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폐동맥 고혈압 : 시장 인사이트, 역학 및 예측(2036년)

Pulmonary Arterial Hypertension - Market Insight, Epidemiology, and Market Forecast - 2036

발행일: | 리서치사: 구분자 DelveInsight | 페이지 정보: 영문 250 Pages | 배송안내 : 2-10일 (영업일 기준)

    
    
    




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폐동맥 고혈압(PAH)에 대한 인사이트 및 동향

  • DelveInsight의 분석에 따르면, 2025년 주요 시장(미국, EU4(독일, 프랑스, 이탈리아, 스페인), 영국 및 일본)의 폐동맥 고혈압 시장 규모는 약 80억 달러였습니다.
  • 2025년 기준으로, 미국 내 PAH 유병 사례 수는 약 5만 건, 진단된 유병 사례 수는 약 4만 4,000건으로 보고되고 있습니다.
  • 폐동맥 고혈압(PAH)에 관한 역학적 인사이트는 데이터가 오래되었고 지역에 따라 편차가 있으며, 조사 방법에 일관성이 없고, 비특이적인 초기 증상으로 인해 진단 누락이 빈번하게 발생하는 등의 이유로 여전히 제한적입니다.
  • PAH 치료의 주요 목적은 환자의 증상을 호전시키고 임상 상태의 악화를 늦추는 것입니다. PAH 치료에는 일반적인 조치, 지지 요법, 약물 요법 및 외과적 치료가 사용됩니다.
  • 폐동맥 고혈압 치료의 전반적인 양상은 셀렉시파그(UPTRAVI), 트레프로스티닐(TYVASO), 리오시구아트(ADEMPAS) 등 기능 분류에 걸쳐 있는 여러 가지 승인된 치료법에 의해 뒷받침되고 있습니다. 현재의 표준 치료법은 표적 치료제, PDE5 억제제, 가용성 구아닐산 시클라제 자극제, 엔도세린 수용체 길항제(ERA), 그리고 프로스타사이클린 경로 치료제로 구성되어 있으며, 이들 약물이 복합적으로 작용하여 주요 병인 경로를 조절하고 있습니다.
  • 예측 기간 동안 시장에 출시될 것으로 기대되는 다양한 치료법으로는 라리네파그(United Therapeutics), RT234(발데나필 흡입 분말)(Respira Therapeutics), 세랄티닙(GB002)(Gossamer Bio), 그리고 L606(리포솜형 트레프로스티닐)(Pharmosa Biopharm/Liquidia) 등이 포함됩니다.
  • PAH는 드물고 매우 복잡하며 진행성인 질환으로, 불치병이며 종종 조기 사망으로 이어집니다. 최상의 치료 결과를 얻기 위해서는 조기 진단이 필요하지만, PAH의 임상 증상은 모호하며, 일반적으로 다른 많은 질환과 증상이 겹치기 때문에 진단이 늦어지거나 오진으로 이어지는 경우가 종종 있습니다. 지난 수십 년간 PAH의 병태생물학에 대한 이해가 깊어짐에 따라 그 정의는 변경되었지만, 인지도가 낮아 추가적인 어려움을 초래하고 있습니다.

폐동맥 고혈압 시장 규모 및 전망

  • 주요 7개국(G7)의 폐동맥 고혈압 시장 규모(2025년) : 약 80억 달러
  • PAH 성장률(2022-2036년) : 연평균 성장률(CAGR) 6.7%

본 폐동맥 고혈압 시장 보고서에서는 표준 치료, 임상 실무, 진화하는 치료 알고리즘 등 현재의 치료 현황에 대한 종합적인 분석을 제공합니다. PAH 환자의 부담 동향, 수익 및 시장 점유율 동향, 피크 시기의 환자 점유율 및 치료 도입 현황에 대한 분석을 평가함과 동시에, 주요 7개 국가 및 지역 전체에 걸친 상세한 시장 규모 평가 및 성장률 예측(과거 데이터 및 2022-2036년 예측)을 제시하고 있습니다. 본 보고서에서는 PAH 분야의 주요 미충족 수요 사항을 부각시키고, 경쟁 구도 및 임상 상황을 분석하여 고부가가치의 성장 기회를 도출함으로써, 향후 시장 성장 가능성에 대한 명확한 전망을 제시하고 있습니다.

폐동맥 고혈압(PAH)의 이해와 치료 알고리즘

PAH의 개요와 진단

폐동맥 고혈압(PAH)은 폐혈관 저항 및 동맥압의 상승을 특징으로 하며, 인구 100만 명당 약 15-60명이 이 질환을 앓고 있는 것으로 추정되며, 여성에서 더 많이 진단되고 있습니다. PAH는 호흡 곤란, 피로, 흉통, 무력감 등 다양한 비특이적 증상을 유발하며, 이 질환의 진행성 및 쇠약화시키는 특성으로 인해 발생하는 심각한 유병률 및 사망률과 관련이 있으며, 결국 우심부전이나 사망으로 이어집니다. 질병의 증상이 환자의 신체 활동 능력이나 정서 상태에 미치는 영향은 건강 관련 삶의 질(HRQoL)에 부정적인 영향을 미칩니다.

PAH는 심장이나 폐 질환에서 나타나는 증상과 유사한 증상을 특징으로 합니다. PAH 진단에는 흉부 X선 검사, 혈액 검사, 심장 카테터 검사, 심전도 검사, 심초음파 검사 등 여러 가지 검사 방법이 사용됩니다.

현재 폐동맥 고혈압(PAH)의 치료 현황

PAH 치료에 일반적으로 사용되는 4가지 약물군(PDE5 억제제, sGC 자극제, 엔도세린 수용체 길항제(ERA), 프로스타사이클린 유사체·작용제)은 프로스타사이클린, 엔도세린, 일산화질소라는 3가지 주요 신호 전달 경로를 표적으로 삼고 있습니다.

마시텐탄은 PAH 치료에 사용되는 엔도세린 수용체 길항제로, 폐혈관 저항을 낮춤으로써 운동 내성을 개선하고 증상을 완화합니다. 마시텐탄 요법의 최적 시작 시기나 용량 조절에 대해서는 여전히 불분명한 점이 있지만, 특히 진행성 사례의 경우, PAH 관리에 있어 중요한 선택지 중 하나로 간주되고 있습니다. 프로스타사이클린 계열 약물은 PAH 치료에 사용되는 가장 오랜 역사를 가진 약물군으로, cAMP 농도를 상승시켜 폐혈관 확장 작용을 발휘합니다. 에포프로스테놀은 PAH 치료 전용으로 최초로 도입된 약물로, 1995년에 미국 FDA(식품의약국)의 승인을 받았습니다. 그러나 외래 진료에서는 정맥 내 투여로만 제한되었고, 중심정맥 접근이 필요했기 때문에 그 범용성에는 한계가 있었습니다. 현재는 투여 경로가 개선된 여러 가지 프로스타사이클린 유도체가 존재합니다. 여기에는 흡입형 이프로프로스트 및 트레프로스티닐, 피하 또는 정맥 투여용 트레프로스티닐, 경구 투여용 트레프로스티닐·지오라민, 그리고 경구용 프로스타사이클린 작용제인 셀렉시파그가 포함됩니다.

폐동맥 고혈압(PAH)의 역학

PAH의 역학 분석 및 예측에 관한 주요 조사 결과

  • 2025년, 미국의 PAH 확진 환자 수는 약 4만 명으로 가장 많았으며, 2036년까지 더욱 증가할 것으로 예측됩니다.
  • 2025년, 미국에서는 특발성/유전성 PAH가 아형별 환자 수에서 가장 많은 비중을 차지하여 약 1만 9,000건에 달했으며, 2036년까지는 더욱 증가할 것으로 전망됩니다.
  • 2025년, EU4 국가 및 영국 중에서 독일이 PAH 확진 환자 수에서 가장 많았으며, 약 7,000건이 보고되었습니다.
  • 2025년, 일본에서는 여성 PAH 확진 환자 수가 약 1만 9,000명, 남성 환자 수가 약 5,000명으로 보고되었으며, 2036년까지 그 수가 증가할 것으로 예측되고 있습니다.

폐동맥 고혈압 시장 전망

현재의 치료법은 주로 폐혈관을 확장시켜 저항을 낮추고, 심장 기능을 개선하는 것을 목적으로 하고 있습니다. 치료 전략은 임상적, 기능적 및 혈역학적 매개변수를 바탕으로 한 위험도 평가를 기반으로 수립되며, 저위험 상태를 달성하고 생존율, 삶의 질, 증상 관리 등의 예후를 개선하는 것을 목표로 합니다. 주로 사용되는 약물의 분류로는 PDE5 억제제, sGC 자극제, 엔도세린 수용체 길항제, 그리고 프로스타사이클린 유사체/작용제가 있으며, 이들은 일산화질소, 엔도세린, 프로스타사이클린의 각 경로를 표적으로 합니다.

PAH 치료제로 시판되고 있는 약물은 몇 가지가 있으며, 각각 서로 다른 작용기전을 가지고 있습니다. TYVASO/TYVASO DPI/TREPROSTINIL, ORENITRAM 및 OPSYNVI/YUVANCI는 프로스타사이클린 경로를 표적으로 합니다. 소타텔셉트(WINREVAIR)는 신호 전달을 조절하여 혈관 기능을 개선합니다.

PAH 환자 치료를 위해 개발 중인 새로운 치료법에는 후기 및 중기 단계의 후보 약물이 포함됩니다. DelveInsight사의 추정 및 예측에 따르면, 예측 기간 동안 큰 변화를 가져올 가능성이 있는 약물로는 세랄티닙(GB-002), 트레프로스티닐 리포솜 제제(L-606), 라리네파그 등이 있습니다.

  • EU4 및 영국 시장 규모는 2025년에 약 4억 달러로 추정됩니다.
  • EU4 및 영국 중에서 2025년에는 독일이 약 1억 달러로 가장 큰 시장 점유율을 차지했으며, 그 뒤를 이어 프랑스와 영국이 각각 2025년에 약 8,000만 달러를 기록했습니다.
  • 2025년, 일본의 폐동맥 고혈압 시장 규모는 약 3억 달러였으며, 예측 기간(2026-2036년)에 걸쳐 확대될 것으로 전망됩니다.

약제 분류별/폐동맥 고혈압(PAH)의 주요 신흥 치료법에 대한 인사이트(2022-2036년 전망)

저분자 화합물 : 저분자 화합물을 이용한 치료법은 질환의 진행에 관여하는 주요 분자 경로를 정확하게 표적으로 삼을 수 있다는 특징 덕분에, 폐동맥 고혈압(PAH) 관리에서 점점 더 중요한 역할을 하고 있습니다. 이러한 저분자량 덕분에, 특히 흡입을 통한 효율적인 전달이 가능해지며, 전신 노출을 최소화하면서도 폐혈관계로의 약물 직접 침착을 보장합니다. 이러한 표적 중심의 접근 방식을 통해 혈관 수축, 혈관 증식, 리모델링과 같은 중요한 기전을 조절할 수 있게 됩니다. 세랄티닙(GB002)과 같은 약물은 PDGF 수용체 신호 전달을 억제하여 혈관 리모델링을 억제함으로써 이러한 전략을 구현하고 있습니다. 마찬가지로, 휴대용 건식 분말 흡입기를 통해 투여되는 트레프로스티닐 흡입 제제는 편의성과 국소적 효능을 높여주고 있습니다.

재조합 융합 단백질 : 재조합 융합 단백질은 폐동맥 고혈압(PAH)에 대한 첨단 생물학적 치료법 중 하나로, 치료 효과를 높이기 위해 서로 다른 단백질의 기능 도메인을 결합하여 설계되었습니다. 이러한 약물은 혈관 리모델링 및 비정상적인 세포 증식에 관여하는 주요 신호 전달 경로를 표적으로 하여, 기존의 혈관 확장제를 뛰어넘는 질환 수정적 접근법을 제공합니다. 대표적인 예로, 액티빈 신호 전달 억제제인 소타텔셉트(WINREVAIR)를 들 수 있습니다. 이는 폐혈관계에서 증식 촉진 경로와 증식 억제 경로의 균형을 회복시키는 것입니다.

자주 묻는 질문

  • 2025년 폐동맥 고혈압(PAH) 시장 규모는 어떻게 되나요?
  • 폐동맥 고혈압(PAH)의 연평균 성장률(CAGR)은 어떻게 되나요?
  • 2025년 미국 내 PAH 유병 사례 수는 얼마인가요?
  • PAH 치료에 사용되는 주요 약물군은 무엇인가요?
  • 2025년 일본의 PAH 확진 환자 수는 어떻게 되나요?
  • PAH 치료에 대한 주요 목표는 무엇인가요?
  • 2025년 EU4 및 영국의 PAH 시장 규모는 어떻게 되나요?

목차

제1장 주요 인사이트

제2장 서론

제3장 폐동맥 고혈압 : 시장 개요

제4장 PAH 역학 및 시장에 관한 조사 방법

제5장 폐동맥 고혈압 : 주요 요약

제6장 주요 이벤트

제7장 질환 배경 및 개요

제8장 관리와 치료

제9장 역학 및 예측 조사 방법

제10장 환자 경과

제11장 경쟁 구도 : 시판약

제12장 경쟁 구도 : 신흥 의약품

제13장 시장 분석

제14장 KOL(Key Opinion Leader)의 견해

제15장 SWOT 분석

제16장 미충족 수요

제17장 시장 참여 및 상환

제18장 부록

제19장 DelveInsight의 서비스 내용

제20장 면책사항

KTH

Pulmonary Arterial Hypertension (PAH) Insights and Trends

  • According to DelveInsight's analysis, PAH market size was ~USD 8 billion in the leading markets (the United States, the EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan) in 2025.
  • PAH reported approximately 50,000 prevalent cases and around 44,000 diagnosed prevalent cases in the US in 2025.
  • Epidemiological understanding of Pulmonary Arterial Hypertension remains limited due to outdated, regionally variable data, inconsistent diagnostic methodologies, and frequent underdiagnosis driven by non-specific early symptoms.
  • The main purpose of the treatment of PAH is to improve patients' symptoms and slow the rate of clinical deterioration. General measures, supportive therapy, pharmacological treatment, and surgical treatment are used for treating PAH.
  • The treatment landscape of Pulmonary Arterial Hypertension is anchored by multiple approved therapies across functional classes, including selexipag (UPTRAVI), treprostinil (TYVASO), and riociguat (ADEMPAS), among others. Current standard of care is driven by targeted agents, PDE5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists (ERAs), and prostacyclin pathway therapies-which collectively modulate the key pathogenic pathways.
  • Various therapies that are expected to enter the market during the forecast period include ralinepag (United Therapeutics), RT234 (vardenafil inhalation powder) (Respira Therapeutics), seralutinib (GB002) (Gossamer Bio), and L606 (liposomal treprostinil) (Pharmosa Biopharm/Liquidia).
  • PAH is a rare, highly complex, and progressive disorder that is incurable and often leads to premature death. Early diagnosis is required for the best outcomes; however, the clinical presentation of PAH is ambiguous and usually overlaps with numerous other illnesses, often leading to a delay in diagnosis or misdiagnosis. In the past decades, an increased understanding of the pathobiology of PAH has led to changes in its definition, but a lack of awareness adds to the difficulty.

Pulmonary Arterial Hypertension (PAH) Market size and forecast

  • 2025 PAH Market Size in the 7MM: ~USD 8 Billion
  • PAH Growth Rate (2022-2036): 6.7% CAGR (Compound Annual Growth Rate)

DelveInsight's "Pulmonary Arterial Hypertension (PAH) - Market Insights, Epidemiology, and Market Forecast - 2036" report delivers an in-depth understanding of PAH, historical and forecasted epidemiology, as well as the PAH market trends in the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.

The PAH market report delivers a comprehensive analysis of the current treatment landscape, including standards of care, clinical practices, and evolving therapeutic algorithms. It evaluates, PAH patient burden trends, revenue & market share dynamics, peak patient share & therapy uptake analysis, and provides an in-depth market size assessment, and growth rate projections (Historical & Forecast 2022-2036) across the 7MM regions. The report highlights key unmet medical needs in PAH and maps the competitive and clinical landscape to uncover high-value opportunities, providing a clear outlook on future market growth potential.

Pulmonary Arterial Hypertension (PAH) Understanding and Treatment Algorithm

PAH Overview and Diagnosis

Pulmonary arterial hypertension (PAH), characterized by increased pulmonary vascular resistance and arterial pressure, affects an estimated 15-60 per million of the population and is more commonly diagnosed in women. PAH causes a range of nonspecific symptoms (including breathlessness, fatigue, chest pain, and weakness) and is associated with significant morbidity and mortality triggered by the debilitating progressive nature of the disease, which eventually leads to right heart failure and death. The effect of disease symptoms on the patient's physical mobility and emotional state adversely affects health-related quality of life (HRQoL).

PAH is characterized by similar symptoms that appear during heart and lung conditions. Several techniques and blood tests, such as chest X-rays, blood tests, cardiac catheterization, electrocardiograms, and echocardiograms, are used for diagnosing PAH.

Current Pulmonary Arterial Hypertension (PAH) Treatment Landscape

The four drug classes commonly used to treat PAH-PDE5 inhibitors, sGC stimulators, Endothelin Receptor Antagonists (ERAs), and prostacyclin analogs and agonists-target three major signaling pathways: prostacyclin, endothelin, and nitric oxide.

Macitentan is an endothelin receptor antagonist used to treat PAH, improving exercise capacity and reducing symptoms by lowering pulmonary vascular resistance. While the optimal initiation and adjustment of macitentan therapy remain unclear, it is considered a key option for managing PAH, especially for advanced cases. Prostacyclin medications are the oldest group used to treat PAH; these increase cAMP concentrations to produce pulmonary vasodilation. Epoprostenol was the first drug introduced specifically for treating PAH and was approved by the US FDA in 1995. However, it was limited in its versatility by its restriction to IV use in the outpatient setting, requiring central venous access. There are several different prostacyclin derivatives with various modes of delivery that are improved; these include inhaled iloprost and treprostinil, SC or IV treprostinil, oral treprostinil diolamine, and an oral prostacyclin agonist selexipag.

Pulmonary Arterial Hypertension (PAH) Unmet Needs

The section "unmet needs of PAH" outlines the critical gaps between the current state of patient care, diagnosis, and the ideal & effective management of the disease. It highlights the obstacles experienced by patients, clinicians, and researchers and identifies potential solutions for future progress.

1. Scarce epidemiology data

2. Biomarker based diagnosis

3. Curative targeted therapies

4. Early diagnosis and awareness

5. Comprehensive treatment algorithm and others.....

Pulmonary Arterial Hypertension (PAH) Epidemiology

Key Findings from PAH Epidemiological Analysis and Forecast

  • In 2025, the US accounted for the highest diagnosed prevalent cases of PAH with approximately 40,000 cases, which are expected to increase by 2036.
  • Idiopathic/heritable PAH had the highest number of subtype-specific cases in the US in 2025 with nearly (19,000 cases), which is predicted to rise by 2036.
  • In 2025, the Germany reported the highest number of diagnosed prevalent cases of PAH among EU4 and the UK, with approximately 7,000 cases.
  • In 2025, Japan reported nearly 19,000 diagnosed prevalent cases of PAH in females and nearly 5,000 cases in males, with numbers projected to rise by 2036.

Pulmonary Arterial Hypertension (PAH) Drug Analysis & Competitive Landscape

The PAH drug chapter provides a detailed, market-focused review of approved therapies and the emerging pipeline across Phase I-III clinical trials. It covers mechanism of action, clinical trial data, regulatory approvals, patents, collaborations, strategic partnerships upcoming key catalyst for each therapy, along with their advantages, limitations, and recent developments. This section offers critical insights into the PAH treatment landscape, supporting market assessment, competitive analysis, and growth forecasting for the PAH therapeutics market.

Approved Therapies for Pulmonary Arterial Hypertension (PAH)

Inhaled treprostinil (YUTREPIA): Liquidia Technologies

Treprostinil (YUTREPIA), developed by Liquidia Technologies, is an inhaled dry powder formulation of treprostinil delivered through a convenient, palm-sized device designed using Liquidia's PRINT technology to enhance deep-lung delivery to treat PAH.

  • In May 2025, FDA authorized YUTREPIA as a new therapeutic option for adults with PAH (WHO Group 1) and PH ILD (WHO Group 3).

Pulmonary Arterial Hypertension (PAH) Pipeline Analysis

Ralinepag: United Therapeutics

Ralinepag is a novel, oral, selective, and potent prostacyclin receptor agonist being developed by United Therapeutics for the treatment of PAH. In vitro studies indicate that ralinepag has high binding affinity and selectivity at the human prostacyclin (IP) receptor. In phase II studies, ralinepag demonstrated a potential for a once-a-day dosing profile and potentially enhanced affinity compared to selexipag.

  • In March 2026, United Therapeutics announced that ralinepag had achieved a 55% reduction in the risk of clinical worsening in a pivotal PAH study, delivering exceptional and highly statistically significant efficacy.

Pulmonary Arterial Hypertension (PAH) Key Players, Market Leaders and Emerging Companies

  • United Therapeutics
  • Mochida Pharmaceutical
  • Johnson & Johnson
  • Merck
  • Gossamer Bio
  • Pharmosa Biopharm/ Liquidia
  • Respira Therapeutics And more

Pulmonary Arterial Hypertension (PAH) Drug Updates

  • United Therapeutics intends to submit a New Drug Application (NDA) for ralinepag to the US Food and Drug Administration (FDA) by the second half of 2026.
  • In February 2026, Gossamer Bio announced topline Phase III results for seralutinib, an investigational inhaled therapy for adults with PAH.
  • In May 2025, the FDA officially approved Liquidia Corporation's treprostinil inhalation powder (YUTREPIA) for the treatment of PAH and pulmonary hypertension associated with interstitial lung disease (PH ILD) in adults.

Pulmonary Arterial Hypertension (PAH) Market Outlook

Current therapies primarily work by dilating pulmonary blood vessels to reduce resistance and improve heart function, with treatment strategies guided by risk assessment based on clinical, functional, and hemodynamic parameters to achieve a low-risk status and improve outcomes such as survival, quality of life, and symptom control. The main drug classes used include PDE5 inhibitors, sGC stimulators, endothelin receptor antagonists, and prostacyclin analogs/agonists, which target the nitric oxide, endothelin, and prostacyclin pathways.

There are several marketed drugs for PAH, each with distinct mechanisms. TYVASO/TYVASO DPI/TREPROSTINIL, ORENITRAM, and OPSYNVI/YUVANCI target prostacyclin pathways. Sotatercept (WINREVAIR) modulates signaling to improve vascular function.

The emerging therapies developed to treat PAH patients include late-stage and mid-stage candidates. As per DelveInsight's estimates, the potential drugs that can mark a significant change in the forecast period includes seralutinib (GB-002), treprostinil liposomal (L-606), Ralinepag and others.

  • The total market size of EU4 and the UK was estimated to be approximately USD 400 million in 2025.
  • Among EU4 and the UK, Germany accounted for the highest market share with approximately USD 100 million in 2025, followed by France and the UK with approximately USD 80 million in the respective year in 2025.
  • In 2025, the total market size of PAH was approximately USD 300 million in Japan which is anticipated to increase during the forecast period (2026-2036).

Drug Class/Insights into Leading Emerging Therapies in Pulmonary Arterial Hypertension (PAH) (2022-2036 Forecast)

Small Molecule: Small-molecule therapies are playing an increasingly pivotal role in the management of pulmonary arterial hypertension (PAH), driven by their ability to precisely target key molecular pathways involved in disease progression. Their low molecular weight enables efficient delivery, particularly via inhalation, ensuring direct drug deposition in the pulmonary vasculature while minimizing systemic exposure. This targeted approach allows modulation of critical mechanisms such as vasoconstriction, vascular proliferation, and remodeling. Agents like seralutinib (GB002) exemplify this strategy by inhibiting PDGF receptor signaling to address vascular remodeling. Similarly, inhaled formulations of treprostinil, delivered through portable dry powder inhalers, enhance convenience and localized efficacy.

Recombinant fusion proteins: Recombinant fusion proteins represent an advanced class of biologic therapies in pulmonary arterial hypertension (PAH), engineered by combining functional domains of different proteins to enhance therapeutic activity. These agents target key signaling pathways involved in vascular remodeling and abnormal cell proliferation, offering a disease-modifying approach beyond conventional vasodilators. A leading example is sotatercept (WINREVAIR), an activin signaling inhibitor that restores the balance between pro- and anti-proliferative pathways in the pulmonary vasculature.

Pulmonary Arterial Hypertension (PAH) Drug Uptake

This section focuses on the uptake rate of potential drugs expected to be launched in the market during the forecast period (2026-2036). The analysis covers the PAH drug's uptake, performance at peak, factors affecting performance during prime years of growth, patient uptake by therapy, and anticipated sales generated by each drug.

The current mainstay therapies to treat PAH act to dilate the pulmonary vasculature, decreasing pulmonary vascular resistance and secondarily improving right ventricular function, thereby improving functional capacity. The overall treatment goal is to improve survival, quality of life, exercise capacity, symptom burden, and clinical worsening, with risk stratification tools increasingly used to guide therapy and improve each of these elements.

Vardenafil (RT234), developed by Respira Therapeutics, is a first-in-class inhaled, as-needed (PRN) therapy for PAH that delivers vardenafil via a novel Axial Oscillating Sphere (AOS) dry powder inhaler, enabling rapid pulmonary vasodilation and on-demand relief of exertional breathlessness while improving exercise tolerance. Positioned as a complementary option to chronic background therapies, RT234 is expected to see moderate adoption, with an estimated ~7 years to peak uptake.

Detailed insights of emerging therapies' drug uptake is included in the report

Market Access and Reimbursement of therapies in Pulmonary Arterial Hypertension (PAH)

The report further provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenario of 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.

Reimbursement is a crucial factor that affects the drug's access to the market. Often, the decision to reimburse comes down to the price of the drug relative to the benefit it produces in treated patients. To reduce the healthcare burden of these high-cost therapies, many payment models are being considered by payers and other industry insiders.

NOTE: Further Details are provided in the final report....

Pulmonary Arterial Hypertension (PAH) therapies Price Scenario & Trends

Pricing and analogue assessment of PAH therapies highlights evolving price dynamics structures. This section summarizes the cost of approved treatments, closest and most appropriate analogue selection for emerging therapies, and understanding of how pricing influences market access, adherence, and long-term uptake.

  • Pricing of PAH Approved Drugs

Selexipag (UPTRAVI) is an oral, selective prostacyclin IP receptor agonist approved for the treatment of PAH, more than three-quarters (76%) of PAH patients treated with UPTRAVI (selexipag) either maintained (56%) or reduced (20%) their 1-year mortality risk score. It is associated with a relatively high annual treatment cost in the United States, estimated at approximately USD 260,000.

Industry Experts and Physician Views for Pulmonary Arterial Hypertension (PAH)

To keep up with PAH market trends, we take Key Opinion Leaders (KOLs) and Subject Matter Experts (SMEs) opinions working in the domain through primary research to fill the data gaps and validate our secondary research. Industry Experts were contacted for insights on the PAH emerging therapies, evolving treatment landscape, patient adherence to conventional therapies, therapy switching trends, drug adoption and uptake, accessibility challenges, and epidemiology and real-world prescription patterns in PAH, including MD, PhD, Instructor, Pos tdoctoral Researcher, Professor, Researcher, and others.

DelveInsight's analysts connected with 8+ KOLs to gather insights at country level. Centers such as the University of Pennsylvania Hospital, University of Chicago, University of Pittsburgh, Medizinische Hochschule Hannover etc. were contacted.

Qualitative Analysis: SWOT and Attribute Analysis

We perform qualitative and market Intelligence analysis using various approaches, such as SWOT analysis and attribute analysis.

In the SWOT analysis of PAH, strengths, weaknesses, opportunities, and threats in terms of disease diagnosis, patient awareness, patient burden, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provide attribute analysis analyzes emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, route of administration, and order of entry. Scoring is given based on these parameters to analyze the effectiveness of therapy.

The team of analysts analyzes promising 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, whereas the therapies' safety is evaluated, wherein the acceptability, tolerability, and adverse events are majorly observed. In addition, the scoring is also based on the route of administration, order of entry, probability of success, and the addressable patient pool for each therapy. According to these parameters, the final weightage score and the ranking of the emerging therapies are decided.

Scope of the Report:

  • The report covers a segment of key events, an executive summary, and a descriptive overview of PAH, explaining their causes, signs and symptoms, pathogenesis, and currently available treatments.
  • Comprehensive insight has been provided into the epidemiology segments and forecasts, the future growth potential of the diagnosis rate, and disease progression along treatment guidelines.
  • Additionally, an all-inclusive account of both the current and emerging treatments, along with the elaborative profiles of late-stage and prominent therapies, will have an impact on the current treatment landscape.
  • A detailed review of the PAH market, historical and forecasted market size, market share by therapies, detailed assumptions, and rationale behind our approach is included in the report, covering the 7MM drug outreach.
  • The report provides an edge while developing business strategies by understanding trends through SWOT analysis and expert insights/KOL views, patient journey, and treatment preferences that help in shaping and driving the 7MM PAH market.

Report Insights

  • PAH Patient Population Forecast
  • PAH Therapeutics Market Size
  • PAH Pipeline Analysis
  • PAH Market Size and Trends
  • PAH Market Opportunity (Current and Forecasted)

Report Key Strengths

  • Epidemiology-based (Epi-based) bottom-up forecasting
  • Artificial Intelligence (AI) - enabled market research report
  • 11-year forecast
  • PAH Market Outlook (North America, Europe, Asia-Pacific)
  • Patient Burden Trends (by geography)
  • PAH Treatment Addressable Market (TAM)
  • PAH Competitive Landscape
  • PAH Major Companies Insights
  • PAH Price Trends and Analogue Assessment
  • PAH Therapies Drug Adoption/Uptake
  • PAH Therapies Peak Patient Share analysis

Report Assessment

  • PAH Current Treatment Practices
  • PAH Unmet Needs
  • PAH Clinical Development Analysis
  • PAH Emerging Drugs Product Profiles
  • PAH Market Attractiveness
  • PAH Qualitative Analysis (SWOT and attribute analysis)

FAQs:

Market Insights

  • What was the PAH market size, the market size by therapies, market share (%) distribution in 2025, and what would it look like by 2036? What are the contributing factors for this growth?
  • What are the anticipated pricing variations among different geographies for the emerging therapies in the future?
  • What can be the future treatment paradigm of PAH?
  • What are the disease risks, burdens, and unmet needs of PAH? What will be the growth opportunities across the 7MM concerning the patient population with PAH?
  • Who is the major future competitor in the market, and how will the competitors affect their market share?
  • What are the current options for the treatment of PAH? What are the current guidelines for treating PAH in the US, Europe, and Japan?

Reasons to Buy:

  • The report will help in developing business strategies by understanding the latest trends and changing treatment dynamics driving the PAH market.
  • Bottom up forecasting builds from the affected population to product forecasts, delivering a robust, data driven approach ideal for new therapies and novel classes.
  • 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.
  • Understand the existing market opportunities in varying geographies and the growth potential over the coming years.
  • Identifying strong upcoming players in the market will help devise strategies to help get 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.
  • To understand KOLs' perspectives on 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.
  • This Artificial Intelligence (AI) enabled report summarize and simplify complex datasets with in the report into clear, actionable insights for stakeholders, investors, and healthcare providers, enabling faster, data driven decisions.

Table of Contents

1. Key Insights

2. Report Introduction

3. Pulmonary Arterial Hypertension Market Overview at a Glance

  • 3.1. Market Share (%) Distribution of PAH in 2025 in the 7MM
  • 3.2. Market Share (%) Distribution of PAH in 2036 in the 7MM

4. Methodology of PAH Epidemiology and Market

5. Executive Summary of PAH

6. Key Events

7. Disease Background and Overview

  • 7.1. Introduction to PAH
  • 7.2. Signs and Symptoms
  • 7.3. Classification of PAH
    • 7.3.1. WHO classification
    • 7.3.2. Functional Classification of PAH
  • 7.4. Etiology
  • 7.5. Risk factors
  • 7.6. Pathophysiology
    • 7.6.1. Nitric oxide (NO) pathway
    • 7.6.2. Prostacyclin-thromboxane A2 pathway
    • 7.6.3. Endothelin-1 pathway
  • 7.7. Diagnosis

8. Management and Treatment

  • 8.1. Treatment Guidelines
    • 8.1.1. American College of Chest Physicians (CHEST) Guidelines
    • 8.1.2. European Society of Cardiology (ESC)/the European Respiratory Society (ERS) Guidelines for the Treatment of Pulmonary Hypertension

9. Epidemiology and Market Forecast Methodology

  • 9.1. Key Findings
  • 9.2. Assumptions and Rationale: The 7MM
    • 9.2.1. Total Prevalent Cases of PAH
    • 9.2.2. Total Diagnosed Prevalent Cases of PAH
    • 9.2.3. Age-specific Diagnosed Prevalent cases of PAH
    • 9.2.4. Gender-specific Diagnosed Prevalent cases of PAH
    • 9.2.5. Class-specific Diagnosed Prevalent Cases of PAH
    • 9.2.6. Subtype-specific Diagnosed Prevalent cases of PAH
  • 9.3. Total Prevalent Cases of PAH in 7MM
  • 9.4. Total Diagnosed Cases of PAH in 7MM
  • 9.5. Total Treated Cases of PAH in 7MM
  • 9.6. The United States
    • 9.6.1. Total Prevalent Cases of PAH in the US
    • 9.6.2. Total Diagnosed Prevalent Cases of PAH in the US
    • 9.6.3. Age-specific Cases of PAH in the US
    • 9.6.4. Class-specific Cases of PAH in the US
    • 9.6.5. Gender-specific Cases of PAH in the US
    • 9.6.6. Subtype-specific Cases of PAH in the US
    • 9.6.7. Total Treated Cases of PAH in the US
  • 9.7. EU4 and the UK
    • 9.7.1. Total Prevalent Cases of PAH in EU4 and the UK
    • 9.7.2. Total Diagnosed Prevalent Cases of PAH in EU4 and the UK
    • 9.7.3. Age-specific Cases of PAH in EU4 and the UK
    • 9.7.4. Class-specific Cases of PAH in EU4 and the UK
    • 9.7.5. Gender-specific Cases of PAH in the EU4 and the UK
    • 9.7.6. Subtype-specific Cases of PAH in the EU4 and the UK
    • 9.7.7. Total Treated Cases of PAH in EU4 and the UK
  • 9.8. Japan
    • 9.8.1. Total Prevalent Cases of PAH in the Japan
    • 9.8.2. Total Diagnosed Prevalent Cases of PAH in the Japan
    • 9.8.3. Age-specific Cases of PAH in the Japan
    • 9.8.4. Class-specific Cases of PAH in the Japan
    • 9.8.5. Gender-specific Cases of PAH in the Japan
    • 9.8.6. Subtype-specific Cases of PAH in the Japan
    • 9.8.7. Total Treated Cases of PAH in the Japan

10. Patient Journey

11. Competitive Landscape: Marketed Drugs

  • 11.1. Key Cross Competition
  • 11.2. Inhaled treprostinil (YUTREPIA): Liquidia Technologies
    • 11.2.1. Product Description
    • 11.2.2. Regulatory Milestone
    • 1.1.1. Other Developmental Activities
    • 11.2.3. Clinical Developmental Activities
    • 11.2.4. Safety and Efficacy
  • 11.3. Treprostinil (TYVASO/TYVASO DPI/TREPROST Inhalation Solution): United Therapeutics/Mochida Pharmaceutical
    • 11.3.1. Product Description
    • 11.3.2. Regulatory Milestone
    • 11.3.3. Other Developmental Activities
    • 11.3.4. Clinical Developmental Activities
    • 11.3.5. Safety and Efficacy
  • 11.4. Macitentan and tadalafil (OPSYNVI/YUVANCI): Johnson & Johnson
    • 11.4.1. Product Description
    • 11.4.2. Regulatory Milestones
    • 11.4.3. Other Developmental Activities
    • 11.4.4. Clinical Developmental Activities
    • 11.4.5. Safety and Efficacy

12. Competitive Landscape: Emerging Drugs

  • 12.1. Key Cross
  • 12.2. Ralinepag: United Therapeutics
    • 12.2.1. Product Description
    • 12.2.2. Other Developmental Activities
    • 12.2.3. Clinical Developmental Activities
    • 12.2.4. Safety and Efficacy
    • 12.2.5. Analysts' Views
  • 12.3. Seralutinib (GB002): Gossamer Bio
    • 12.3.1. Product Description
    • 12.3.2. Other Developmental Activities
    • 12.3.3. Clinical Developmental Activities
    • 12.3.4. Safety and Efficacy
    • 12.3.5. Analysts' Views
  • 12.4. Vardenafil (RT234): Respira Therapeutics
    • 12.4.1. Product Description
    • 12.4.2. Other Developmental Activities
    • 12.4.3. Clinical Developmental Activities
    • 12.4.4. Safety and Efficacy
    • 12.4.5. Analysts' Views
  • 12.5. L606 (liposomal treprostinil): Pharmosa Biopharm/Liquidia
    • 12.5.1. Product Description
    • 12.5.2. Other Developmental Activities
    • 12.5.3. Clinical Developmental Activities
    • 12.5.4. Safety and Efficacy
    • 12.5.5. Analysts' Views

13. Market Analysis

  • 13.1. Key Findings
  • 13.2. Market Outlook
  • 13.3. Conjoint Analysis
  • 13.4. Key Market Forecast Assumptions
  • 13.5. Total Market Size of PAH in the 7MM
  • 13.6. The United States
    • 13.6.1. Total Market Size of PAH in the United States
    • 13.6.2. Market Size of PAH by Therapies in the United States
  • 13.7. EU4 and the UK
    • 13.7.1. Total Market Size of PAH in EU4 and the UK
    • 13.7.2. Market Size of PAH by Therapies in EU4 and the UK
  • 13.9. Japan
    • 13.9.1. Total Market Size of PAH in Japan
    • 13.9.2. Market Size of Pulmonary Arterial Hypertension by Therapies in Japan

14. KOL Views

15. SWOT Analysis

16. Unmet Needs

17. Market Access and Reimbursement

  • 17.1. The United States
    • 17.1.1. Center for Medicare & Medicaid Services (CMS)
  • 17.2. EU4 and the UK
    • 17.2.1. Germany
    • 17.2.2. France
    • 17.2.3. Italy
    • 17.2.4. Spain
    • 17.2.5. The United Kingdom
  • 17.3. Japan
    • 17.3.1. MHLW

18. Appendix

  • 18.1. Bibliography
  • 18.2. Report Methodology

19. DelveInsight Capabilities

20. Disclaimer

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