Two pediatric cases of ANCA-negative eosinophilic granulomatosis with polyangiitis successfully treated with dupilumab

Galant‐Swafford, Jessica; Geng, Bob; Leibel, Sydney; Akuthota, Praveen; Tucker, Suzanne; Cernelc-Kohan, Matejka; Sheets, Robert; Nation, Javan; Jefferson, Akilah A.

doi:10.1016/j.jaip.2020.06.052

Cited by 14 publications

(8 citation statements)

References 9 publications

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“…There have now been case reports of the use of biologics other than mepolizumab to treat vasculitis; these drugs include omalizumab [48], benralizumab [49-52], and dupilumab [53]. Omalizumab is given for asthma but not vasculitis in EGPA.…”

Section: Discussionmentioning

confidence: 99%

Clinical Features of Patients with Active Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab

Tsurikisawa

Oshikata

Watanabe

et al. 2021

Int Arch Allergy Immunol

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Background: In some patients with eosinophilic granulomatosis with polyangiitis (EGPA), remission cannot be induced, despite treatment with corticosteroids and immunosuppressants. We evaluated the clinical features of patients with EGPA in whom mepolizumab was effective. Methods: There were 59 EGPA patients treated at Hiratsuka City Hospital, Japan, between April 2018 and September 2020, and 30 of them received mepolizumab. Twenty (66.7%) experienced a “marked effect” (the daily dose of corticosteroid or immunosuppressant could be decreased, or the interval between intravenous immunoglobulin (IVIG) treatments could be prolonged) and 10 (33.3%) experienced a “weak effect” (these measures were not achieved). Eosinophil numbers, serum IgG levels, daily doses of corticosteroids and immunosuppressants, and the interval between IVIG treatments at diagnosis and before and after mepolizumab initiation were determined. Results: Eosinophil numbers at diagnosis were significantly higher in the marked-effect group than in the weak-effect group (p < 0.05) but not before mepolizumab initiation or at the last visit. Birmingham Vasculitis Activity Scores (BVASs) before mepolizumab initiation (p < 0.05) and at last visit (p < 0.01), and frequency of relapse before treatment initiation (p < 0.05) were significantly higher, and the serum IgG level before mepolizumab treatment was significantly lower in the weak-effect group than in the marked-effect group. The weak-effect group received higher doses of corticosteroids, even if the corticosteroid dose could be reduced for a while after mepolizumab initiation. Conclusion: High peripheral blood eosinophil numbers at EGPA diagnosis were suggestive of a positive clinical response to mepolizumab.

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Section: Discussionmentioning

confidence: 99%

Clinical Features of Patients with Active Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab

Tsurikisawa

Oshikata

Watanabe

et al. 2021

Int Arch Allergy Immunol

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“…Type 2 in ammation appears to play a crucial role in the pathogenesis of EGPA. Although it is speculated that dupilumab, which broadly suppresses type 2 in ammation, may induce the development of EGPA in present case, some reports had demonstrated the clinical e cacy of dupilumab for EGPA [21][22][23] . The different clinical responses to dupilumab in patients with EGPA may suggest that EGPA is a heterogeneous disease, similar to asthma and rhinosinusitis.…”

Section: Discussionmentioning

confidence: 60%

A case of eosinophilic granulomatosis with polyangiitis developed after dupilumab administration in patients with eosinophilic chronic rhinosinusitis and asthma

Suzaki

Tanaka

Yanai

et al. 2022

Preprint

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Background:Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis characterized by eosinophil-rich granulomatous inflammation and small-to-medium vessel vasculitis associated with asthma, rhinosinusitis and eosinophilia. EGPA is often difficult to distinguish from severe asthma and eosinophilic chronic rhinosinusitis in cases when there are no findings that clearly suggest vasculitis. Dupilumab, an anti-IL-4Rα monoclonal antibody, is expected to be effective in eosinophilic airway inflammatory diseases, such as severe asthma and refractory chronic rhinosinusitis. Although transient eosinophilia and eosinophilic pneumoniae have been reported in patients with severe asthma and chronic rhinosinusitis with nasal polyps after the administration of dupilumab, few studies have examined the development of EGPA.Case presentation:We report a case of 61-year-old woman treated with dupilumab for refractory eosinophilic chronic rhinosinusitis and eosinophilic otitis media complicated by severe asthma. Although she had a previous history of eosinophilic pneumoniae and myeloperoxidase (MPO) ANCA positivity, there were no apparent findings of vasculitis suggesting EGPA before the initiation of dupilumab therapy. After the second administration of dupilumab, several adverse events developed, including worsening of ECRS, EOM and asthma, and neuropathy. Blood test showed an increase in the levels of eosinophils and re-elevation of MPO-ANCA levels after the administration of dupilumab therapy. Therefore, dupilumab therapy was discontinued owing to the development of EGPA, and prednisolone and azathioprine administration was initiated for a remission induction therapy. Conclusion:To the best of our knowledge, this is the first case report that suggests that dupilumab may directly trigger the manifestation of vasculitis in patients who were previously MPO-ANCA-positive. Although the precise mechanism of how dupilumab could trigger the development of EGPA requires further elucidation, to measure MPO-ANCA in patients with multiple eosinophilic disorders prior to the initiation of dupilumab might be helpful when considering the possibility of a latent EGPA. When administering dupilumab to patients with previous history of MPO-ANCA positivity, clinicians must carefully monitor and collaborate with other specialists in the pertinent fields of study for appropriate usage.

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“…However, to our knowledge, there have been no reports of the effectiveness of rituximab in EGPA patents with pulmonary thromboembolism. Moreover, we have found only one report of success with dupilumab for EGPA, in two pediatric cases (a 13-year-old boy and a 16year-old girl) [9]. Here, we encountered a rare case of pulmonary thromboembolism 12 years after the onset of EGPA.…”

Section: Discussionmentioning

confidence: 90%

Rituximab and Dupilumab Improve Eosinophilic Granulomatosis With Polyangiitis With Multiple Pulmonary Thrombi

Adachi¹,

Oshikata²,

Kaneko³

et al. 2021

Preprint

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BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is characterized by a necrotizing vasculitis with tissue and peripheral blood eosinophilia affecting small and medium-sized arteries, capillaries, and veins. Venous thromboembolic events have occurred in 19 of 232 (8.2%) patients with EGPA. However, there are only a few reported cases of EGPA complicated by pulmonary embolism or infarction. Case presentationWe report the case of a 43-year-old woman with eosinophilic granulomatosis with polyangiitis patient with acute respiratory and heart failure due to bilateral pulmonary artery thrombosis and left femoral vein thrombosis in addition to cardiac involvement as myocarditis, pericardial effusion, and diastolic dysfunction, gastrointestinal symptoms and peripheral neuropathy 12 years after disease onset. The condition was refractory to treatment with systemic corticosteroids, intravenous cyclophosphamide, and mepolizumab, but the acute cardiac failure associated with the thrombosis, cardiac and gastrointestinal symptoms, and multiple polyneuropathy improved after a switch to rituximab. But her heart failure did not improve sufficiently, she continued to need oxygen inhalation at 1 L/min and asthma exacerbations occurred. We changed the patient’s treatment with mepolizumab to dupilumab. Not only did she have no asthma attacks after switching to dupilumab, but also her vasculitis symptoms improved. Oxygen therapy was discontinued as heart failure improved five months after starting the dupilumab. ConclusionsThis may be the first case report of the successful treatment of pulmonary thromboembolism associated with EGPA by rituximab. In addition, in this patient, treatment with dupilumab was effective not only for the asthma symptoms but also for the symptoms of vasculitis.

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Two pediatric cases of ANCA-negative eosinophilic granulomatosis with polyangiitis successfully treated with dupilumab

Cited by 14 publications

References 9 publications

Clinical Features of Patients with Active Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab

Clinical Features of Patients with Active Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab

A case of eosinophilic granulomatosis with polyangiitis developed after dupilumab administration in patients with eosinophilic chronic rhinosinusitis and asthma

Rituximab and Dupilumab Improve Eosinophilic Granulomatosis With Polyangiitis With Multiple Pulmonary Thrombi

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