엘러스 단로스 증후군(EDS) 시장 : 인사이트, 역학, 시장 예측(2034년)

Key Highlights:

• Although considered rare, the true prevalence of EDS is anticipated to be higher than currently appreciated, primarily due to a lack of awareness among the healthcare community. Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common subtype of EDS, accounting for approximately 90% of all EDS cases, the only subtype relying on a clinical diagnosis and currently without a defined genetic basis.
• Establishing the diagnosis of EDS is often problematic for patients, which interferes with the early detection, treatment, and prevention of further escalations of recognized symptoms, disability, and more elaborate complications.
• There is a lack of widespread clinical awareness and the absence of genetic markers for some subtypes (especially hEDS), which leads to delayed or missed diagnoses.
• Vascular EDS (vEDS) poses the greatest risk of mortality among Ehlers-Danlos subtypes due to the potential for arterial and organ rupture, yet there are currently no FDA-approved treatments available.
• With no currently approved treatments for vEDS anywhere in the world, this breakthrough and orphan drug designation by the FDA marks an important step forward in support of Zevra Therapeutics' goal to provide treatment options like EDSIVO to rare disease patients, who are often overlooked or underserved.

DelveInsight's "Ehlers-Danlos Syndrome (EDS) - Market Insight, Epidemiology, and Market Forecast - 2034" report delivers an in-depth understanding of EDS, historical and forecasted epidemiology, as well as the EDS market trends in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.

The EDS market report provides current treatment practices, emerging drugs, EDS share of individual therapies, and current and forecasted EDS market size from 2020 to 2034, segmented by seven major markets. The report also covers current EDS treatment practices/algorithms and unmet medical needs to curate the best of the opportunities and assess the underlying potential of the market.

Geography Covered:

• The United States
• EU4 (Germany, France, Italy, and Spain) and the United Kingdom
• Japan

Study Period: 2020-2034

Ehlers-Danlos Syndrome (EDS) Disease Understanding and Treatment Algorithm

Ehlers-Danlos Syndrome (EDS) Overview

EDS is a group of rare genetic connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. It includes 13 subtypes, with vascular EDS (vEDS) being the most severe due to risks like arterial rupture and organ perforation. Caused by collagen-related gene mutations, EDS is diagnosed through clinical evaluation and genetic testing. There are currently no FDA-approved treatments, and management focuses on supportive care, including physiotherapy, cardiovascular monitoring, and pain control. Early diagnosis and increased awareness remain critical for improving patient outcomes.

Ehlers-Danlos Syndrome (EDS) Diagnosis

Diagnosis of EDS involves a combination of clinical assessment, family history, and genetic testing. Clinicians evaluate characteristic features such as joint hypermobility, skin elasticity, and tissue fragility, using standardized tools like the Beighton score for hypermobility. For certain subtypes, especially vascular EDS, molecular genetic testing is essential to confirm mutations in specific collagen-related genes (e.g., COL3A1). In cases where genetic tests are inconclusive, diagnosis relies on clinical criteria established by the 2017 International Classification of EDS. Due to overlapping symptoms with other conditions, early recognition by experienced specialists is crucial to ensure accurate diagnosis and management.

Ehlers-Danlos Syndrome (EDS) Treatment

Treatment for EDS is primarily supportive, as there are currently no FDA-approved therapies targeting the underlying genetic causes. This typically involves physical therapy to strengthen muscles and stabilize joints, along with pain management strategies tailored to the individual. Cardiovascular monitoring is essential, especially for patients with vascular EDS due to the risk of arterial rupture. Preventive measures such as avoiding high-impact activities and protecting fragile skin and tissues are also important. Genetic counseling is recommended for affected individuals and their families. Celiprolol, a B1-blocker, is the only drug in late-stage development, being investigated for its potential to reduce life-threatening vascular events in vEDS, though it is not yet approved by the FDA. Multidisciplinary care remains the cornerstone of effective management.

Ehlers-Danlos Syndrome (EDS) Epidemiology

The EDS epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by the total prevalent cases of EDS, diagnosed prevalent cases of EDS, and subtype-specific cases of EDS in the 7MM market covering the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan from 2020 to 2034.

• The most common forms are hEDS, which affects between 1 in 10,000 and 1 in 15,000 people, and EDS classical type, which affects 1 per 10,000 to 1 per 20,000 people.
• Aside from the common subtypes, most others are exceedingly rare, with specific subtypes like EDS kyphoscoliotic type identified in about 60 total patients and EDS dysfibronectinemic type identified in only one single family.
• The prevalence for the vEDS subtype was 42%, for unspecified EDS was 19%, and for hEDS was 20%.
• Most EDS types are inherited in an autosomal dominant pattern, giving each child a 50% chance of inheriting the condition. While both sexes can carry the gene equally, women with hypermobile EDS often show more symptoms, making it appear more common in females.

Ehlers-Danlos Syndrome (EDS) Drug Chapters

Emerging Drugs

EDSIVO (celiprolol): Zevra Therapeutics

Celiprolol is a selective B1-adrenergic receptor antagonist investigational for the treatment of vEDS. Its mechanism of action is designed to reduce the mechanical stress on collagen fibers within the arterial wall through vascular dilation and smooth muscle relaxation.

Celiprolol has been granted Orphan Drug and Breakthrough Therapy designations by the US FDA. Zevra recently restarted enrollment in the DiSCOVER trial, a Phase III trial being conducted under a Special Protocol Assessment (SPA) agreement with the US FDA. Celiprolol is protected by intellectual property rights until 2038.

In November 2023, Zevra Therapeutics announced the completion of its acquisition of Acer Therapeutics, which marks a significant step forward in executing Zevra's strategy to become a leader in developing and commercializing treatments for rare diseases. Zevra has obtained access to Acer's rare disease assets OLPRUVA and EDSIVO.

Drug Class Insight

In the treatment of vEDS, B1-adrenergic receptor antagonists like celiprolol have emerged as a cornerstone therapy.

B1-adrenergic receptor antagonist

B1-adrenergic receptor antagonists play a critical role in reducing cardiovascular stress that can contribute to life-threatening complications. B1 receptors are primarily located in the heart, where their activation increases heart rate and contractility, leading to elevated blood pressure. In individuals with vEDS, where the blood vessels are unusually fragile due to defects in type III collagen, even normal cardiovascular forces can increase the risk of arterial dissection or rupture. By blocking B1 receptors, drugs like celiprolol help lower heart rate and systolic blood pressure, thereby reducing the mechanical stress exerted on weakened vascular walls. This protective effect is key to minimizing vascular events and improving outcomes in patients with vEDS.

Ehlers-Danlos Syndrome (EDS) Market Outlook

Because the condition is genetic, there is no cure for EDS. Treatment focuses on managing symptoms, particularly by reducing stress on the aorta and other arteries, addressing skeletal and joint complications, and alleviating associated pain. The therapeutic landscape for EDS remains largely unmet, with no FDA-approved or globally marketed drugs specifically targeting the condition. Management is limited to supportive care, including blood pressure control, physical therapy, and cautious surgical intervention when needed. Celiprolol, a B1-adrenergic receptor antagonist with partial B2-agonist activity, is the only emerging pharmacological candidate showing potential, especially in reducing the risk of vascular events in vEDS. It is currently in Phase III clinical development but remains unapproved by the FDA, with access largely restricted to certain European countries. The lack of approved therapies underscores a significant unmet medical need. Future progress will depend not only on advancing candidates like celiprolol but also on increased research investment to develop targeted treatments that address the underlying genetic and vascular mechanisms of EDS.

Ehlers-Danlos Syndrome (EDS) Drugs Uptake

This section focuses on the uptake rate of potential drugs expected to be launched in the market during 2025-2034. The landscape of EDS treatment has experienced a profound transformation with the uptake of novel drugs.

Ehlers-Danlos Syndrome (EDS) Pipeline Development Activities

The report provides insights into different therapeutic candidates in late and early stages. It also analyzes key players involved in developing targeted therapeutics.

Pipeline Development Activities

The report covers detailed information on collaborations, acquisitions and mergers, licensing, and patent details for EDS' emerging therapies.

KOL- Views

To keep up with current market trends, we take KOLs and SMEs' opinions working in the domain through primary research to fill the data gaps and validate our secondary research. Some of the leaders like MD, Professor and Vice Chair of the Department of Medicine and Director, PhD, and others. Their opinion helps to understand and validate current and emerging therapies and treatment patterns or EDS market trends. This will support the clients in potential upcoming novel treatments by identifying the overall scenario of the market and the unmet needs.

Delveinsight's analysts connected with 10+ KOLs to gather insights; however, interviews were conducted with 5+ KOLs in the 7MM. Centers such as the Washington University School of Medicine, University Medical Center Hamburg-Eppendorf, and University Graduate School of Medicine, etc. were contacted. Their opinion helps understand and validate EDS epidemiology and market trends.

Qualitative Analysis

We perform qualitative and market intelligence analysis using various approaches, such as SWOT and Conjoint analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of disease diagnosis, patient awareness, patient burden, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided. These pointers are based on the Analyst's discretion and assessment of the patient burden, cost analysis, and existing and evolving treatment landscape.

Conjoint Analysis analyzes multiple approved and emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, designation, route of administration, and order of entry. Scoring is given based on these parameters to analyze the effectiveness of therapy.

The analyst analyzes multiple emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, route of administration, and order of entry.

In efficacy, the trial's primary and secondary outcome measures are evaluated.

Further, the therapies' safety is evaluated, wherein the acceptability, tolerability, and adverse events are majorly observed, and it sets a clear understanding of the side effects posed by the drug in the trials.

Market Access and Reimbursement

Reimbursement may be referred to as the negotiation of a price between a manufacturer and a payer that allows the manufacturer access to the market. It is provided to reduce the high costs and make the essential drugs affordable. Health technology assessment (HTA) plays an important role in reimbursement decision-making and recommending the use of a drug. These recommendations vary widely throughout the seven major markets, even for the same drug. In the US healthcare system, both Public and Private health insurance coverage are included. Also, Medicare and Medicaid are the largest government-funded programs in the US. The major healthcare programs, including Medicare, Medicaid, Health Insurance Program (CHIP), and the state and federal health insurance marketplaces, are overseen by the Centers for Medicare & Medicaid Services (CMS). Other than these, Pharmacy Benefit Managers (PBMs) and third-party organizations that provide services and educational programs to aid patients are also present.

The report further provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenario of currently used therapies, programs making accessibility easier and out-of-pocket costs more affordable, insights on patients insured under federal or state government prescription drug programs, etc.

Scope of the Report:

• The report covers a descriptive overview of EDS, explaining its causes, signs and symptoms, pathogenesis, and currently available therapies.
• Comprehensive insight has been provided into EDS epidemiology and treatment.
• Additionally, an all-inclusive account of the current therapies for EDS is provided, along with the assessment of new therapies, which will have an impact on the current treatment landscape.
• A detailed review of the EDS market, historical and forecasted, is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies, by understanding trends shaping and driving the 7MM EDS market.

Ehlers-Danlos Syndrome (EDS) Report Insights

Ehlers-Danlos Syndrome (EDS) Report Insights

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

Ehlers-Danlos Syndrome (EDS) Report Key Strengths

• Ten-Year Forecast
• 7MM Coverage
• EDS Epidemiology Segmentation
• Key Cross Competition
• Highly Analyzed Market
• Drugs Uptake

Ehlers-Danlos Syndrome (EDS) Report Assessment

• Current Treatment Practices
• Unmet Needs
• Analyst Views
• Pipeline Product Profiles
• Market Attractiveness
• Qualitative Analysis (SWOT and Conjoint Analysis)

FAQs:

• What was the EDS market share (%) distribution in 2020, and what would it look like in 2034?
• What would be the EDS total market size, as well as market size by therapies across the 7MM during the study period (2020-2034)?
• What are the key findings about the market across the 7MM, and which country will have the largest EDS market size during the study period (2020-2034)?
• At what CAGR, the EDS market expected to grow at the 7MM level during the study period (2020-2034)?
• What would be the EDS market growth till 2034?
• What are the disease risks, burdens, and unmet needs of EDS?
• What is the historical EDS patient pool in the United States, the EU4 (Germany, France, Italy, and Spain), and the UK, and Japan?
• What will be the growth opportunities across the 7MM concerning the patient population of EDS?
• Among the 7MM, which country would have the most prevalent cases of EDS?
• At what CAGR is the population expected to grow across the 7MM during the study period (2020-2034)?
• How many companies are developing therapies for the treatment of EDS?
• What are the key collaborations (industry-industry, industry-academia), mergers and acquisitions, and licensing activities related to EDS therapies?
• What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitations of existing therapies?
• What are the 7MM historical and forecasted market of EDS?

Reasons to buy:

• The report will help in developing business strategies by understanding trends shaping and driving the EDS market.
• To understand the future market competition in the EDS market and insightful review of the SWOT analysis of EDS.
• Organize sales and marketing efforts by identifying the best opportunities for EDS in the US, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.
• Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
• To understand the future market competition in the EDS.

Table of Contents

1. Key Insights

2. Report Introduction

3. Executive Summary of Ehlers-Danlos Syndrome

4. Key Events

5. Ehlers-Danlos Syndrome Epidemiology and Market Forecast Methodology

6. Ehlers-Danlos Syndrome Market Overview at a Glance

• 6.1. Market Share (%) Distribution of Ehlers-Danlos Syndrome by Therapies in 2024
• 6.2. Market Share (%) Distribution of Ehlers-Danlos Syndrome by Therapies in 2034

7. Disease Background and Overview: Ehlers-Danlos Syndrome

• 7.1. Introduction
• 7.2. Signs and Symptoms
• 7.3. Causes
• 7.4. Diagnosis

8. Treatment and Management

• 8.1. Treatment Guidelines and Recommendations

9. Epidemiology and Patient Population of Ehlers-Danlos Syndrome in the 7MM

• 9.1. Key Findings
• 9.2. Assumptions and Rationale
• 9.3. Total Prevalent Cases of EDS in the 7MM
• 9.4. The United States
  • 9.4.1. Total Prevalent Cases of EDS in the United States
  • 9.4.2. Diagnosed Prevalent Cases of EDS in the United States
  • 9.4.3. Subtype-specific Cases of EDS in the United States
• 9.5. EU4 and the UK
  • 9.5.1. Total Prevalent Cases of EDS in EU4 and the UK
  • 9.5.2. Diagnosed Prevalent Cases of EDS in EU4 and the UK
  • 9.5.3. Subtype-specific Cases of EDS in EU4 and the UK
• 9.6. Japan
  • 9.6.1. Total Prevalent Cases of EDS in Japan
  • 9.6.2. Diagnosed Prevalent Cases of EDS in Japan
  • 9.6.3. Subtype-specific Cases of EDS in Japan

10. Patient Journey

11. Emerging Therapies

• 11.1. Key Competitors
• 11.2. EDSIVO (celiprolol): Zevra Therapeutics
  • 11.2.1. Product Description
  • 11.2.2. Other Developmental Activity
  • 11.2.3. Clinical Developmental Activities
  • 11.2.4. Safety and efficacy
  • 11.2.5. Analyst Views

12. Ehlers-Danlos Syndrome: Seven Major Market Analysis

• 12.1. Key Findings
• 12.2. Key Market Forecast Assumptions
  • 12.2.1. Cost Assumptions and Rebate
  • 12.2.2. Pricing Trends
  • 12.2.3. Analogue Assessment
  • 12.2.4. Launch Year and Therapy Uptake
• 12.3. Market Outlook
• 12.4. Total Market Size of EDS in the 7MM
• 12.5. United States Market Size
  • 12.5.1. Total Market Size of EDS in the United States
  • 12.5.2. Market Size of EDS by Therapies in the United States
• 12.6. EU4 and the UK Market Size
  • 12.6.1. Total Market Size of EDS in EU4 and the UK
  • 12.6.2. Market size of EDS by Therapies in EU4 and the UK
• 12.7. Japan Market Size
  • 12.7.1. Total Market Size of EDS in Japan
  • 12.7.2. Market Size of EDS by Therapies in Japan

13. Unmet Needs

14. SWOT Analysis

15. KOL Views

16. Market Access and Reimbursement

17. Appendix

• 17.1. Bibliography
• 17.2. Report Methodology

18. DelveInsight Capabilities

19. Disclaimer

20. About DelveInsight