Unmet needs and evidence gaps in hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis

Wechsler, Michael E.; Hellmich, Bernhard; Cid, María C.; Jayne, David; Tian, Xinping; Baylis, Lee; Roufosse, Florence

doi:10.1016/j.jaci.2023.03.011

Cited by 12 publications

(8 citation statements)

References 87 publications

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“… 1 Thus in some cases unambiguous differentiation from hypereosinophilic syndrome may not be possible. 33 ANCA-associated vasculitis classification criteria are only applicable when small-vessel vasculitis has already been demonstrated. 34 Further, different phenotypes of EGPA have been described, differentiating ANCA-positive patients with more vasculitic manifestations from ANCA-negative patients: glomerulonephritis, peripheral neuropathy, and skin purpura are more frequent in ANCA-positive patients, while lung infiltrates and cardiomyopathy are more frequent in ANCA-negative patients.…”

Section: Discussionmentioning

confidence: 99%

Benralizumab Reduces Respiratory Exacerbations and Oral Glucocorticosteroid Dose in Patients with Severe Asthma and Eosinophilic Granulomatosis with Polyangiitis

Mümmler,

Mertsch,

Barnikel

et al. 2024

JAA

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Section: Discussionmentioning

confidence: 99%

Benralizumab Reduces Respiratory Exacerbations and Oral Glucocorticosteroid Dose in Patients with Severe Asthma and Eosinophilic Granulomatosis with Polyangiitis

Mümmler,

Mertsch,

Barnikel

et al. 2024

JAA

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“…Furthermore, four-month eosinophil monitoring can be included in the six-month evaluation period suggested by the GINA guidelines [33,51] for the diagnosis of SBA. Finally, our model could be also used for other diseases related to eosinophilia-for example, in hypereosinophilic syndrome [52][53][54] and eosinophilic esophagitis [55]-to evaluate the trend of eosinophils in response to several factors and therapy.…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Patterns in Patients with Seasonal Allergy Affected by Bronchial Asthma and Rhinitis/Rhinosinusitis: Efficacy of Benralizumab in Patients with the Persistent Pattern

D’Aiuto,

Mormile,

Granata

et al. 2024

JCM

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Background: Eosinophilia can be influenced by multiple factors. This study aims to set a protocol for monitoring blood absolute eosinophil count (AEC) in patients with seasonal allergy affected by bronchial asthma (BA), allergic rhinitis (AR), or chronic rhinosinusitis with or without nasal polyposis (CRSw/sNP). Methods: We planned a total of four annual blood samples to measure AEC in- and out-seasonal pollen exposure (i.e., one measurement every three months for one year). Results: We identified two distinct groups of patients (non-eosinophilic and eosinophilic). Patients in the eosinophilic group presented with four different patterns (episodic, transient, floating, and persistent). Most patients with episodic, transient, and floating patterns were affected by mild allergy and the increase in eosinophils was related to allergen exposure. In contrast, patients with the persistent pattern mostly presented with more severe allergy (i.e., severe BA and relapsing CRSwNP) and the eosinophilia was unrelated to allergen exposure. The subgroup of patients with severe BA, relapsing CRSwNP, and persistent eosinophilc pattern were treated with benralizumab, which induced a noteworthy improvement in both severe BA and CRSwNP. Conclusions: Multiple AEC measurements in patients with seasonal allergy can better reflect patient’s eosinophilic status and help define the relationship of AEC enhancement with allergen exposure.

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“…For example, an ongoing mepolizumab post-marketing surveillance study in Japan reported improved clinical symptoms, blood eosinophil count and OCS use by week 48 compared with baseline levels [ 33 ]. Despite recent advances in treatment options for patient with EGPA, unmet needs remain and future research should include a focus on identifying predictors of response to mepolizumab, delineating the optimal combinations of therapies to improve outcomes, and quantifying the effects of mepolizumab treatment on health-related quality of life [ 34 – 36 ].…”

Section: Discussionmentioning

confidence: 99%

Mepolizumab has clinical benefits including oral corticosteroid sparing irrespective of baseline EGPA characteristics

Jayne,

Terrier,

Hellmich

et al. 2023

ERJ Open Res

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BackgroundThe MIRRA trial (GSK ID: 115921/NCT02020889) demonstrated that mepolizumab increased remission time and reduced oral corticosteroid (OCS) use compared with placebo in patients with relapsing or refractory eosinophilic granulomatosis with polyangiitis (EGPA). The present analysis investigated the impact of baseline characteristics on clinical outcomes and characterised the OCS-sparing effect of mepolizumab.MethodsIn a Phase III, randomised controlled trial for patients with EGPA (MIRRA), patients received standard of care plus mepolizumab 300 mg or placebo every 4 weeks for 52 weeks. The accrued duration of remission, the proportion of patients in remission at Weeks 36 and 48, and the proportion of patients with clinical benefit (remission, OCS, or relapse-related) were assessed according to baseline EGPA characteristic subgroups (post hoc). Mepolizumab-related OCS-sparing benefits were also quantified.ResultsAccrued duration of remission and the proportion of patients in remission at Weeks 36 and 48 were greater with mepolizumab than placebo across baseline subgroups: refractory disease, immunosuppressant use, EGPA duration, relapse number, and OCS use ≤20 mg·day−1. The proportion of patients with clinical benefit was greater with mepolizumabversusplacebo (range: 76–81%versus25–39%), irrespective of immunosuppressant use or EGPA duration. Patients treated with mepolizumabversusplacebo accrued significantly more weeks on OCS ≤4 mg·day−1(odds ratio [95% confidence interval]: 5.06 [2.47,10.38]) and had a mean of 1423.1 mg less per-patient OCS exposure over 52 weeks.ConclusionsMepolizumab treatment provided benefits to patients with EGPA across varying baseline clinical characteristics and can be considered an OCS-sparing treatment in EGPA.

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Unmet needs and evidence gaps in hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis

Cited by 12 publications

References 87 publications

Benralizumab Reduces Respiratory Exacerbations and Oral Glucocorticosteroid Dose in Patients with Severe Asthma and Eosinophilic Granulomatosis with Polyangiitis

Benralizumab Reduces Respiratory Exacerbations and Oral Glucocorticosteroid Dose in Patients with Severe Asthma and Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic Patterns in Patients with Seasonal Allergy Affected by Bronchial Asthma and Rhinitis/Rhinosinusitis: Efficacy of Benralizumab in Patients with the Persistent Pattern

Mepolizumab has clinical benefits including oral corticosteroid sparing irrespective of baseline EGPA characteristics

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