Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis
(2019) In RMD Open 5(1).- Abstract
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... (More)
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.
(Less)
- author
- Teixeira, Vitor ; Mohammad, Aladdin J. LU ; Jones, Rachel B. ; Smith, Rona and Jayne, David
- organization
- publishing date
- 2019
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- ANCA, asthma, eosinophilic granulomatosis with polyangiitis, rituximab, systemic vasculitis
- in
- RMD Open
- volume
- 5
- issue
- 1
- article number
- e000905
- publisher
- BMJ Publishing Group
- external identifiers
-
- scopus:85067033226
- pmid:31245051
- ISSN
- 2056-5933
- DOI
- 10.1136/rmdopen-2019-000905
- language
- English
- LU publication?
- yes
- id
- 829223ea-2f86-4041-99be-e43366d91819
- date added to LUP
- 2019-07-01 08:59:13
- date last changed
- 2024-04-16 13:22:27
@article{829223ea-2f86-4041-99be-e43366d91819,
abstract = {{<p>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.</p>}},
author = {{Teixeira, Vitor and Mohammad, Aladdin J. and Jones, Rachel B. and Smith, Rona and Jayne, David}},
issn = {{2056-5933}},
keywords = {{ANCA; asthma; eosinophilic granulomatosis with polyangiitis; rituximab; systemic vasculitis}},
language = {{eng}},
number = {{1}},
publisher = {{BMJ Publishing Group}},
series = {{RMD Open}},
title = {{Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis}},
url = {{http://dx.doi.org/10.1136/rmdopen-2019-000905}},
doi = {{10.1136/rmdopen-2019-000905}},
volume = {{5}},
year = {{2019}},
}