Idiopathic Interstitial Pneumonia Market

Key Highlights

• The Idiopathic Interstitial Pneumonia (IIP) market is anticipated to sustain a steady Compound Annual Growth Rate (CAGR) during the forecast period (2025–2034). This growth is driven by increasing awareness and diagnosis of IIP, rising prevalence of interstitial lung diseases, advancements in targeted therapies, expansion of treatment options, and ongoing research and development in antifibrotic and immunomodulatory drugs.
• The increasing cases of IIP may be attributed to aging populations, improved diagnostic capabilities, greater awareness among healthcare professionals, environmental and occupational exposures, and the rising prevalence of risk factors such as smoking and chronic lung conditions.
• Current treatment for IPF primarily involves antifibrotic therapies such as OFEV (nintedanib) and pirfenidone (including generics), which slow disease progression and reduce decline in lung function. Supportive care, including oxygen therapy, pulmonary rehabilitation, and symptom management, is also commonly used, while lung transplantation is an option for eligible patients with advanced disease.
• There remains an unmet need in IPF treatment, as current therapies have limited efficacy in reversing fibrosis, fully controlling symptoms, or improving long-term outcomes, highlighting the need for more effective and safer emerging therapies.
• Emerging therapies aim to address these gaps by targeting novel pathways involved in fibrosis and inflammation; notable examples include nerandomilast (BI 1015550), admilparant (BMS-986278), and NIKTIMVO (axatilimab-csfr), among others, offering potential for improved outcomes and better disease management.
• Reflecting progress in the field, in May 2025, Boehringer Ingelheim shared detailed Phase III FIBRONEER-IPF results, published in the New England Journal of Medicine and presented as a late-breaking abstract at the American Thoracic Society (ATS) 2025 International Conference.

DelveInsight’s comprehensive report titled “Idiopathic Interstitial Pneumonia (IIP) — Market Insights, Epidemiology, and Market Forecast – 2034” offers a detailed analysis of IIP. The report presents historical and projected epidemiological data covering total prevalent cases of idiopathic interstitial pneumonia, total diagnosed prevalent cases of idiopathic interstitial pneumonia, type-specific diagnosed prevalent cases of idiopathic interstitial pneumonia, and treated cases of idiopathic interstitial pneumonia. In addition to epidemiology, the market report encompasses various aspects related to the patient population. These aspects include the diagnosis process, prescription patterns, physician perspectives, market accessibility, treatment options, and prospective developments in the market across seven major markets: the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan, spanning from 2020 to 2034.

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

Idiopathic Interstitial Pneumonia Overview

IIPs are a subgroup of diffuse parenchymal lung diseases (also termed interstitial lung diseases) of unknown cause, marked by varying degrees of inflammation and fibrosis that primarily affect the lung interstitium, but may also involve airspaces, small airways, and vessels. They represent distinct clinicopathological entities that can be differentiated from other diffuse lung diseases through a multidisciplinary evaluation combining history, physical examination, radiology (particularly high-resolution computed tomography [HRCT]), pathology, and laboratory studies.

The American Thoracic Society/European Respiratory Society (ATS/ERS) classification defines the major IIP subtypes:

• Idiopathic pulmonary fibrosis (IPF) – the most common and most lethal form
• Nonspecific interstitial pneumonia (NSIP) – a provisional diagnosis requiring further clarification
• Cryptogenic organizing pneumonia (COP)
• Acute interstitial pneumonia (AIP)
• Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)
• Desquamative interstitial pneumonia (DIP)
• Lymphoid interstitial pneumonia (LIP)

Idiopathic Interstitial Pneumonia Diagnosis and Treatment Algorithm

The diagnosis of IIPs is a dynamic process that may need revision as new data emerge. Evaluation begins with history, examination, and HRCT, which is central for distinguishing IPF/Usual Interstitial Pneumonia (UIP) from other IIPs. When findings are inconclusive, surgical lung biopsy, often via video-assisted thoracoscopy, is advised, with samples from multiple lobes. Histopathological patterns provide the basis for classification and typically separate subtypes more clearly than imaging. Bronchoalveolar lavage and transbronchial biopsy have limited roles, mainly in excluding sarcoidosis or infection. The final diagnosis should result from a multidisciplinary review of all clinical, radiologic, and pathologic findings, ensuring accurate classification and guiding research and management.

Management varies by subtype. IPF, the most common and fatal form, is treated with antifibrotic agents like nintedanib and pirfenidone, which slow progression. Inflammatory-predominant forms such as NSIP, COP, and DIP may respond to corticosteroids or immunosuppressants. Supportive care with oxygen therapy, pulmonary rehabilitation, and vaccination is important for all patients. In advanced disease, particularly IPF, lung transplantation remains the only curative option. Emerging trials are investigating novel therapies to address the heterogeneity of IIPs and improve outcomes.

Idiopathic Interstitial Pneumonia Epidemiology

The epidemiology section of the IIP market report offers information on the patient populations, including historical and projected trends for each of the seven major markets. Examining key opinion leader views from physicians or clinical experts can assist in identifying the reasons behind historical and projected trends. The diagnosed patient pool, their trends, and the underlying assumptions are all included in this section of the report.

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

Key Findings

IIP comprises a heterogeneous group of lung disorders, with IPF being the most common and deadliest subtype, representing around 55% of all IIP cases.

NSIP is considered a relatively rare subtype of IIP, accounting for 14–36% of reported cases. Although uncommon, it is still encountered more often than other subtypes such as DIP and RB-ILD, which together represent about 10–17% of cases, and AIP, which is the rarest form, seen in only 0–2% of cases. This places NSIP in an intermediate position within the spectrum of IIPs—less frequent than the IPF but notably more prevalent than the rarest entities.

• In 2024, the 7MM reported an estimated 190,000 diagnosed prevalent cases of IPF, with the US contributing the highest proportion at around 100,000 cases. The burden is anticipated to rise over the forecast period (2025–2034).
• In 2024, the EU4 and the UK together reported about 70,000 diagnosed prevalent cases of IPF, with the UK having the highest count at approximately 21,000 cases, while Germany recorded the lowest at around 11,000 cases.
• In 2024, Japan reported approximately 21,000 diagnosed prevalent cases of IPF, representing about 11% of the total burden across the 7MM.
• The epidemiology of IIP is expected to change during the forecast period (2025–2034).

Idiopathic Interstitial Pneumonia Market Outlook

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

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

Idiopathic Interstitial Pneumonia Drug Chapters

Marketed Idiopathic Interstitial Pneumonia Drugs

OFEV (nintedanib): Boehringer Ingelheim

OFEV (nintedanib), developed by Boehringer Ingelheim, is an oral small molecule therapy approved for the treatment of IPF. It functions as a multiple receptor tyrosine kinase (RTK) and non-receptor tyrosine kinase (nRTK) inhibitor, targeting pathways involved in fibrosis and disease progression. OFEV was first approved in the US in 2014, followed by approvals in the EU and Japan in 2015, and is indicated for use in adults and pediatric patients aged 12 years and older. Its availability has provided a key therapeutic option in slowing the decline of lung function in patients with IPF.

Nintedanib Accord, a generic version of OFEV, received EU approval in April 2024, marking the entry of lower-cost alternatives into the IPF treatment landscape and potentially impacting market dynamics and accessibility.

Note: Detailed marketed therapies assessment will be provided in the final report

Emerging Idiopathic Interstitial Pneumonia Drugs

The IIP market is expected to evolve gradually, driven by the limited number of emerging therapies currently in development. Key players such as nerandomilast (BI 1015550), admilparant (BMS-986278), NIKTIMVO (axatilimab-csfr), among others are showing active commitment to addressing this unmet need, with ongoing efforts to advance novel treatment options for this complex condition.

Nerandomilast (BI 1015550): Boehringer Ingelheim

Nerandomilast is a first-in-class oral PDE4B inhibitor being developed by Boehringer Ingelheim for IPF and PPF. As part of the company’s next wave of antifibrotic therapies, it offers a novel approach aimed at preserving lung function and improving patient outcomes.

Nerandomilast has been granted both Breakthrough Therapy Designation (BTD) and Orphan Drug Designation (ODD) for the treatment of IPF.

Its NDA for IPF is currently under priority review, with an expected decision in Q4 2025.

Admilparant (BMS-986278): Bristol-Myers Squibb

Admilparant is a potential first-in-class, oral, small molecule lysophosphatidic acid receptor 1 (LPA1) antagonist currently being evaluated as a novel antifibrotic treatment for patients with IPF and PPF. Increased LPA levels and activation of LPA1 are involved in the pathogenesis of pulmonary fibrosis. A preclinical in vitro and in vivo study found that antagonizing LPA1 may be beneficial in treating lung injury and fibrosis.

BMS-986278 has received both Fast Track designation (FTD) and ODD from the US FDA for the treatment of IPF.

Admilparant is currently in a Phase III clinical trial, with data expected in 2026.

NIKTIMVO (axatilimab-csfr): Incyte/Syndax

Axatilimab, a first-in-class antibody targeting the colony stimulating factor-1 receptor (CSF-1R), is being evaluated in collaboration with Syndax for the treatment of IPF.

Currently, axatilimab is being evaluated in a Phase II trial in patients with IPF.

Note: Detailed emerging therapies assessment will be provided in the final report.

Idiopathic Interstitial Pneumonia Market Segmentation

DelveInsight’s ‘Idiopathic Interstitial Pneumonia (IIP) – Market Insights, Epidemiology, and Market Forecast – 2034’ report provides a detailed outlook of the current and future IIP market, segmented within countries, by therapies, and by classes. Further, the market of each region is then segmented by each therapy to provide a detailed view of the current and future market share of all therapies.

Idiopathic Interstitial Pneumonia Market Size by Countries

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

Idiopathic Interstitial Pneumonia Market Size by Therapies

Idiopathic Interstitial Pneumonia Market Size by Therapies is categorized into current and emerging markets for the study period 2020–2034.

Note: Detailed market segment assessment will be provided in the final report.

Idiopathic Interstitial Pneumonia Drugs Uptake

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

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

Note: Detailed assessment of drug uptake and attribute analysis will be provided in the full report on IIP.

Idiopathic Interstitial Pneumonia Market Access and Reimbursement

DelveInsight’s ‘Idiopathic Interstitial Pneumonia (IIP) – Market Insights, Epidemiology, and Market Forecast – 2034’ report provides a descriptive overview of the market access and reimbursement scenario of IIP.

This section includes a detailed analysis of the country-wise healthcare system for each therapy, enlightening the market access, reimbursement policies, and health technology assessments.

KOL Views

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

Idiopathic Interstitial Pneumonia: KOL Insights

DelveInsight’s analysts connected with 25+ KOLs to gather insights; however, interviews were conducted with 10+ KOLs in the 7MM. These KOLs were from organizations, institutes, and hospitals, such as University of California, US, University Hospital Tübingen, Germany, Louis Pradel University Hospital, Lyon, France, ellvitge-Idibell University Hospital, Barcelona, Spain, University of Leeds, Leeds, UK, and Toyama Medical and Pharmaceutical University, Japan, among others.

“Genetic screening that can identify variants of the mucin-causing MUC5B gene — the key risk factor that predisposes people for IPF — may hold the key to the development and administration of targeted therapies that may prevent the disease before it can progress and cause harm.”

“Before the entry of approved drugs, the treatment of IPF was terrible. After the diagnosis of disease, patient’s survival was about a couple of years. Even after years of approval of 2 emerging drugs, the scenario is not very much changed. Due to the lack of awareness of drugs, very less number of patients are been treated with the approved drugs.”

“PIRESPA was the first therapy approved for IPF in Japan, followed by OFEV, which gained approval in 2015. These treatments have significantly reshaped the management landscape for IPF, though continued efforts are needed to expand therapeutic options.”

Note: Detailed assessment of KOL Views will be provided in the full report on Idiopathic Interstitial Pneumonia.

Competitive Intelligence Analysis

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

Idiopathic Interstitial Pneumonia Pipeline Development Activities

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

Pipeline Development Activities

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

Idiopathic Interstitial Pneumonia Report Insights

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

Idiopathic Interstitial Pneumonia Report Key Strengths

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

Idiopathic Interstitial Pneumonia Report Assessment

• Idiopathic Interstitial Pneumonia Current Treatment Practices
• Unmet Needs
• Idiopathic Interstitial Pneumonia Pipeline Product Profiles
• Idiopathic Interstitial Pneumonia Market Attractiveness

Key Questions

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

Reasons to buy

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

Frequently Asked Questions

What are the treatment goals for IIP?

The primary objectives in treating IIP are to slow disease progression, alleviate symptoms, preserve lung function, improve quality of life, and extend survival. Since these disorders are often progressive and associated with significant morbidity and mortality, treatment strategies focus not only on targeting the underlying fibrotic or inflammatory processes but also on providing supportive care, preventing complications, and optimizing overall patient well-being.

What are the challenges in managing IIP?

Managing IIP presents challenges such as heterogeneity of subtypes, overlapping clinical and radiologic features, difficulty in establishing an accurate diagnosis, limited availability of curative treatments, variable response to therapy, risk of disease progression despite treatment, and the need for close multidisciplinary collaboration to guide management and improve patient outcomes.

What are the key factors driving the growth of the IIP market?

The IIP market is driven by a rising disease burden, growing awareness and earlier diagnosis, availability of approved antifibrotic therapies, ongoing research into novel treatment approaches, and increasing healthcare investments in rare and chronic lung diseases. Additionally, the introduction of generics and pipeline innovations are expected to further shape accessibility and market growth.

How will the Idiopathic Interstitial Pneumonia Market and Epidemiology Forecast Report benefit the clients?

The report will provide comprehensive insights into the current IIP market landscape, emerging therapies, competitive dynamics, regulatory requirements, and market access considerations, enabling informed decision-making, strategic planning, and optimization of business strategies to capitalize on market opportunities and drive growth.