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Unmasking EGPA
Unmasking EGPA: Enhancing HCP Awareness, Early Detection, and Disease Management to Improve Patient Outcomes

Released: August 28, 2025

Anisha Dua
Anisha Dua, MD, MPH
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Key Takeaways
  • Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease that is often present in patients with asthma, chronic rhinosinusitis with nasal polyps, increased eosinophilia, and/or peripheral neuropathy, among other underlying diseases.
  • Patients with EGPA face a high disease burden and increased healthcare utilization and relapse rates, making its early identification and intervention critical for improving outcomes.
  • Interleukin-5 inhibitors like benralizumab and mepolizumab are FDA approved to treat adults with EGPA and as an add-on maintenance treatment for patients aged 6 years or older who have severe asthma with an eosinophilic phenotype.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis that causes eosinophilic infiltration and granulomatous inflammation in small to medium blood vessels. Enhanced awareness and recognition of EGPA is extremely important for early diagnosis. By recognizing clues from underlying disease and maintaining a high index of suspicion, healthcare professionals (HCPs) can intervene early to help prevent organ damage, control inflammation, and, most important, improve patients’ quality of life.

EGPA Prevalence and Disease Burden
Although EGPA is a rare disease, it is increasingly prevalent among those with asthma. Therefore, it is important to consider the presence of this disease in patients with asthma, especially those who rely on oral glucocorticoids. HCPs should also consider EGPA in patients with increased eosinophilia and other underlying diseases, including chronic rhinosinusitis with nasal polyps, peripheral neuropathy, and skin lesions, among other clinical manifestations. Although EGPA is not commonly encountered in clinical practice, its prevalence is increasing—partly due to HCPs’ increased recognition and awareness of this rare disease. There also are tools that we can use across multiple specialties to recognize and diagnose EGPA early, as it truly requires a multidisciplinary approach due to the variability in presentation and affected organs.

EGPA is associated with a substantial impact on healthcare utilization, and active disease can significantly influence patients’ functioning and quality of life. Because EGPA can become more active while tapering glucocorticoids, many patients end up on long-term, low-dose glucocorticoids and experience difficulty tapering off these therapies. This then results in a multitude of treatment-related toxicities like cataracts, hypertension, diabetes, osteoporotic fractures, difficulty sleeping, weight gain, and more. In addition, disease relapse is common and can include a range of manifestations—from vasculitic flare-ups to pulmonary or ears, nose, and throat manifestations. It is important for HCPs to recognize the features of a disease flare that would require changes to or escalation in therapy. My colleagues and I will address how certain interventions can be more beneficial than others, depending on patients’ comorbidities, preferences, and clinical manifestations of their active disease, in an upcoming symposium.

EGPA Treatment Landscape
The therapeutic landscape for EGPA continues to evolve as we gain a better understanding of its pathophysiology. Biologics like benralizumab and mepolizumab target the Th2 cytokine interleukin (IL)-5 and both are indicated as an add-on maintenance treatment for patients 6 years of age or older who have severe asthma with an eosinophilic phenotype. These agents recently were studied in phase III trials for use in patients with EGPA, and positive results led to their FDA approval—ultimately providing us with new therapies for managing patients.

When deciding what treatment to use in these complex patient cases, HCPs must get a sense of the disease severity and extent of organ involvement. Disease severity is often determined by whether the active clinical manifestations of EGPA are life- or organ-threatening. Based on the multiorgan and varied manifestations that patients with EGPA can present with, it also is important understand the latest expert guidance to inform your management strategies in the clinical setting. The American College of Rheumatology and Vasculitis Foundation have developed guidelines for the management of antineutrophil cytoplasmic antibody-associated vasculitis, including EGPA. This release was then followed by updated guidelines from the European Alliance of Associations for Rheumatology. Although there are some differences between these 2 guidelines, they provide the much-needed framework and key considerations for initiation of treatment and management for EGPA.

Be sure to attend the symposium to understand when to consider immunosuppressive therapies, such as cyclophosphamide, rituximab, and other oral DMARD agents, for patients with EGPA. You will also learn about the pivotal trials for the IL-5 inhibitors mepolizumab and benralizumab, the long-term efficacy and safety data from the MIRRA trial’s open label extension, and real-world evidence demonstrating how to use these agents in your daily practice. We will further discuss the head-to-head MANDARA trial which led to recent FDA approval of benralizumab, adding another medication to our toolbox of options for treating this complex disease.

Finally, the multidisciplinary management of patients with EGPA is critical. The perspective and practice patterns provided by different specialties are necessary for understanding each patient case. Furthermore, communication between these specialties and the patient are vital for optimizing care. Given the rarity of this disease, it is important for HCPs to offer guidance, resources, and honest, informative conversations to patients that help them understand the implications of EGPA and their treatment. Early recognition and diagnostic workup, a multidisciplinary care approach, increased therapeutic options, disease activity monitoring, and relapse management are all topics of major importance for practicing HCPs who care for patients with EGPA. The symposium will provide you with new perspectives and tools to help you recognize EGPA early, guide you on how to help patients throughout their journey, and shed light on the exciting therapeutic discoveries that are allowing us to target symptoms, provide better disease control, and improve patients’ lives.

Your Thoughts
Do you currently use mepolizumab or benralizumab in your practice? You can get involved in the conversation by answering the poll question or posting a comment below.

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