이 질병은 유전성이기 때문에 EDS의 근치 치료법은 존재하지 않습니다. 치료는 증상 관리에 중점을 두고 특히 대동맥 및 기타 동맥에 대한 부하 경감, 골격·관절 합병증에 대한 대응, 관련 통증의 완화를 도모합니다. EDS의 치료 상황은 여전히 미충족이며, 본 질환을 표적으로 한 FDA 승인 약물이나 세계적으로 상시되고있는 약물은 존재하지 않습니다. 관리는 지원 요법(혈압 관리, 물리치료, 필요에 따라 신중한 수술 개입 등)으로 제한됩니다. B1 아드레날린 수용체 길항제이고 부분적인 B2 작용제 활성을 갖는 celiprolol은 특히 혈관성 EDS(vEDS)에서 혈관 사건 위험 감소에서 잠재성을 나타내는 유일한 새로운 약리학적 후보 약물입니다. 현재 단계 III 임상 개발 단계이지만 FDA 승인은 얻지 못했으며 주로 특정 유럽 국가에 의해 액세스가 제한됩니다. 승인된 치료법의 부족은 큰 암메트 의료 요구를 돋보이게합니다. 미래의 진행은 celiprolol과 같은 후보 약물의 진보뿐만 아니라 EDS의 근본적인 유전적 및 혈관적 메커니즘을 다루는 표적 치료법을 개발하기위한 연구 투자 증가에 달려 있습니다.
본 보고서는 엘러스 단로스 증후군(EDS)의 주요 7개 시장(미국, 독일, 스페인, 이탈리아, 프랑스, 영국, 일본)에 대한 조사 분석을 통해 각국 시장 규모, 역학 데이터, 각 치료법 시장 점유율, 신치료법, 암멧 필요 등의 정보를 제공합니다.
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.
Study Period: 2020-2034
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.
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.
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.
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.
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.