Eosinophilic Granulomatosis with Polyangiitis - Market Insight, Epidemiology, and Market Forecast - 2034

Key Highlights:

• Eosinophilic granulomatosis with polyangiitis (EGPA), previously referred to as Churg-Strauss syndrome, is a rare form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. It is characterized by systemic necrotizing granulomatous inflammation that targets small to medium-sized blood vessels, potentially leading to severe, life-threatening damage across multiple organ systems.
• According to the American Lung Association, EGPA is classified as an ultra-rare disease, with an estimated prevalence of approximately 5,000 cases in the United States.
• Based on the secondary analysis, EGPA has an estimated prevalence of 2 cases per one million individuals within the German population, indicating its classification as an exceptionally rare disease in that demographic context.
• The rising cases of EGPA may be attributed to improved diagnostic techniques, heightened clinical awareness, and broader use of ANCA testing. Additionally, increased prevalence of asthma and eosinophilic disorders may contribute to the upward trend in prevalence rates observed in recent years.
• EGPA management involves FDA-approved biologics like NUCALA (mepolizumab, GSK) and FASENRA (benralizumab, AstraZeneca), which target IL-5 to control eosinophilic activity. Additionally corticosteroids and immunosuppressants offering a comprehensive approach to reduce inflammation, prevent relapses, and minimize long-term steroid dependence.
• Numerous companies are actively developing EGPA therapies, including NS Pharma (Nippon Shinyaku) with NS-229 and GSK with depemokimab among others. These innovative treatments aim to address critical unmet clinical needs and show encouraging efficacy. Securing regulatory approval for these therapies could greatly enhance the EGPA treatment landscape, stimulate market growth, and yield significant revenue by improving patient outcomes and promoting wider clinical adoption.

DelveInsight's comprehensive report titled "Eosinophilic Granulomatosis with Polyangiitis (EGPA) - Market Insights, Epidemiology, and Market Forecast - 2034" offers a detailed analysis of EGPA. The report presents historical and projected epidemiological data covering diagnosed prevalent cases of EGPA, gender-specific diagnosed prevalent cases of EGPA and age-specific diagnosed prevalent cases of EGPA. In addition to epidemiology, the market report encompasses various aspects related to the patient population. These aspects include the diagnosis process, prescription patterns, physician perspectives, market accessibility, treatment options, and prospective developments in the market across 7MM: the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan, spanning from 2020 to 2034.

The report analyzes the existing treatment practices and unmet medical requirements in EGPA. It evaluates the market potential and identifies potential business prospects for enhancing therapies or interventions. This valuable information enables stakeholders to make well-informed decisions regarding product development and strategic planning for the market.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Overview

EGPA, formerly known as Churg-Strauss Syndrome, is a rare vasculitic condition affecting small to medium-sized blood vessels. It features systemic granulomatous and eosinophilic inflammation, commonly presenting with asthma and elevated peripheral eosinophil counts. EGPA can involve multiple organs, including the paranasal sinuses, lungs, heart, skin, nerves, gastrointestinal tract, eyes, and kidneys. Clinical presentation and prognosis vary based on ANCA status. ANCA-positive patients often exhibit vasculitis affecting the skin, peripheral nerves, and kidneys, while ANCA-negative individuals typically show eosinophilic infiltration in the lungs and heart. Interestingly, ANCA positivity is associated with a lower mortality rate but a higher frequency of disease relapse compared to ANCA-negative cases.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Diagnosis and Treatment Overview

EGPA is diagnosed through a combination of clinical evaluation and diagnostic tests. Physicians typically begin with a detailed medical history and physical exam, focusing on asthma, allergies, and systemic symptoms. Blood tests assess eosinophil levels-elevations above 5% of total white blood cells are suggestive. Urinalysis may reveal kidney involvement through excess red blood cells or protein. Imaging such as chest X-rays or CT scans helps identify lung or sinus abnormalities. A biopsy may confirm EGPA by detecting eosinophilic granulomas or vasculitis.

Treatment begins with corticosteroids to control inflammation, often at high doses initially, then tapered. Immunosuppressive agents like rituximab, cyclophosphamide, methotrexate, azathioprine, and mycophenolate mofetil may be added for severe or refractory cases. Targeted biologics such as mepolizumab and benralizumab are effective in reducing eosinophil activity and are especially useful in ANCA-negative patients with prominent eosinophilic features.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Epidemiology

The epidemiology section of the EGPA market report offers information on the patient populations, including historical and projected trends for each of the 7MM. Examining Key Opinion Leader (KOL) views from physicians or clinical experts can assist in identifying the reasons behind historical and projected trends. The diagnosed patient pool, their trends, and the underlying assumptions are all included in this section of the report.

This section also presents the data with relevant tables and graphs, offering a clear and concise view of the prevalence of EGPA. Additionally, the report discloses the assumptions made during the analysis, ensuring data interpretation and presentation transparency. This epidemiological data is valuable for understanding the disease burden and its impact on the patient population across various regions.

Key Findings

• Based on the study findings, the prevalence of EGPA in Europe is approximately 12.13 cases per million individuals, highlighting its rarity within the population.
• According to the secondary analysis, in Japan, the prevalence of EGPA is estimated at 58.6 cases per 1,000,000 individuals, indicating a relatively higher occurrence compared to other regions.
• In Germany, the prevalence of EGPA was notably higher among females, accounting for 56% of reported cases, suggesting a potential gender-related predisposition to the disease.
• A database study conducted in Japan in 2020 revealed a marked age-related disparity in EGPA prevalence, with 181.3 cases per million observed among individuals aged 65-74 years, compared to 42.5 cases per million in the 18-39 age group. This suggests a significantly higher disease burden in older populations.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Market Outlook

The EGPA therapeutics market is further expected to increase by the major drivers, such as the rising prevalent population, technological advancements, and upcoming therapies in the forecast period (2025-2034).

The treatment landscape for EGPA integrates the US FDA-approved biologics such as NUCALA (mepolizumab, GSK) and FASENRA (benralizumab, AstraZeneca), which target interleukin-5 (IL-5) to suppress eosinophilic activity and reduce inflammation. These drugs offer steroid-sparing benefits and improved disease control. Corticosteroids remain a cornerstone for acute management, while immunosuppressants like azathioprine, methotrexate, or cyclophosphamide are used for severe or refractory cases. This multi-modal approach aims to induce remission, prevent relapses, and minimize long-term organ damage. Personalized treatment strategies based on ANCA status and disease severity are increasingly guiding therapeutic decisions, enhancing patient outcomes and quality of life.

With ongoing research and continued dedication, the future holds hope for even more effective treatments and, ultimately, a cure for this challenging condition. According to DelveInsight, the EGPA market in the 7MM is expected to change significantly during the forecast period (2025-2034).

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Drug Chapters

Marketed Drugs

NUCALA (mepolizumab): GSK

Mepolizumab is a humanized monoclonal antibody (mAb) that targets interleukin-5 (IL-5), classified as an IgG1 kappa. It is produced using recombinant DNA technology in Chinese hamster ovary cells. IL-5 plays a central role in the development, activation, recruitment, and survival of eosinophils. By blocking IL-5 signaling, mepolizumab reduces eosinophil levels. While its effect on eosinophil regulation is clear, the precise mechanism of action in EGPA not fully understood. Mepolizumab has received regulatory approval for EGPA in the United States, European Union, and Japan.

• In November 2021, the European Commission approved NUCALA (mepolizumab) as an add-on treatment for eosinophilic granulomatosis with polyangiitis (EGPA), following a positive Committee for Medicinal Products for Human Use (CHMP) recommendation supporting its clinical benefit.
• In December 2017, GSK reported that the US Food and Drug Administration (FDA) had approved NUCALA (mepolizumab) as the first targeted therapy for EGPA, formerly referred to as Churg-Strauss syndrome.

FASENRA (benralizumab): AstraZeneca

Benralizumab developed by AstraZeneca is a humanized, afucosylated IgG1/κ mAb targeting IL-5Ra on eosinophils and basophils. Its Fc domain enhances binding to FcYRIII on NK cells, inducing eosinophil and basophil apoptosis via antibody-dependent cell-mediated cytotoxicity (ADCC). Benralizumab has gained regulatory approval for EGPA in the United States, European Union, and Japan

• In October 2024, the European Union (EU) approved FASENRA (benralizumab) for EGPA, following MANDARA trial results showing nearly 60% remission and 41% of patients discontinuing oral corticosteroids.
• In September 2024, the US FDA approved AstraZeneca's FASENRA (benralizumab) for treating adult patients diagnosed with EGPA in the United States.

Emerging Drugs

Depemokimab: GSK

Depemokimab, a mAB targeting IL-5, is the first ultra-long-acting biologic undergoing Phase III trials in patients. The Phase III program also explores its potential in other IL-5-driven conditions, including EGPA.

• Currently, depemokimab is in Phase III (OCAN) for the treatment of EGPA.
• As per the 2025 company's corporate presentation, Phase III data for depemokimab in EGPA is expected to be released in the second half of 2026.

NS-229: NS Pharma (Nippon Shinyaku)

NS-229 is a highly selective Janus kinase (JAK) 1 inhibitor developed internally, designed to suppress over activation of T cells, B cells, and specific white blood cells. By modulating immune responses, NS-229 is expected to minimize tissue damage and alleviate multiple symptoms associated with EGPA.

• Currently, NS-229 is in Phase II for the treatment of EGPA.
• In April 2025, NS Pharma reported that the US FDA had granted Orphan Drug Designation (ODD) to NS-229, a therapy in development for the treatment of the rare condition EGPA.
• In January 2024, NS Pharma revealed that the European Commission (EC) had awarded ODD to NS-229.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Market Segmentation

DelveInsight's 'Eosinophilic Granulomatosis with Polyangiitis (EGPA) - Market Insights, Epidemiology, and Market Forecast - 2034' report provides a detailed outlook of the current and future EGPA market, segmented within countries, by therapies, and by classes. Further, the market of each region is then segmented by each therapy to provide a detailed view of the current and future market share of all therapies.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Market Size by Countries

The EGPA market size is assessed separately for various countries, including the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan. In 2024, the United States held a significant share of the overall 7MM EGPA market, primarily attributed to the country's higher prevalence of the condition and the elevated cost of the available treatments. This dominance is projected to persist, especially with the potential early introduction of new products.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Drugs Uptake

This section focuses on the sales uptake of potential EGPA drugs that have recently been launched or are anticipated to be launched in the EGPA market between 2020 and 2034. It estimates the market penetration of EGPA drugs for a given country, examining their impact within and across classes and segments. It also touches upon the financial and regulatory decisions contributing to the probability of success (PoS) of the drugs in the EGPA market.

The emerging EGPA therapies are analyzed based on various attributes such as safety and efficacy in randomized clinical trials, order of entry and other market dynamics, and the unmet need they fulfill in the EGPA market.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Market Access and Reimbursement

DelveInsight's 'Eosinophilic Granulomatosis with Polyangiitis (EGPA) - Market Insights, Epidemiology, and Market Forecast - 2034' report provides a descriptive overview of the market access and reimbursement scenario of EGPA. This section includes a detailed analysis of the country-wise healthcare system for each therapy, enlightening the market access, reimbursement policies, and health technology assessments.

Key Opinion Leader (KOL) Views

To keep up with current EGPA market trends and fill gaps in secondary findings, we interview KOLs and SMEs' working in the EGPA domain. Their opinion helps understand and validate current and emerging therapies and treatment patterns or EGPA market trends. This will support the clients in potential upcoming novel treatments by identifying the overall scenario of the market and the EGPA unmet needs.

Eosinophilic Granulomatosis with Polyangiitis (EGPA): KOL Insights

DelveInsight's analysts connected with 20+ KOLs to gather insights; however, interviews were conducted with 10+ KOLs in the 7MM. These KOLs were from organizations, institutes, and hospitals, such as, Wilson Case Western University, US, University of Tubingen, Kirchheim Teck, Germany; University Claude Bernard, Lyon, France; University of Cagliari, Cagliari, Italy; Hospital Clinic de Barcelona, Barcelona, Spain; Respiratory Research & Development, GSK, London, UK; Kochi Medical School, Nankoku, Japan, among others.

"As per KOL from the US, "Clinical studies show that approximately 50% of patients treated with mepolizumab achieved a 50% reduction in glucocorticoid use. This underscores its value as a steroid-sparing option, offering meaningful disease control while minimizing long-term toxicity. For patients with EGPA, mepolizumab represents a significant advancement in personalized, targeted therapy."

"As per KOL from the UK, "Despite recent biologic approvals, EGPA patients still face unmet needs in relapse prevention, tailored treatment for ANCA subtypes, and effective strategies to minimize long-term steroid dependence."

"As per KOL from Japan, "The cause of EGPA remains unknown. In autoimmune diseases like vasculitis, increased immune activity leads to attacks on healthy tissues for unclear reasons. EGPA is most commonly seen in the lungs, though other organs may also be affected with varying severity."

Competitive Intelligence Analysis

We conduct a Competitive and Market Intelligence analysis of the EGPA. Market, utilizing various competitive intelligence tools such as SWOT analysis and Market entry strategies. The inclusion of these analyses is contingent upon data availability, ensuring a comprehensive and well-informed assessment of the market landscape and competitive dynamics.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Pipeline Development Activities

The report offers an analysis of therapeutic candidates in Phase II and III stages and examines companies involved in developing targeted therapeutics for EGPA. It provides valuable insights into the advancements and progress of potential treatments in clinical development for this condition.

Pipeline Development Activities

The report covers information on collaborations, acquisition and merger, licensing, patent details, and other information for emerging EGPA therapies.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Report Insights

• EGPA Patient Population
• Therapeutic Approaches
• EGPA Pipeline Analysis
• EGPA Market Size and Trends
• EGPA Market Opportunities
• Impact of Upcoming Therapies

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Report Key Strengths

• 10 Years Forecast
• The 7MM Coverage
• EGPA Epidemiology Segmentation
• Key Cross Competition
• Highly Analyzed EGPA Market
• EGPA Drugs Uptake

Eosinophilic Granulomatosis with Polyangiitis (EGPA) Report Assessment

• EGPA Current Treatment Practices
• Unmet Needs
• EGPA Product Profiles
• EGPA Market Attractiveness

Key Questions:

• How common is EGPA?
• What are the key findings of EGPA epidemiology across the 7MM, and which country will have the highest number of patients during the study period (2020-2034)?
• What are the currently available treatments for EGPA?
• What are the disease risk, burden, and unmet needs of EGPA?
• At what CAGR is the EGPA market and its epidemiology is expected to grow in the 7MM during the forecast period (2025-2034)?
• How would the unmet needs impact the EGPA market dynamics and subsequently influence the analysis of the related trends?
• What would be the forecasted patient pool of EGPA in the 7MM covering the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan?
• Among EU4 and the UK, which country will have the highest number of patients during the forecast period (2025-2034)?
• How many companies are currently developing therapies for the treatment of EGPA?

Reasons to buy:

• The report will help in developing business strategies by understanding the latest trends and changing treatment dynamics driving the EGPA market.
• Insights on patient burden/disease prevalence, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• To understand the existing market opportunity in varying geographies and the growth potential over the coming years.
• Distribution of historical and current patient share based on real-world prescription data along with reported sales of current treatment in the US, EU4 (Germany, France, Italy, and Spain), the UK, and Japan.
• Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
• Detailed analysis and ranking of class-wise potential current and emerging therapies under the attribute analysis section to provide visibility around leading classes.
• Highlights of market access and reimbursement policies of approved therapies, barriers to accessibility of off-label expensive therapies, and patient assistance programs.
• To understand the perspective of KOL around the accessibility, acceptability, and compliance-related challenges of existing treatment to overcome barriers in the future.
• Detailed insights on the unmet needs of the existing market so that the upcoming players can strengthen their development and launch strategy.

Frequently Asked Questions:

1. What are the treatment goals for EGPA?

The primary treatment goals for EGPA are to induce and maintain remission, control eosinophilic inflammation, and prevent organ damage. Reducing reliance on corticosteroids while minimizing disease relapses is essential. Therapy aims to alleviate symptoms, improve quality of life, and preserve organ function. Long-term management also focuses on minimizing treatment-related side effects and tailoring therapy based on ANCA status and disease severity to achieve optimal outcomes.

2. What are the challenges in managing EGPA?

Managing EGPA is challenging due to its heterogeneous presentation, variable ANCA status, and potential for multi-organ involvement. Balancing immunosuppression to control disease while minimizing side effects is complex. Frequent relapses, steroid dependence, and distinguishing active disease from damage complicate care. Limited biomarkers and individualized treatment needs further add to difficulties. Monitoring long-term complications and tailoring therapy to disease severity remain critical yet demanding aspects of management.

3. What are the key factors driving the growth of the EGPA market?

The EGPA market is gaining momentum, fueled by rising disease awareness, improved diagnostic tools, and breakthroughs in biologics like anti-IL-5 therapies. Precision medicine is reshaping treatment paradigms, while growing clinical trials and regulatory support accelerate innovation. As patient advocacy amplifies unmet needs, pharma investment surges. Together, these forces create a dynamic ecosystem where science, strategy, and patient-centric care converge to drive market expansion and therapeutic evolution.

4. How will the EGPA market and epidemiology forecast report benefit the clients?

The EGPA market and epidemiology forecast report offers clients strategic insights into disease prevalence, patient segmentation, and market dynamics. It helps identify commercial opportunities, assess treatment gaps, and support planning for product development, market entry, or expansion. The report also aids stakeholders in understanding regulatory landscapes, forecasting trends, and making data-driven investment or policy decisions.