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간질성 폐질환 관련 폐고혈압(PH-ILD) : 시장 인사이트, 역학 및 시장 예측(2036년)

Pulmonary Hypertension associated with Interstitial Lung Disease (PH-ILD) - Market Insight, Epidemiology, and Market Forecast - 2036

발행일: | 리서치사: 구분자 DelveInsight | 페이지 정보: 영문 190 Pages | 배송안내 : 2-10일 (영업일 기준)

    
    
    




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간질성 폐질환 관련 폐고혈압(PH-ILD)에 대한 인사이트와 동향

  • DelveInsight의 분석에 따르면, 주요 7개국의 PH-ILD 시장은 2025년에 약 30억 달러 규모에 달했으며, 첨단 치료법의 도입과 임상 관리 개선을 배경으로 2036년까지 연평균 성장률(CAGR) 3.9%로 확대될 것으로 전망됩니다.
  • 2025년 기준으로, 미국 내 PH-ILD 확진 환자 수는 약 8만 3,000명에 달하며, 주요 7개국 중 가장 많은 환자 수를 차지하고 있습니다. 한편, EU4 및 영국에서는 약 7만 건이 확인되었으며, 이로 인해 전문적인 치료법에 대한 수요가 크게 증가하고 있습니다.
  • 현재의 표준 치료법에는 트레프로스티닐(YUTREPIA) 및 트레프로스티닐(TYVASO/TYVASO DPI/TREPROST) 등의 승인된 흡입 요법이 포함되어 있으며, 이들은 중증 폐혈관 합병증을 가진 환자의 증상 관리와 삶의 질(QoL) 향상에 중점을 두고 있습니다.
  • PH-ILD의 파이프라인은 차세대 흡입제 및 경구제로 전환되고 있으며, 트레프로스티닐 리포솜(L606) 및 트레프로스티닐 팔미틸 흡입 분말(TPIP)과 같은 후기 개발 단계의 약물에 더해, 모슬리시구아트, 세랄티닙, H1614(HB-1614)와 같은 신흥 후보 약물들이 예측 기간 동안 치료 방식을 혁신하고 시장 확대를 주도할 것으로 기대됩니다.
  • PH-ILD에 대한 연구는 지속적으로 발전하고 있으며, 높은 유병률과 사망률에 대처하고, 다양한 환자 집단에 대해 보다 정확한 진단과 최신 치료 지침을 확보하기 위해서는 지속적인 임상시험이 필요하다는 점이 강조되고 있습니다.
  • PH-ILD의 경우, 간질성 폐질환(ILD)과 증상이 중복되어 오진이 빈번하게 발생하기 때문에 진단 지연으로 인해 여전히 치료에 큰 격차가 존재하고 있습니다. 우심 카테터 검사 등 침습적 시술에 대한 의존도 역시 조기 발견을 더욱 저해하고 있으며, 환자의 예후 개선과 적시적 개입을 실현하기 위한 비침습적 바이오마커 및 첨단 영상 진단 기술에 대한 시급하고 충족되지 않은 수요가 부각되고 있습니다.
  • PH-ILD 시장의 주요 성장 요인은 이 질환에 대한 인식 제고와 진단 방법의 개선입니다. 이를 통해 조기 발견 및 치료 시작이 가능해짐에 따라, 치료 가능한 환자층이 확대될 것으로 예상되며, 기존 치료법 및 개발 중인 치료법에 대한 수요를 견인할 것으로 전망됩니다.

간질성 폐질환 관련 폐고혈압(PH-ILD)의 시장 규모 및 전망

  • 2025년 주요 7개국(G7)의 PH-ILD 시장 규모 : 30억 달러
  • 주요 7개국 PH-ILD 성장률(2026-2036년) : 연평균 성장률(CAGR) 3.9%

본 PH-ILD 시장 보고서는 표준 치료, 임상 실무, 진화하는 치료 알고리즘을 포함하여 현재의 치료 현황에 대한 종합적인 분석을 제공합니다. PH-ILD 환자의 부담 동향, 수익 및 시장 점유율 동향, 피크 시기의 환자 점유율 및 치료법 보급 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 제공합니다. 본 보고서에서는 PH-ILD 분야의 주요 미충족 의료 수요를 부각시키고, 경쟁 구도 및 임상 환경을 분석하여 고부가가치의 성장 기회를 도출하는 한편, 향후 시장 성장 가능성에 대한 명확한 전망을 제시하고 있습니다.

대상 지역:

북미 : 미국

유럽 : 독일, 프랑스, 이탈리아, 스페인, 영국

아시아태평양 : 일본

간질성 폐질환 관련 폐고혈압(PH-ILD)의 이해와 치료 알고리즘

간질성 폐질환 관련 폐고혈압(PH-ILD)의 개요와 진단

PH-ILD는 광범위한 폐 질환을 지칭하며, 그 대부분은 폐포와 주변 혈관 사이에 위치한 얇은 조직층에서 섬유화나 염증을 동반합니다. 간질(인터스티티움)로 알려진 이 미세한 영역은 두께가 불과 수 마이크로미터에 불과하지만, 폐포의 구조를 유지하고 혈액으로의 원활한 산소 전달을 가능하게 하는 매우 중요한 역할을 수행하고 있습니다. PH-ILD 발병 위험을 높이는 요인은 몇 가지가 있습니다. 만성적인 흡연이나 환경적·직업적 유해 물질에 장기간 노출되면 폐 조직에 손상을 입히고 염증과 섬유화를 유발하며, 그 결과 폐동맥압이 상승하게 됩니다.

PH-ILD를 진단하기 위해서는 비침습적 검사와 침습적 검사를 병행해야 합니다. ILD 환자에서 원인을 알 수 없는 호흡곤란이 악화되거나, 폐 질환의 진행 단계에 비해 과도한 증상이 나타나는 경우에는 PH에 대한 추가적인 평가가 권장됩니다. ILD와 PH에는 많은 공통된 증상이 있기 때문에 이 둘을 구별하기 어려운 경우가 있습니다. 진단을 지원하기 위해 의료진은 종종 폐기능 검사, 6분 보행 검사(6MWD), 심장초음파 검사, 그리고 흉부 X선이나 CT 스캔과 같은 영상 검사 등 비침습적 검사 방법에 의존하고 있습니다. 그러나 PH-ILD의 확정 진단에 있어서는 우심실 카테터 검사(RHC)가 여전히 가장 정확하고 결정적인 방법으로 여겨지고 있습니다.

간질성 폐질환 관련 폐고혈압(PH-ILD)의 치료 현황

PH-ILD의 치료는 주로 기저 폐질환의 관리에 중점을 두고 있으나, 특이적으로 승인된 치료법이 부족한 점이 치료 표준화를 위한 큰 과제로 대두되고 있습니다. 현재 치료의 주축을 이루고 있는 것은 트레프로스티닐(YUTREPIA)이나 트레프로스티닐(TYVASO/TYVASO DPI/TREPROST) 등 승인된 흡입 요법이며, 이러한 치료법은 증상 관리와 삶의 질 향상에 중점을 두고 있습니다. 이러한 선택지가 있음에도 불구하고, 치료상의 격차가 여전히 존재하기 때문에 많은 임상의들은 PH-ILD 환자의 거의 절반에게 폐동맥 고혈압(PAH) 적응증으로 승인된 약물을 비승인 용도로 처방하고 있다고 보고하고 있습니다. 그러나 트레프로스티닐 리포솜 제제(L606)나 트레프로스티닐 팔미틸 흡입 분말(TPIP)과 같은 개발 후기 단계의 파이프라인 후보들이 등장함에 따라, 이러한 상황은 변화하고 있습니다.

간질성 폐질환 관련 폐고혈압(PH-ILD)의 역학

간질성 폐질환 관련 폐고혈압(PH-ILD)의 역학 분석 및 예측에 관한 주요 조사 결과

  • 2025년 기준으로, 주요 7개국에서 PH-ILD로 진단받은 환자 수는 총 약 18만 2,000명으로 추정되며, 이는 이 질환이 초래하는 막대한 부담을 반영하고 있습니다.
  • PH-ILD 외에도, 주요 7개국에서 간질성 폐질환(ILD)으로 진단받은 환자 수는 훨씬 더 많으며, 2025년에는 약 100만 건 이상에 달할 것으로 예상되어, 관련 폐고혈압을 발병할 위험이 있는 환자가 다수 존재한다는 사실이 부각되고 있습니다.
  • 2025년, 미국은 PH-ILD 시장에서 가장 큰 점유율을 차지했으며, 첨단 진단 인프라와 높은 임상적 인식에 힘입어 진단된 환자 수는 약 8만 3,000명에 달했습니다.
  • 2025년, EU 4개국과 영국을 합친 PH-ILD 환자 수는 약 7만 건이었으며, 그중 영국은 유럽 내에서 가장 큰 비중을 차지했고, PH-ILD로 진단받은 환자 수는 약 1만 8,000건에 달했습니다.
  • 2025년, 일본에서는 약 29,000건의 PH-ILD 확진 사례가 확인되었으며, 이는 아시아 시장에서 안정적이고 임상적으로 중요한 환자 기반을 형성하고 있습니다.
  • 2025년 미국의 성별 분석에 따르면, 남성의 유병률이 약간 더 높아 약 43,000건인 반면, 여성은 약 40,000건인 것으로 나타났습니다.

간질성 폐질환 관련 폐고혈압(PH-ILD) 시장의 전망

PH-ILD 시장의 전망은 높은 미충족 의료 수요, 질환에 대한 인식 제고, 그리고 이 질환에 수반되는 심폐 기능 장애의 진행성 특성 등에 힘입어 변화하고 있습니다. 현재로서는 근치적인 치료법은 존재하지 않지만, 지지요법, 산소 요법 및 표적형 폐혈관 확장제의 사용 확대와 더불어, 질환의 병태생리에 대한 이해가 깊어짐에 따라 시장 성장이 뒷받침되고, 장기적인 환자 관리의 성과가 향상될 것으로 기대됩니다.

현재의 치료법을 형성하고 있는 주요 시판 치료제

  • 트레프로스티닐(YUTREPIA) - Liquidia Corporation : YUTREPIA는 폐동맥 고혈압 치료를 위해 개발된 트레프로스티닐 나트륨 흡입용 건조 분말 제제입니다. 본 제제는 일회용 휴대용 흡입기를 사용하여 투여되며, 각 용량의 캡슐에는 트레프로스티닐 외에도L-류신, 폴리솔베이트 80, 염화나트륨, 구연산나트륨, 트레할로스 등의 기능성 첨가제가 배합되어 있어, 폐로의 효율적인 전달과 제제의 안정성이 확보되어 있습니다.
  • 트레프로스티닐(TYVASO/TYVASO DPI/TREPROST) - United Therapeutics사 : 트레프로스티닐(TYVASO) 흡입액 및 트레프로스티닐(TYVASO DPI) 흡입 분말은 폐동맥 고혈압을 동반하는 질환인 폐동맥 고혈압(PAH)(PAH; 그룹 1)의 치료를 위해 성인에게 사용되는 처방약입니다. 또한, 염증으로 인해 폐 내 압력이 상승하는 PH-ILD(PH-ILD; 그룹 3)의 치료에도 사용됩니다.

기타

전반적으로, PH-ILD의 경우 2022년부터 2036년까지 주요 7개국 시장에서 성장세가 비침습적 검사 도구 및 우심실 카테터 검사 등 확정 진단법을 포함한 진단 기법의 발전과 더불어, 질환에 대한 인식 제고 및 치료 옵션의 진화에 힘입어 견인될 것으로 예상되며, 기존 치료법과 개발 중인 후보 약물 모두에 큰 상업적 잠재력을 창출할 것으로 전망됩니다.

  • 주요 7개국 중 미국은 PH-ILD 시장에서 가장 큰 시장 규모를 차지하고 있으며, 2025년에는 약 25억 달러에 달할 것으로 전망됩니다.
  • EU4와 영국을 합친 시장은 전문적인 흡입 요법의 도입 확대에 힘입어 2025년에는 총 시장 규모가 약 5억 달러에 달하며 중요한 시장 부문을 차지했습니다.
  • 2025년 일본의 시장 규모는 약 3억 달러로, 주요 7개국 시장 전체에서 안정적이면서도 지속적인 성장세를 보이고 있습니다.
  • 치료 동향에서 최근 가장 중요한 변화는 기존의 혈관확장제나 적응증 외로 사용되는 PAH 치료제의 한계를 극복하는 데 초점이 맞춰져 있다는 점입니다. 현재 치료의 주류를 이루고 있는 것은, 2025년에 주요 7개국에서 약 11억 달러 이상의 매출을 기록한 트레프로스티닐(TYVASO/TYVASO DPI/TREPROST)과 같은 승인된 흡입 요법이나, 새롭게 등장한 트레프로스티닐(YUTREPIA)입니다.
  • 트레프로스틴일 리포솜(L606)이나 트레프로스틴일 팔미틸(TPIP)과 같은 차세대 치료법으로의 전환은 정밀한 약물전달 분야에서 큰 도약을 의미합니다. 이러한 첨단 치료법은 폐 내 약물 농도를 최적화하고 보다 종합적인 질환 관리를 실현하는 것을 목적으로 하며, 중증 PH-ILD 표현형을 가진 환자의 장기적인 삶의 질(QOL) 및 기능적 예후를 크게 개선합니다.
  • 흡입 요법 : TYVASO, TREPROST, TYVASO DPI 및 YUTREPIA와 같은 제품은 환자에게 표적화된 흡입 투여 옵션을 제공하여, 폐혈관 저항(PVR)을 감소시키고 운동 능력을 향상시키는 데 기여하고 있습니다. United Therapeutics사와 Liquidia Corporation사가 개발한 이러한 치료법들은 이 중대한 미충족 의료 수요에 대응하는 데 있어 핵심적인 역할을 해왔습니다. 흡입액(TYVASO) 및 건조 분말 흡입기(TYVASO DPI)는 PH-ILD에 대해 특별히 승인된 최초의 트레프로스티닐 흡입 요법으로, IIP, IPF, CPFE 및 WHO 그룹 3에 해당하는 결합 조직 질환을 앓고 있는 환자분들의 중요한 미충족 의료 수요를 충족시킵니다.
  • 저분자 화합물 : 'YUTREPIA'는 Liquidia Corporation이 개발한 트레프로스티닐의 DPI 제제로, 미국에서 PH-ILD 적응증으로 승인되었습니다. 특발성 간질성 폐렴(IIP), 특발성 폐섬유증(IPF), 폐섬유증·폐기종 합병증(CPFE), 및 WHO 그룹 3 결합 조직 질환 환자를 대상으로 하는 YUTREPIA는 TYVASO DPI를 대체할 수 있는 선택지가 되어, 투여 효율과 환자 경험의 향상이 기대됩니다.

자주 묻는 질문

  • 2025년 주요 7개국의 PH-ILD 시장 규모는 어떻게 되나요?
  • PH-ILD 시장의 연평균 성장률(CAGR)은 어떻게 되나요?
  • 2025년 미국 내 PH-ILD 확진 환자 수는 얼마인가요?
  • PH-ILD의 주요 치료법은 무엇인가요?
  • PH-ILD의 진단 방법은 어떻게 되나요?
  • PH-ILD 시장의 주요 성장 요인은 무엇인가요?
  • PH-ILD의 치료 현황은 어떤가요?

목차

제1장 주요 인사이트

제2장 소개

제3장 간질성 폐질환 관련 폐고혈압(PH-ILD) : 시장 개요

제4장 주요 요약

제5장 주요 사건

제6장 질환 배경과 개요 : PH-ILD

제7장 역학 및 시장 조사 방법

제8장 역학 및 환자 인구

제9장 환자 경과

제10장 시판 치료제

제11장 새로운 의약품 개요

제12장 PH-ILD : 주요 7개국 시장 분석

제13장 KOL의 견해

제14장 미충족 수요

제15장 SWOT 분석

제16장 시장 진입 및 상환

제17장 부록

제18장 DelveInsight의 서비스 내용

제19장 면책사항

제20장 DelveInsight 소개

KSM 26.07.20

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Insights and Trends

  • According to DelveInsight's analysis, the PH-ILD market across the 7MM was valued at approximately USD 3 billion in 2025 and is projected to expand at a Compounded Annual Growth Rate (CAGR) of 3.9% through 2036, driven by the anticipated entry of advanced therapies and improved clinical management.
  • In 2025, the United States accounted for approximately 83,000 diagnosed prevalent cases of PH-ILD, representing the largest patient base in the 7MM, while EU4 and the UK contributed nearly 70,000 cases, driving significant demand for specialized therapeutic options.
  • The current standard of care includes approved inhaled therapies such as treprostinil (YUTREPIA) and treprostinil (TYVASO/TYVASO DPI/ TREPROST), which focus on symptom management and improving the quality of life (QoL) for patients with severe pulmonary vascular complications.
  • The PH-ILD pipeline is transitioning toward next-generation inhaled and oral modalities, with late-stage assets such as treprostinil liposomal (L606) and treprostinil palmitil inhalation powder (TPIP), alongside emerging candidates like mosliciguat, seralutinib, and H1614 (HB-1614), expected to reshape the treatment landscape and drive market expansion throughout the forecast period.
  • Research in PH-ILD continues to evolve, emphasizing the need for continuous clinical trials to address high morbidity and mortality rates, ensuring better diagnostic precision and updated treatment guidelines for diverse patient populations.
  • Significant treatment gaps in PH-ILD persist due to delayed diagnosis, as overlapping symptoms with Interstitial Lung Disease (ILD) often lead to misdiagnosis. The reliance on invasive procedures like Right Heart Catheterization further limits early detection, underscoring an urgent unmet need for non-invasive biomarkers and advanced imaging to improve patient prognosis and timely intervention.
  • A key market driver PH-ILD is the increasing recognition of the disease and improvement in diagnostic practices, enabling earlier identification and treatment initiation, which is expected to expand the treatable patient pool and drive demand for both existing therapies and emerging pipeline treatments.

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Market size and forecast

  • 2025 PH-ILD Market Size in the 7MM: USD 3 billion
  • PH-ILD Growth Rate (2026-2036) in the 7MM: 3.9% CAGR

DelveInsight's 'Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) - Market Insights, Epidemiology and Market Forecast - 2036' report delivers an in-depth understanding of PH-ILD I, historical and forecasted epidemiology, as well as the PH-ILD market trends in the United States, EU4 (Germany, Spain, Italy, and France), the United Kingdom, and Japan.

The PH-ILD market report delivers a comprehensive analysis of the current treatment landscape, including standards of care, clinical practices, and evolving therapeutic algorithms. It evaluates, PH-ILD 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 PH-ILD and maps the competitive and clinical landscape to uncover high-value opportunities, providing a clear outlook on future market growth potential.

Geography Covered:

North America: The United States

Europe: Germany, France, Italy, Spain and the United Kingdom

Asia-Pacific: Japan

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Understanding and Treatment Algorithm

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Overview and Diagnosis

PH-ILD refers to a wide range of lung conditions, many of which involve scarring or inflammation in the thin layer of tissue between the air sacs and nearby blood vessels. This delicate region, known as the interstitium, is just micrometers thick but plays a vital role by maintaining the structure of the alveoli and enabling smooth oxygen transfer into the bloodstream. Several factors can increase the risk of developing PH-ILD. Chronic smoking and long-term exposure to environmental or occupational toxins can damage the lung tissue, contributing to inflammation and fibrosis that elevate pulmonary artery pressure.

Diagnosing patients with PH-ILD requires a combination of noninvasive and invasive methods. If a patient with ILD shows unexplained worsening of breathlessness or has symptoms that seem disproportionate to the extent of their lung disease, further evaluation for PH is recommended. Since ILD and PH share many overlapping signs, distinguishing between the two can be challenging. To aid diagnosis, healthcare providers often rely on noninvasive tools like pulmonary function tests, the 6MWD, echocardiography, and imaging such as chest X-rays or CT scans. However, for a definitive diagnosis of PH-ILD, RHC remains the most accurate and conclusive method.

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Treatment Landscape

The treatment of PH-ILD primarily focuses on managing the underlying lung disease, yet the scarcity of specifically approved therapies presents a significant challenge in standardizing care. Currently, the landscape is anchored by approved inhaled therapies such as treprostinil (YUTREPIA) and treprostinil (TYVASO/TYVASO DPI/ TREPROST), which focus on symptom management and improving quality of life. Despite these options, many clinicians still report prescribing Pulmonary Arterial Hypertension-approved medications off-label for nearly half of their PH-ILD patients due to persistent therapeutic gaps. However, the paradigm is shifting as late-stage pipeline assets like treprostinil liposomal (L606) and treprostinil palmitil inhalation powder (TPIP).

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Unmet Needs

The section "unmet needs of PH-ILD" 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. Diagnostic challenges and risk of misdiagnosis

2. Need of noninvasive biomarker development

3. The critical need for diversified therapies in PH-ILD

4. Expanding treprostinil routes to ease the daily burden

5. Lack of standard diagnostic pathways leaves PH-ILD patients at risk

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Epidemiology

Key Findings from Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Epidemiological Analysis and Forecast

  • As of 2025, the total diagnosed prevalent population of PH-ILD across the 7MM is estimated at nearly 182,000, reflecting the significant burden of the disease.
  • Beyond PH-ILD, the broader diagnosed prevalent population of ILD in the 7MM was significantly higher, reaching approximately more than 1 million cases in 2025, underscoring the large pool of patients at risk of developing associated pulmonary hypertension.
  • In the year 2025, the United States represented the largest share of the PH-ILD market, with nearly 83,000 diagnosed prevalent cases, supported by advanced diagnostic infrastructure and higher clinical awareness.
  • In 2025, EU4 and the UK together contributed approximately 70,000 cases of PH-ILD with the United Kingdom representing the largest individual European segment with nearly 18,000 of PH-ILD diagnosed cases.
  • Japan accounted for approximately 29,000 diagnosed prevalent cases of PH-ILD in 2025, representing a stable and clinically significant patient base in the Asian market.
  • Gender-specific analysis in the United States for 2025 shows a slightly higher prevalence in males, with approximately 43,000 cases, compared to nearly 40,000 cases in females.

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Drug Analysis & Competitive Landscape

The PH-ILD drug chapter provides a detailed, market-focused review of approved therapies and the emerging pipeline across mid and late Phase 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 PH-ILD treatment landscape, supporting market assessment, competitive analysis, and growth forecasting for the PH-ILD therapeutics market.

Approved Therapies for Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD)

Treprostinil (YUTREPIA): Liquidia Corporation

YUTREPIA is a dry powder inhalation formulation of treprostinil sodium, a prostacyclin mimetic designed for the treatment of PAH. Delivered through a single-use, handheld inhaler, each capsule contains treprostinil sodium blended with carefully selected excipients L-leucine, polysorbate 80, sodium chloride, sodium citrate, and trehalose.

  • In May 2025, YUTREPIA (treprostinil) inhalation powder received full FDA approval for improving exercise capacity in adults with PAH and PH-ILD. As the first dry powder prostacyclin therapy enabled by Liquidia's PRINT technology, YUTREPIA delivers uniform particles to the deep lung via a low-resistance, breath-actuated device, offering enhanced usability and potential adherence benefits.
  • In August 2024, Liquidia received tentative FDA approval to market YUTREPIA, its inhaled dry powder formulation of treprostinil, for the treatment of PAH and PH-ILD. This approval allows commercialization once the existing regulatory exclusivity expires in May 2025. The New Drug Application (NDA) for YUTREPIA was submitted via the 505(b) (2) pathway, using nebulized TYVASO as the reference listed drug.

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Pipeline Analysis

Treprostinil liposomal (L606): Pharmosa BioPharm/Liquidia Corporation

L606 is a liposomal formulation of treprostinil, with liposome particle sizes ranging from 110-140 nm, suspended in a sodium citrate-bicarbonate buffer. This advanced formulation is engineered to enhance drug stability and prolong systemic circulation by protecting treprostinil from rapid degradation and clearance. The liposomal encapsulation enables a sustained pulmonary release, allowing for more consistent drug exposure over time, while minimizing peaks and troughs that may lead to side effects.

  • In January 2025, at the 43rd J.P. Morgan Healthcare Conference, Liquidia Corporation presented advancements in treprostinil (L606), highlighting its twice-daily dosing, improved tolerability, and rapid delivery via a next-generation breath-actuated handheld nebulizer with smart technology. The company also outlined plans to expand its clinical potential by exploring additional indications such as Idiopathic Pulmonary Fibrosis (IPF), Pulmonary Hypertension associated with Chronic Obstructive Pulmonary Disease (PH-COPD), and Systemic Sclerosis-related Raynaud's phenomenon (SSc-RP), while also targeting inadequate responders in Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension associated with Interstitial Lung Disease (PH-ILD).

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD), Market Leaders and Emerging Companies

  • Liquidia Corporation
  • United Therapeutics
  • Pharmosa BioPharm
  • Halo Biosciences
  • Roivant Sciences/Pulmovant
  • Insmed and others

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Drug Updates

  • In May 2025, Gossamer Bio reported the completion of enrollment for the ongoing global Phase III PROSERA study, which is evaluating seralutinib in patients with Functional Class II and III PAH.
  • In June 2025, Halo Biosciences reported the publication of results from its Phase IIa SATURN study in Thorax, marking a pivotal milestone in the development of HB-1614 for PH-ILD.
  • In June 2025, Insmed reported positive topline results from its Phase IIb study of TPIP as an once-daily treatment for PAH.
  • In September 2024, TREPROST (treprostinil) inhalation solution received approval in Japan for a new indication targeting adults at risk of disease progression, marking a significant regulatory milestone in expanding access to treprostinil therapy for patients in the Japanese markets.

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Market Outlook

The market outlook for PH-ILD is evolving, driven by high unmet clinical need, increasing disease recognition, and the progressive nature of cardiopulmonary impairment associated with the condition. While no curative therapies currently exist, growing adoption of supportive care, oxygen therapy, and targeted pulmonary vasodilators, along with advancements in understanding disease pathophysiology, are expected to support market growth and improve long-term patient management outcomes.

Key marketed therapies shaping current management

  • Treprostinil (YUTREPIA) - Liquidia Corporation: YUTREPIA is an inhaled dry powder formulation of treprostinil sodium developed for the management of Pulmonary Arterial Hypertension. It is administered using a disposable handheld inhaler, where each dose capsule combines treprostinil with functional excipients including L-leucine, polysorbate 80, sodium chloride, sodium citrate, and trehalose to ensure efficient pulmonary delivery and formulation stability.
  • Treprostinil (TYVASO/TYVASO DPI/ TREPROST) - United Therapeutics: Treprostinil (TYVASO) inhalation solution and treprostinil (TYVASO DPI) inhalation powder are prescription medicines used in adults to treat pulmonary arterial hypertension (PAH) (PAH; Group 1), a condition involving high blood pressure in the arteries of the lungs. They are also used to treat PH-ILD disease (PH-ILD; Group 3), which involves elevated pressure in the lungs due to inflammation.

And more

Overall, in PH-ILD, market growth across the 7MM from 2022-2036 is expected to be driven by advancements in diagnostic approaches, including noninvasive tools and confirmatory methods like Right Heart Catheterization, along with increasing disease awareness and evolving treatment options, creating strong commercial potential for both existing therapies and emerging pipeline candidates.

  • Among the 7MM, the United States accounted for the largest market size of PH-ILD, valued at approximately USD 2,500 million in 2025.
  • EU4 and the UK combined represented a significant market segment, with a total market size of approximately USD 500 million in 2025, driven by the increasing adoption of specialized inhaled therapies.
  • Japan accounted for a market size of approximately USD 300 million in 2025, representing a stable and growing portion of the total 7MM market.
  • The most meaningful recent shift in the treatment landscape has been the focus on addressing the limitations of traditional vasodilators and off-label PAH medications. Currently, the landscape is anchored by approved inhaled therapies like treprostinil (TYVASO/TYVASO DPI/ TREPROST) which generated over ~USD 1,100 million in the 7MM in 2025 and the emergence of treprostinil (YUTREPIA).
  • The transition toward next-generation therapies, such as treprostinil liposomal (L606) and treprostinil palmitil (TPIP), represents a significant leap in precision delivery. These advanced modalities aim to optimize drug concentration in the lungs and provide more comprehensive disease control, significantly improving the long-term quality of life and functional outcomes for patients with severe PH-ILD phenotypes.

Drug Class/Insights into Leading Emerging and Marketed Therapies in Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) (2022-2036 Forecast)

The current treatment landscape for PH-ILD is structured across several therapeutic categories, reflecting disease severity, underlying ILD subtype, and patient-specific response. Supplemental oxygen therapy and pulmonary rehabilitation remain foundational options for managing chronic hypoxemia and improving exercise capacity. For patients with more advanced disease, the treatment paradigm is evolving to include targeted pulmonary vasodilators such as inhaled prostacyclins or PDE5 inhibitors which aim to reduce pulmonary vascular resistance (PVR) and alleviate right heart strain.

  • Inhaled Therapy: Products such as TYVASO/ TREPROST/TYVASO DPI and YUTREPIA provide patients with targeted inhaled delivery options that help reduce PVR and improve exercise capacity. These therapies, developed by United Therapeutics and Liquidia Corporation, have played a central role in addressing this significant unmet need. inhalation solution (TYVASO ) and dry powder inhaler (TYVASO DPI) are the first inhaled treprostinil therapies approved specifically for PH-ILD, addressing an important unmet need for patients with IIP, IPF, CPFE, and WHO Group 3 connective tissue diseases.
  • Small Molecule: YUTREPIA is Liquidia Corporation's DPI formulation of treprostinil, approved for PH-ILD in the US. Targeting patients with idiopathic interstitial pneumonia (IIP), idiopathic pulmonary fibrosis (IPF), combined pulmonary fibrosis and emphysema (CPFE), and WHO Group 3 connective tissue diseases, YUTREPIA offers an alternative to TYVASO DPI with potentially improved delivery and patient experience.

Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) 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 PH-ILD 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 emergence of next-generation inhaled therapies is reshaping the treatment landscape of PH-ILD, with a focus on improving drug delivery, efficacy, and patient convenience, treprostinil (YUTREPIA), developed by Liquidia Corporation, is an advanced dry powder inhalation formulation of treprostinil sodium, a prostacyclin mimetic, designed for efficient pulmonary delivery via a handheld device. Engineered with optimized excipients, it enables precise drug deposition in the lungs, enhancing therapeutic outcomes, and is expected to witness a medium-to-fast uptake, reflecting its potential to significantly improve patient-friendly disease management.

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

Market Access and Reimbursement of Approved therapies in Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD)

The report further provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenario of approved 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 Hypertension Associated with Interstitial Lung Disease (PH-ILD) Price Scenario & Trends

Pricing and analogue assessment of PH-ILD 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 Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Approved Drugs

Treprostinil (YUTREPIA): The annual treatment cost of YUTREPIA in the United States is approximately USD 300,000, reflecting its role as an advanced inhaled therapy for managing PH-ILD associated with ILD.

Industry Experts and Physician Views for Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD)

To keep up with PH-ILD 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 PH-ILD 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 including MD, PhD, Instructor, Postdoctoral Researcher, Professor, Researcher, and others.

DelveInsight's analysts engaged with 10+ key opinion leaders (KOLs) across major markets to capture country-level insights in PH-ILD Leading centers such as University of California and Royal College of Physicians, among others, were consulted to validate clinical practices, treatment patterns, and emerging therapeutic perspectives.

Their opinion helps understand and validate current and emerging PH-ILD, therapies, highlight unmet medical needs, provide epidemiological context, and support strategic decisions for Market access, therapy adoption, and pipeline prioritization in PH-ILD.

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 PH-ILD, 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. 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, a descriptive overview of PH-ILD, 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 PH-ILD 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 PH-ILD market.

Report Insights

  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Patient Population Forecast
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Therapeutics Market Size
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Pipeline Analysis
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Market Size and Trends
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) 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
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Market Outlook (North America, Europe, Asia-Pacific)
  • Patient Burden Trends (by geography)
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Treatment Addressable Market (TAM)
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Competitive Landscape
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Major Companies Insights
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Price trends and Analogue Assessment
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Therapies Drug Adoption/Uptake
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Therapies Peak Patient Share analysis

Report Assessment

  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Current Treatment Practices
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Unmet Needs
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Clinical Development Analysis
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Emerging Drugs Product Profiles
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Market Attractiveness
  • Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) Qualitative Analysis (SWOT and Attribute analysis)

FAQs:

Market Insights

  • What was the PH-ILD 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 PH-ILD?
  • What are the disease risks, burdens, and unmet needs of PH-ILD? What will be the growth opportunities across the 7MM concerning the patient population with PH-ILD ?
  • 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 PH-ILD?
  • What are the current guidelines for treating PH-ILD 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 PH-ILD 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/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 within 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 Hypertension Associated with Interstitial Lung Disease (PH-ILD) Market Overview at a Glance

  • 3.1. Market Share (%) Distribution of PH-ILD by Therapies in the 7MM in 2025
  • 3.2. Market Share (%) Distribution of PH-ILD by Therapies in the 7MM in 2036

4. Executive Summary

5. Key Events

6. Disease Background and Overview: PH-ILD

  • 6.1. Introduction
  • 6.2. Classification
  • 6.3. Signs and Symptoms
  • 6.4. Causes and Risk Factors
  • 6.5. Pathogenesis
  • 6.6. Biomarkers
    • 6.6.1. Diagnosis
    • 6.6.2. Diagnosis Challenges
    • 6.6.3. Diagnostic Algorithm
    • 6.6.4. Diagnostic Guidelines
  • 6.7. Treatment
    • 6.7.1. Treatment Algorithm
    • 6.7.2. Treatment Guidelines

7. Epidemiology and Market Methodology

8. Epidemiology and Patient Population

  • 8.1. Key Findings on Patient Burden in PH-ILD
  • 8.2. Assumptions and Rationale: 7MM
    • 8.2.1. Diagnosed Prevalent Cases of ILD
    • 8.2.2. Type-specific Diagnosed Prevalent Cases of ILD
    • 8.2.3. Type-specific Diagnosed Prevalent Cases of PH-ILD
    • 8.2.4. Gender-specific Diagnosed Prevalent Cases of PH-ILD
  • 8.3. Total Diagnosed Prevalent Cases of PH-ILD in the 7MM
  • 8.4. The United States
    • 8.4.1. Diagnosed Prevalent Cases of ILD in the US
    • 8.4.2. Type-specific Diagnosed Prevalent Cases of ILD in the US
    • 8.4.3. Type-specific Diagnosed Prevalent Cases of PH-ILD in the US
    • 8.4.4. Total Diagnosed Prevalent Cases of PH-ILD in the US
    • 8.4.5. Gender-specific Diagnosed Prevalent Cases of PH-ILD in the US
  • 8.5. EU4 and the UK
    • 8.5.1. Diagnosed Prevalent Cases of ILD in EU4 and the UK
    • 8.5.2. Type-specific Diagnosed Prevalent Cases of ILD in EU4 and the UK
    • 8.5.3. Type-specific Diagnosed Prevalent Cases of PH-ILD in EU4 and the UK
    • 8.5.4. Total Diagnosed Prevalent Cases of PH-ILD in EU4 and the UK
    • 8.5.5. Gender-specific Diagnosed Prevalent Cases of PH-ILD in EU4 and the UK
  • 8.6. Japan
    • 8.6.1. Diagnosed Prevalent Cases of ILD in Japan
    • 8.6.2. Type-specific Diagnosed Prevalent Cases of ILD in Japan
    • 8.6.3. Type-specific Diagnosed Prevalent Cases of PH-ILD in Japan
    • 8.6.4. Total Diagnosed Prevalent Cases of PH-ILD in Japan
    • 8.6.5. Gender-specific Diagnosed Prevalent Cases of PH-ILD in Japan

9. Patient Journey

10. Marketed Therapies

  • 10.1. Competitive Landscape: Marketed Drugs
  • 10.2. Treprostinil (YUTREPIA): Liquidia Corporation
    • 10.2.1. Product Description
    • 10.2.2. Regulatory Milestones
    • 10.2.3. Other Developmental Activities
    • 10.2.4. Clinical Trials Information
    • 10.2.5. Safety and Efficacy
  • 10.3. Treprostinil (TYVASO/TYVASO DPI/TREPROST): United Therapeutics
    • 10.3.1. Product Description
    • 10.3.2. Regulatory Milestones
    • 10.3.3. Other Developmental Activities
    • 10.3.4. Clinical Trials Information
    • 10.3.5. Safety and Efficacy

11. Emerging Drugs Profiles

  • 11.1. Competitive Landscape: Emerging Drugs
  • 11.2. L606 (Treprostinil liposomal): Pharmosa BioPharm/Liquidia Corporation
    • 11.2.1. Drug Description
    • 11.2.2. Other Developmental Activities
    • 11.2.3. Clinical Trials Information
    • 11.2.4. Safety and Efficacy
    • 11.2.5. Analysts' View
  • 11.3. TPIP (Treprostinil Palmitil Inhalation Powder): Insmed
    • 11.3.1. Drug Description
    • 11.3.2. Other Developmental Activities
    • 11.3.3. Clinical Trial Information
    • 11.3.4. Safety and Efficacy
    • 11.3.5. Analysts' View
  • 11.4. Mosliciguat: Roivant Sciences/Pulmovant
    • 11.4.1. Drug Description
    • 11.4.2. Other Developmental Activities
    • 11.4.3. Clinical Trials Information
    • 11.4.4. Safety and Efficacy
    • 11.4.5. Analysts' View
  • 11.5. Seralutinib: XOMA Royalty/Gossamer Bio
    • 11.5.1. Drug Description
    • 11.5.2. Other Developmental Activities
    • 11.5.3. Clinical Trials Information
    • 11.5.4. Safety and Efficacy
    • 11.5.5. Analysts' View
  • 11.6. Vardenafil (RT234): Respira Therapeutics
    • 11.6.1. Drug Description
    • 11.6.2. Clinical Trials Information
    • 11.6.3. Analysts' View
  • 11.7. H1614 (HB-1614): Halo Biosciences
    • 11.7.1. Drug Description
    • 11.7.2. Other Developmental Activities
    • 11.7.3. Clinical Trials Information
    • 11.7.4. Safety and Efficacy
    • 11.7.5. Analysts' View

12. PH-ILD: 7MM Market Analysis

  • 12.1. PH-ILD Market Key Findings and Insights
  • 12.2. Key Market Forecast Assumptions
    • 12.2.1. Cost Assumptions and Rebates
    • 12.2.2. Pricing Trends
    • 12.2.3. Analogue Assessment
    • 12.2.4. Launch Year and Therapy Uptake
  • 12.3. Market Outlook
  • 12.4. Attribute Analysis
  • 12.5. Total Market Size of PH-ILD in the 7MM
  • 12.6. Market Size of PH-ILD by Therapies in the 7MM
  • 12.7. Market Size of PH-ILD in the United States
    • 12.7.1. Total Market of PH-ILD
    • 12.7.2. Market Size of PH-ILD by Therapies in the United States
  • 12.8. Market Size of PH-ILD in EU4 and the UK
    • 12.8.1. Total Market Size of PH-ILD
    • 12.8.2. Market Size of PH-ILD by Therapies in EU4 and the UK
  • 12.9. Market Size of PH-ILD in Japan
    • 12.9.1. Total Market Size of PH-ILD
    • 12.9.2. Market Size of PH-ILD by Therapies in Japan

13. Key Opinion Leaders' Views

14. Unmet Needs

15. SWOT Analysis

16. Market Access and Reimbursement

  • 16.1. The United States
    • 16.1.1. CMS
  • 16.2. EU4 and the UK
    • 16.2.1. Germany
    • 16.2.2. France
    • 16.2.3. Italy
    • 16.2.4. Spain
    • 16.2.5. The United Kingdom
  • 16.3. Japan
    • 16.3.1. MHLW

17. Appendix

  • 17.1. Acronyms and Abbreviations
  • 17.2. Bibliography
  • 17.3. Report Methodology

18. DelveInsight Capabilities

19. Disclaimer

20. About DelveInsight

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