Eosinophilic Granulomatosis with Polyangiitis: Latest Findings and Updated Treatment Recommendations

Watanabe, Ryu; Hashimoto, Motomu

doi:10.3390/jcm12185996

Cited by 8 publications

(2 citation statements)

References 115 publications

(258 reference statements)

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“…In our case, ruling out critical differential diagnoses, we started a high dose of prednisolone and rituximab for the remission of EGPA. It can cause various critical organ features, such as interstitial pneumonia, carditis, and mononeuritis multiplex [ 12 ]. Such critical complications, once developed, can increase patients’ morbidity and mortality, impinging on their quality of life in rural contexts [ 13 - 15 ].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Granulomatosis With Polyangiitis Gradually Worsening With Consecutive Pregnancies: A Case Report

Ohta,

Fujimori,

Sano

2024

Cureus

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This case report elucidates the diagnosis and management of eosinophilic granulomatosis with polyangiitis (EGPA), a form of systemic vasculitis, in a 32-year-old female presenting with progressive dermal, respiratory, and gastrointestinal symptoms following multiple pregnancies. The patient's history of allergic reactions and pregnancies suggested a gradual progression of EGPA, a condition rarely associated with pregnancy. Initial symptoms were misinterpreted as allergic reactions and acute gastroenteritis, delaying the correct diagnosis. Laboratory findings included eosinophilia and elevated immunoglobulin E, while further investigations ruled out other differential diagnoses, such as chronic eosinophilic leukemia. A clinical diagnosis of EGPA was made based on symptom progression, eosinophilia, and mononeuritis multiplex, absent typical granulomatous changes in the skin biopsy. Treatment with high-dose prednisolone and rituximab halted disease progression and improved symptoms, highlighting the critical importance of prompt diagnosis and treatment in preventing irreversible complications. This case emphasizes the need for general physicians to consider pregnancy as a potential trigger for autoimmune diseases like EGPA, especially in patients presenting with multi-symptom allergic reactions and high inflammatory markers.

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Section: Discussionmentioning

confidence: 99%

Eosinophilic Granulomatosis With Polyangiitis Gradually Worsening With Consecutive Pregnancies: A Case Report

Ohta,

Fujimori,

Sano

2024

Cureus

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“…In EGPA, eosinophils secrete eosinophilic granules, including eosinophilic neurotoxin, major basic proteins, and eosinophilic cationic proteins, which contribute to tissue damage [100]. Each of these cationic proteins is associated with different organ damage, such as vascular endothelial damage with eosinophilic peroxidase, cardiotoxicity and fibrosis with eosinophilic cationic protein, and neurotoxicity with eosinophilic neurotoxin.…”

Section: Egpa Pathogenesismentioning

confidence: 99%

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Fouka,

Drakopanagiotakis,

Steiropoulos

2024

IJMS

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Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.

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Factors associated with drug retention of mepolizumab in patients with eosinophilic granulomatosis with polyangiitis: A multicentre REVEAL cohort study

Shiomi,

Watanabe,

Matsuda

et al. 2024

Modern Rheumatology

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Objectives: To determine the current retention rate of mepolizumab (MPZ) and identify factors associated with drug retention in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in the Kansai multicentre cohort (REVEAL cohort). Methods: Sixty patients diagnosed with EGPA and treated with MPZ between December 2016 and June 2023 were enrolled. The clinical characteristics, including laboratory data, treatments administered, and disease course outcomes were collected retrospectively. The patients were stratified into MPZ continuation (n=53) and discontinuation (n=7) groups, and drug retention was statistically compared using the log-rank test. Results: The median age of patients was 54.5 years, with 55% females, and 33% antineutrophil cytoplasmic antibody-positive at disease onset. MPZ exhibited a retention rate of 78.7% after five years. The reasons for discontinuation included treatment of coexisting diseases, inadequate response, and remission. Patient characteristics at disease onset were comparable between the groups. Patients receiving immunosuppressants (IS) before MPZ introduction demonstrated significantly higher retention rates (P = 0.038). During the final observation, the MPZ continuation group had a lower vasculitis damage index score (P = 0.027). Conclusions: MPZ exhibited a high 5-year retention rate, particularly in patients requiring IS. This study implies that long-term use of MPZ may mitigate irreversible organ damage.

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Eosinophilic Granulomatosis with Polyangiitis: Latest Findings and Updated Treatment Recommendations

Cited by 8 publications

References 115 publications

Eosinophilic Granulomatosis With Polyangiitis Gradually Worsening With Consecutive Pregnancies: A Case Report

Eosinophilic Granulomatosis With Polyangiitis Gradually Worsening With Consecutive Pregnancies: A Case Report

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Factors associated with drug retention of mepolizumab in patients with eosinophilic granulomatosis with polyangiitis: A multicentre REVEAL cohort study

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