publishing Group
2019-06-01
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| author | David Jayne Rachel B Jones Vítor Teixeira Aladdin J Mohammad Rona Smith |
| author_facet | David Jayne Rachel B Jones Vítor Teixeira Aladdin J Mohammad Rona Smith |
| author_sort | David Jayne |
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| description | Introduction Eosinophilic granulomatosis with polyangiitis (EGPA) is a subset of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis with distinct pathophysiological mechanisms, clinical features and treatment responses. Rituximab is a licensed therapy for granulomatosis with polyangiitis and microscopic polyangiitis but there is limited experience of rituximab in EGPA.Methods EGPA patients from a tertiary centre who received rituximab for mostly refractory EGPA or in whom cyclophosphamide was contra indicated were studied. A standardised dataset was collected at time of initial treatment and every 3 months for 24 months. Response was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 and partial response as ≥50% reduction in BVAS from baseline. Remission was defined as a BVAS of 0 on prednisolone dose ≤5 mg.Results Sixty-nine patients (44 female) received rituximab between 2003 and 2017. Improvement (response and partial response) was observed in 76.8% of patients at 6 months, 82.8% at 12 months and in 93.2% by 24 months, while relapses occurred in 54% by 24 months, with asthma being the most frequent manifestation. The median BVAS decreased from 6 at baseline to 1 at 6 months, and 0 at 12 and 24 months. Prednisolone dose (mg/day, median) decreased from 12.5 to 7, 7.5 and 5 at 6, 12 and 24 months, respectively. ANCA positive patients had a longer asthma/ear, nose and throat (ENT) relapse-free survival time and a shorter time to remission.Discussion Rituximab demonstrated some efficacy in EGPA and led to a reduction in prednisolone requirement, but asthma and ENT relapse rates were high despite continued treatment. The ANCA positive subset appeared to have a more sustained response on isolated asthma/ENT exacerbations. |
| format | Article |
| id | doaj-art-f08a2bbfb59649b3a63bfe87c28b6852 |
| institution | Kabale University |
| issn | 2056-5933 |
| language | English |
| publishDate | 2019-06-01 |
| publisher | BMJ Publishing Group |
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| series | RMD Open |
| spelling | doaj-art-f08a2bbfb59649b3a63bfe87c28b68522025-08-20T03:25:23ZengBMJ Publishing GroupRMD Open2056-59332019-06-015110.1136/rmdopen-2019-000905Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitisDavid Jayne0Rachel B Jones1Vítor Teixeira2Aladdin J Mohammad3Rona Smith4Vasculitis and Lupus Clinic, Addenbrooke`s Hospital, Cambridge, UKVasculitis and Lupus Clinic, Addenbrooke`s Hospital, Cambridge, UKVasculitis and Lupus Clinic, Addenbrooke`s Hospital, Cambridge, UKVasculitis and Lupus Clinic, Addenbrooke`s Hospital, Cambridge, UKVasculitis and Lupus Clinic, Addenbrooke`s Hospital, Cambridge, UKIntroduction Eosinophilic granulomatosis with polyangiitis (EGPA) is a subset of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis with distinct pathophysiological mechanisms, clinical features and treatment responses. Rituximab is a licensed therapy for granulomatosis with polyangiitis and microscopic polyangiitis but there is limited experience of rituximab in EGPA.Methods EGPA patients from a tertiary centre who received rituximab for mostly refractory EGPA or in whom cyclophosphamide was contra indicated were studied. A standardised dataset was collected at time of initial treatment and every 3 months for 24 months. Response was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 and partial response as ≥50% reduction in BVAS from baseline. Remission was defined as a BVAS of 0 on prednisolone dose ≤5 mg.Results Sixty-nine patients (44 female) received rituximab between 2003 and 2017. Improvement (response and partial response) was observed in 76.8% of patients at 6 months, 82.8% at 12 months and in 93.2% by 24 months, while relapses occurred in 54% by 24 months, with asthma being the most frequent manifestation. The median BVAS decreased from 6 at baseline to 1 at 6 months, and 0 at 12 and 24 months. Prednisolone dose (mg/day, median) decreased from 12.5 to 7, 7.5 and 5 at 6, 12 and 24 months, respectively. ANCA positive patients had a longer asthma/ear, nose and throat (ENT) relapse-free survival time and a shorter time to remission.Discussion Rituximab demonstrated some efficacy in EGPA and led to a reduction in prednisolone requirement, but asthma and ENT relapse rates were high despite continued treatment. The ANCA positive subset appeared to have a more sustained response on isolated asthma/ENT exacerbations.https://rmdopen.bmj.com/content/5/1/e000905.full |
| spellingShingle | David Jayne Rachel B Jones Vítor Teixeira Aladdin J Mohammad Rona Smith Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis RMD Open |
| title | Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis |
| title_full | Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis |
| title_fullStr | Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis |
| title_full_unstemmed | Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis |
| title_short | Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis |
| title_sort | efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis |
| url | https://rmdopen.bmj.com/content/5/1/e000905.full |
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