New biomarkers for early diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA)

Latorre, Manuela; Bacci, Elena; Seccia, Veronica; Dente, Federico L.; Brighindi, Sara; Franco, Antonella Di; Cianchetti, Silvana; Cardini, Cristina; Baldini, Chiara; Paggiaro, Pierluigi

doi:10.1183/1393003.congress-2017.pa3560

Cited by 2 publications

(5 citation statements)

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“…Most of these results are consistent with those from previous studies showing significant and prompt efficacy of omalizumab for treating the symptoms of asthma and corticosteroid sparing . However, few case reports have described severe disease exacerbations following omalizumab initiation .…”

Section: Discussionsupporting

confidence: 90%

“…In the literature, data for EGPA are rare and conflicting. Some case reports suggest that omalizumab might be effective in patients with active disease (21)(22)(23)(24)(25)(26)(27), but others suggest a temporal association between omalizumab treatment and the development of EGPA (28)(29)(30)(31)(32)(33)(34)(35), thus raising the question of a causal relationship between anti-IgE therapy and disease onset. These findings have dramatically hindered the use of omalizumab for treatment of EGPA.…”

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confidence: 99%

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Anti‐IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss): Data on Seventeen Patients

Jachiet

Samson

Cottin

et al. 2016

Arthritis & Rheumatology

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Objective. To describe the efficacy and safety of omalizumab, an anti-IgE monoclonal antibody, in patients with refractory and/or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA).Methods. We conducted a nationwide retrospective study including EGPA patients who received omalizumab. Response was defined as the absence of asthma and/or sinonasal exacerbations with a prednisone dosage of £7.5 mg/day (complete response) or >7.5 mg/day (partial response).Results. Seventeen patients (median age 45 years) received omalizumab for severe steroiddependent asthma (88%) and/or sinonasal involvement (18%). After a median follow-up of 22 months, 6 patients (35%) achieved a complete response, 5 patients (30%) achieved a partial response, and 6 patients (35%) had no improvement. The median Birmingham Vasculitis Activity Score decreased from 2.5 at baseline to 0.5 at 12 months. The median number of exacerbations per month decreased from 1 at baseline to 0 at 12 months, and the median forced expiratory volume in 1 second increased from 63% of the percent predicted at baseline to 85% of the percent predicted at 12 months. The median prednisone dosage decreased from 16 mg/day at baseline to 11 mg/day at 6 months and 9 mg/day at 12 months. Omalizumab was discontinued in 8 patients

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Section: Discussionsupporting

confidence: 90%

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confidence: 99%

Anti‐IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss): Data on Seventeen Patients

Jachiet

Samson

Cottin

et al. 2016

Arthritis & Rheumatology

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“…In the current analysis, remission benefits with mepolizumab over placebo were observed in patients with and without features of a vasculitic phenotype including those with and without a history of ANCA positivity. This suggests that, although ANCA is often associated with the vasculitic EGPA phenotype (5,6) and may directly contribute to disease pathology (6,33), mepolizumab can facilitate remission via targeting eosinophils and their contribution to disease activity (4,6). Accordingly, ANCA binding level does not always correlate with disease severity, ANCA can persist in disease remission, and some patients with vasculitic manifestations may test ANCA negative (34)(35)(36).…”

Section: Discussionmentioning

confidence: 99%

“…These characteristics often contribute to organ injury, impairment, and life-threatening conditions (3,4). EGPA can be divided into predominantly vasculitic or eosinophilic phenotypes based on clusters of often overlapping clinical features (5,6). Common features of the vasculitic phenotype include the presence of palpable purpura, peripheral neuropathy, and glomerulonephritis (6).…”

Section: Introductionmentioning

confidence: 99%

“…Common features of the vasculitic phenotype include the presence of palpable purpura, peripheral neuropathy, and glomerulonephritis (6). In contrast, the eosinophilic phenotype is more frequently characterized by lung infiltrates and cardiomyopathy and is often associated with elevated blood and tissue eosinophil counts (5,6). As some vasculitisassociated manifestations such as glomerulonephritis or palpable purpura occur more frequently in patients with a positive antineutrophil cytoplasmic antibody (ANCA) status, the vasculitic and eosinophilic phenotypes can be often inferred by ANCA status (5,6).…”

Section: Introductionmentioning

confidence: 99%

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Clinical Benefit of Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis for Patients With and Without a Vasculitic Phenotype

Terrier

Jayne

Hellmich³

et al. 2023

ACR Open Rheumatology

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Objective To evaluate mepolizumab's efficacy in eosinophilic granulomatosis with polyangiitis (EGPA) with and without a vasculitic phenotype. Methods The MIRRA study (NCT02020889/GSK ID: 115921) included adults with relapsing/refractory EGPA and 4 or more weeks of stable oral glucocorticoids (OG). Patients received mepolizumab (300 mg subcutaneously every 4 weeks) or placebo, plus standard of care for 52 weeks. This post hoc analysis assessed EGPA vasculitic phenotype using antineutrophil cytoplasmic antibody (ANCA) history, baseline Birmingham Vasculitis Activity Score (BVAS), and Vasculitis Damage Index (VDI) score. Coprimary endpoints included accrued remission over 52 weeks and proportion in remission at Week 36 and Week 48. Remission was defined as a BVAS equal to 0 and an OG dose of 4 or more mg/day of a prednisone equivalent. Types of relapses (vasculitis, asthma, and sino‐nasal) and EGPA vasculitic characteristics (by study remission status) were also assessed. Results A total of 136 patients were included (n = 68, mepolizumab and placebo). Irrespective of history of ANCA positivity status, baseline BVAS, or baseline VDI, the accrued remission duration and the proportion of patients in remission at Weeks 36 and 48 were greater with mepolizumab compared with placebo. With mepolizumab, remission at both Week 36 and Week 48 was achieved by 54% of patients with and 27% of patients without a history of ANCA positivity compared with 0% and 4%, respectively (placebo); 45% of patients with a BVAS of 0 and 22% of patients with BVAS of greater than 0 compared with 5% and 2%, respectively (placebo); and 29% of patients with a VDI score of less than 5 and 37% of patients with a VDI score of 5 or more compared with 6% and 0%, respectively (placebo). Mepolizumab reduced all types of relapses as compared with placebo. Baseline vasculitic characteristics (neuropathy, glomerulonephritis, alveolar hemorrhage, palpable purpura, and ANCA positivity) were generally similar among patients with and without remission. Conclusion Mepolizumab is associated with clinical benefits for patients with and without a vasculitic EGPA phenotype.

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New biomarkers for early diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA)

Cited by 2 publications

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Anti‐IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss): Data on Seventeen Patients

Anti‐IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss): Data on Seventeen Patients

Clinical Benefit of Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis for Patients With and Without a Vasculitic Phenotype

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