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Primary Care Can Address Complex Lung Diseases, Too
Primary Care Can Address Complex Lung Diseases, Too

Medscape

time26-05-2025

  • Health
  • Medscape

Primary Care Can Address Complex Lung Diseases, Too

Primary care physicians (PCPs) often face challenges in diagnosing complex pulmonary issues in patients, particularly when nonspecific symptoms appear similar to cardiovascular issues, asthma, or chronic obstructive pulmonary disease. However, clinicians can cover both pulmonary and cardiovascular concerns during an exam, potentially shortening delays in the diagnosis of interstitial lung diseases (ILDs), including pulmonary fibrosis (PF). Tejaswini Kulkarni, MD 'ILDs are complex, chronic progressive diseases with a great impact on a patient's quality of life and survival. These are often underdiagnosed, or there is significant delay in diagnosis after onset of symptoms due to a multitude of reasons,' said Tejaswini Kulkarni, MD, associate professor of pulmonary, allergy, and critical care medicine and director of the interstitial lung disease program at the University of Alabama at Birmingham, Alabama. 'Early intervention can slow disease progression, improve quality of life, and potentially extend survival in ILD patients,' she said. 'For primary care physicians, increased awareness of the signs and symptoms of ILD and early recognition are crucial.' Timely Diagnosis Tools Although most PCPs try to evaluate the root causes of nonspecific symptoms, about 2 in 5 tend to bypass symptom evaluation if the patient is already on inhaled therapy for a pulmonary condition, according to a survey by the American College of Chest Physicians (CHEST). Instead, they often modulate therapy — for what may be an incorrect diagnosis. William Lago, MD 'As a practicing primary care physician, it doesn't surprise me that PF and ILD are generally misdiagnosed or experience delays in diagnosis. These diseases are on the rare side, so when a patient comes to their PCP, that doctor first will opt to rule out heart issues that can quickly end a life,' said William Lago, MD, a family medicine physician with the Cleveland Clinic-Wooster Family Health Center in Wooster, Ohio. 'That said, lung diseases like PF are incredibly difficult to live with and can progress rapidly if untreated,' he said. 'An earlier diagnosis means starting treatments to slow fibrosing of the lungs, and with slowed disease progression, a patient's quality of life is often improved.' In general, high-resolution computed tomography (HRCT) is considered the gold standard for imaging when it comes to detecting ILD. However, only 62% of PCPs said they order HRCT when a patient's chest radiograph shows lower lobe opacity, and only half said they order it when a patient has inspiratory crackles or other abnormalities during a pulmonary exam, according to the CHEST survey. In response, CHEST and the Three Lakes Foundation sponsored a clinician toolkit, which was created by PCPs and pulmonologists to help clinicians better identify, manage, and treat ILDs. The toolkit includes a patient questionnaire, a decision-making module with patient case studies, an online module with in-depth ILD symptoms and sounds of crackles, and videos of radiologic features of ILDs. The project, called Bridging Specialties: Timely Diagnosis for ILD, also includes white papers and podcast episodes on overcoming barriers to diagnosis. 'In working on this initiative with my pulmonary colleagues, I'm already finding myself thinking more about PF and ILDs as potential diagnoses when seeing patients,' said Lago, who served on the Bridging Specialties expert steering committee. 'Between the patient questionnaire, the decision-making module, and the other resources in the clinician toolkit, I can see this having an incredible impact on how we diagnose patients.' This teamwork approach can help PCPs improve diagnosis rates alongside other specialists, said Kulkarni, who also served on the Bridging Specialties committee. 'Many patients present with vague or nonspecific symptoms, and ILDs can mimic other, more common respiratory disorders or coronary artery diseases, along with shared features of older age and history of smoking,' she said. 'The differential diagnosis is complex and often requires a multidisciplinary team of pulmonologists, rheumatologists, radiologists, and pathologists to identify the subtype of ILD.' Other medical societies have created informational resources as well, including the American Thoracic Society's ILD and idiopathic pulmonary fibrosis (IPF) resources and the Pulmonary Fibrosis Foundation's webinars and clinical resources. Jeffrey Horowitz, MD 'My top advice is to go to reputable sources. I've had one patient ask me about drinking hydrogen peroxide to treat their condition, which they read on a forum online. Others have asked about stem cell therapy in other countries, which isn't regulated and can do real harm,' said Jeffrey Horowitz, MD, professor of medicine and division director of Pulmonary, Critical Care, and Sleep Medicine at Ohio State University, Columbus, Ohio. 'Overall, I tell clinicians that if somebody is short of breath, has crackles, and has a normal echocardiogram, it's probably not the heart, so do those pulmonary function studies early,' he said. 'Since most nonpulmonologists don't have substantial expertise in this area, it's a good idea to have patients evaluated at an academic medical center with expertise in ILD, which also opens the doors for patients to be enrolled in clinical trials.' Ongoing Research and Treatments Ohio State, for instance, recently joined the IPF-PRO/ILD-PRO Registry, an industry-academic collaborative started by Duke University, Durham, North Carolina, in 2014 to maintain a registry of patients for potential therapies and clinical trials. 'There can be a sense of nihilism regarding this entire spectrum of fibrotic lung disease, which wouldn't be without merit if we were talking about 20 years ago,' Horowitz said. 'Today, there are a lot of reasons to be optimistic as we're making gains and improving care for these patients.' Numerous clinical trials are underway, including positive phase 3 results for FIBRONEER-IPF from Boehringer Ingelheim. The trial found that nerandomilast, an oral form of a phosphodiesterase 4B inhibitor, improved forced vital capacity (FVC) at 52 weeks, as compared with placebo. The drug hasn't yet been approved for use, but full efficacy and safety data are expected sometime in 2025. In addition, United Therapeutics offers inhaled forms of treprostinil, which was initially approved to treat pulmonary arterial hypertension, as well as pulmonary hypertension associated with ILD. New data indicate the medication could also benefit patients with IPF who don't have pulmonary hypertension, Horowitz said. The ongoing trial is enrolling patients across the United States. Other ongoing studies include lysophosphatidic acid, a bioactive lipid mediator that can affect lung inflammation and fibrosis, and bexotegrast, a dual selective inhibitor of α v ß 6 and α v ß 1 integrins developed to treat IPF. Although Pliant Therapeutics announced the discontinuation of a phase 2b trial in March, early data showed efficacy for improved FVC. 'I'm optimistic that the next breakthrough is just around the corner,' Horowitz said. 'After 15 years of doing high-quality, informative studies, we're now opening the doors for new therapeutic targets, and as long as we keep doing trials, we're going to make a breakthrough that's going to transform care for these patients.' Horowitz and colleagues are also studying the cellular matrix and cell death of fibroblasts, including the way lung cells interact with other cells in an aberrant wound repair response, ultimately leading to lung scarring. The latest research is focused on enhancing cell susceptibility to apoptosis, or cell death, and decreasing disease progression. 'These lung diseases are heterogeneous, just like cancer. So viewed through the lens of cancer biology, different patients with their own fibrotic diseases have underlying mechanisms that drive the disease process,' Horowitz said. 'We're pursuing the idea that, if we can target the metabolic pathways that cells use, it might be beneficial for developing therapeutics.' Additional developments are occurring in diagnosis and patient care as well, particularly with a focus on genetic testing and coordinated care across specialists. 'The landscape of ILD treatment is evolving with the introduction of new pharmacological agents, advanced diagnostic techniques, and improved interdisciplinary care models and offers a brighter outlook for patients and healthcare providers,' Kulkarni said. 'Looking ahead to 2025 and beyond, as our understanding of disease pathogenesis continues to grow, the integration of precision medicine and genetic insights has the potential to make patient-centered, individualized care a reality.' Kulkarni, Lago, and Horowitz reported receiving grants, consulting fees, and serving in advisory roles for numerous pharmaceutical and medical organizations.

PureTech Announces Publication of New Research Highlighting Untold Experiences of People Living with Idiopathic Pulmonary Fibrosis (IPF)
PureTech Announces Publication of New Research Highlighting Untold Experiences of People Living with Idiopathic Pulmonary Fibrosis (IPF)

Business Wire

time09-05-2025

  • Health
  • Business Wire

PureTech Announces Publication of New Research Highlighting Untold Experiences of People Living with Idiopathic Pulmonary Fibrosis (IPF)

BOSTON--(BUSINESS WIRE)-- PureTech Health plc (Nasdaq: PRTC, LSE: PRTC) ('PureTech' or the 'Company'), a clinical-stage biotherapeutics company dedicated to changing the lives of patients with devastating diseases, today announced the publication of new research in BMC Pulmonary Medicine that provides a comprehensive view into the lived experiences of people with idiopathic pulmonary fibrosis (IPF). Conducted in collaboration with Tejaswini Kulkarni, MD, MPH, from the University of Alabama at Birmingham, Lisa Lancaster, MD, from Vanderbilt University Medical Center, and Jessica Shore, RN, PhD, from the Pulmonary Fibrosis Foundation (PFF), the study identifies critical barriers to treatment, opportunities for improved community engagement, and the need for patient-centered approaches in the development of new therapeutics. Initial findings from this research were presented at the 2023 Pulmonary Fibrosis Foundation Summit, the American Thoracic Society 2024 International Conference, and the CHEST 2024 Annual Meeting. 'It's remarkable that despite the availability of approved therapies, antifibrotic uptake remains persistently low. This lack of engagement reflects not just clinical barriers but fundamental gaps in understanding the experiences of people living with IPF, and we wanted to highlight their voices in this study,' said Camilla Graham, MD, MPH, Senior Vice President of Medical Affairs at PureTech and lead author of the study. "By integrating the perspectives of people with IPF into every stage of diagnosis, disease management, education, and clinical development, we have a real opportunity to shift the paradigm for IPF care to one that is more empathetic, effective, and aligned with the realities of living with this serious disease. This study is just the beginning; academic centers, professional societies, advocacy organizations, and pharmaceutical companies need to continue to collaborate on patient-informed innovation that bridges the current gaps in diagnosis, treatment, and quality of life." "As a clinician and researcher deeply involved in the care of individuals with IPF, I often witness the challenges patients face in understanding their diagnosis and treatment options,' said Dr. Kulkarni. 'This work uniquely captures the real-world experiences of people living with IPF, bringing to light critical gaps in education, awareness, and early intervention that we see play out in everyday clinical practice. By integrating these perspectives into our clinical strategies and research priorities, we have the opportunity to drive more empathetic, effective care that aligns with what truly matters to those living with IPF.' "At the Pulmonary Fibrosis Foundation, our mission is to accelerate the development of new treatments and improve the quality of life for people affected by pulmonary fibrosis. This study provided an opportunity to truly listen to patients, capturing their experiences with diagnosis, antifibrotic therapy, and oxygen use—areas where we know there are gaps in understanding and support,' said Dr. Shore. 'These findings highlight the critical need for high-quality educational resources, to strengthen advocacy efforts, and to enhance collaboration with healthcare providers to ensure that patient voices remain at the center of care decisions." Key research highlights include: The need for enhanced disease awareness and education Diagnosis timelines varied widely, with some participants experiencing years of delays and extensive evaluations for other conditions before receiving an IPF diagnosis. These findings underscore the need for an increase in awareness campaigns and more efficient diagnostic protocols to ensure earlier identification and intervention. While the majority of participants expressed satisfaction with their healthcare provider interactions, most also turned to the internet and support groups for information. This underscores the need for widely accessible, high-quality resources about IPF and its management. IPF affected many aspects of participants' lives, including their ability to keep up with family, work, travel, and household tasks, while also straining relationships with family and friends. This highlights the need to enhance management strategies that address not only the physical but also the emotional and psychological needs of people living with IPF and their caregivers. The perceived impact of antifibrotics and the need for clearer treatment expectations Of the participants who had never been treated with an antifibrotic, the most common reason for delay was that they were waiting until their symptoms worsened, highlighting a critical gap in understanding the potential benefits of early intervention. While the majority of participants on antifibrotics reported that their medication helped slow disease progression, many still found it difficult to perceive tangible benefits due to the lack of visible improvements. Additionally, side effects associated with antifibrotic treatments often impacted quality of life, leading some participants to reduce dosing or discontinue therapy – further complicating perceptions of effectiveness. Despite these challenges, most people taking antifibrotics shared that the treatment gave them hope. These findings emphasize the need for clearer communication about the expected benefits and limitations of antifibrotic therapy, as well as strategies to manage side effects and maintain therapeutic benefit. The study explored both the adverse effects participants ascribed to their antifibrotic treatment and the broader impact of managing those side effects on daily life. More than half adjusted their dosing to help manage adverse effects. These findings suggest opportunities for more patient-centered education on symptom management and highlight the need for better-tolerated medications to treat IPF. Clear, empathetic communication between healthcare providers and patients is essential to set realistic expectations, manage consequences of IPF and side effects of antifibrotic treatment, and empower patient engagement with all aspects of their care. The need for coordinated care to manage multiple comorbidities Nearly 90% of study participants reported managing additional health challenges alongside IPF, underscoring the importance of coordinated care among IPF-treating providers and the broader care team. The prevalence of comorbidities suggests that improving communication and strategic care planning across specialties could improve outcomes and overall quality of life for people living with IPF. Integrated care pathways that address both IPF and its comorbidities could significantly reduce the burden on people living with IPF and their caregivers. The need for holistic support in managing the benefits and burdens of supplemental oxygen While participants widely recognized the symptom relief provided by supplemental oxygen therapy, many described the associated logistical challenges, daily disruptions, financial burdens, and emotional toll. These insights underscore the need for treatment planning that accounts for both the benefits and challenges of oxygen therapy, while exploring innovations in delivery and holistic support to enhance quality of life. The research, which included 106 people living with IPF across the United States, utilized both qualitative interviews and online surveys to capture their experiences. Sponsored by PureTech, the study underscores the Company's commitment to amplifying patient voices in the development of breakthrough treatments. By understanding the lived experiences and unmet needs of people with IPF, PureTech aims to drive meaningful advances in care, education, and therapeutic development that are deeply reflective of patient realities. The paper, titled ' Perspectives of People Living with Idiopathic Pulmonary Fibrosis: A Qualitative and Quantitative Study,' is now available on the journal's website. Acknowledgments and Appreciation PureTech would like to extend its sincere gratitude to all the participants who generously shared their time and experiences to make this research possible. PureTech is also grateful to the Pulmonary Fibrosis Foundation and Breathe Support Network, whose support was instrumental in developing a comprehensive study that reached a diverse group of participants to provide these critical insights. PureTech is proud to amplify the voices of those living with IPF and remains committed to fostering patient-driven innovation and care that is reflective of real-world needs. About Idiopathic Pulmonary Fibrosis (IPF) Idiopathic Pulmonary Fibrosis (IPF) is a rare, progressive and fatal lung disease characterized by irreversible scarring of lung tissue. Median survival following diagnosis is estimated to be two to five years. 1 IPF affects more than 230,000 people across the United States and EU5 (France, Germany, Italy, Spain, and the United Kingdom). 2 Although two therapies are approved to treat IPF, their use remains limited, and nearly three out of four people with IPF in the United States have never received either treatment. 3 There remains a significant need for therapies that can more effectively slow or stabilize disease progression, improve long-term outcomes, and maintain quality of life for people living with IPF. About PureTech Health PureTech is a clinical-stage biotherapeutics company dedicated to giving life to new classes of medicine to change the lives of patients with devastating diseases. The Company has created a broad and deep portfolio through its experienced research and development team and its extensive network of scientists, clinicians, and industry leaders that is being advanced both internally and through its Founded Entities. PureTech's R&D engine has resulted in the development of 29 therapeutics and therapeutic candidates, including three that have been approved by the U.S. Food and Drug Administration. A number of these programs are being advanced by PureTech or its Founded Entities in various indications and stages of clinical development, including registration-enabling studies. All of the underlying programs and platforms that resulted in this portfolio of therapeutic candidates were initially identified or discovered and then advanced by the PureTech team through key validation points. For more information, visit or connect with us on X (formerly Twitter) @puretechh. Cautionary Note Regarding Forward-Looking Statements This press release contains statements that are or may be forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995. All statements contained in this press release that do not relate to matters of historical fact should be considered forward-looking statements, including without limitation those related to our idiopathic pulmonary fibrosis program and development plans, our future prospects, developments and strategies. The forward-looking statements are based on current expectations and are subject to known and unknown risks, uncertainties and other important factors that could cause actual results, performance and achievements to differ materially from current expectations, including, but not limited to, those risks, uncertainties and other important factors described under the caption "Risk Factors" in our Annual Report on Form 20-F for the year ended December 31, 2024, filed with the SEC and in our other regulatory filings. These forward-looking statements are based on assumptions regarding the present and future business strategies of the Company and the environment in which it will operate in the future. Each forward-looking statement speaks only as at the date of this press release. Except as required by law and regulatory requirements, we disclaim any obligation to update or revise these forward-looking statements, whether as a result of new information, future events or otherwise. ____________________ 1 Fisher, M., Nathan, S. D., Hill, C., Marshall, J., Dejonckheere, F., Thuresson, P., & Maher, T. M. (2017). Predicting Life Expectancy for Pirfenidone in Idiopathic Pulmonary Fibrosis. Journal of Managed Care & Specialty Pharmacy, 23(3-b Suppl), S17–S24. 2 GlobalData Epidemiology and Market Size Search, EU5=United Kingdom, France, Germany, Italy and Spain 3 Dempsey TM, Payne S, Sangaralingham L, Yao X, Shah ND, Limper AH. Adoption of the Antifibrotic Medications Pirfenidone and Nintedanib for Patients with Idiopathic Pulmonary Fibrosis. Ann Am Thorac Soc. 2021 Jul;18(7):1121-1128 Expand

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