Pulmonary Hypertension Awareness Month : Two European Studies Highlight the Urgency of Patients and Experts to Improve the Diagnosis and Treatment of Pulmonary Hypertension Associated With Interstitial Lung Disease

Within the month of awareness of pulmonary hypertension, two studies carried out in Europe have been published, highlighting the urgency of patients and doctors to improve the diagnosis and treatment of pulmonary hypertension associated with interstitial lung disease.

PH is a condition characterised by elevated blood pressure in the pulmonary arteries (the arteries that carry blood from the heart to the lungs)², whereas ILD are a group of disorders that cause scarring and inflammation of the lung tissue, leading to reduced lung function and difficulty in oxygen transfer². When PH develops with ILD, it complicates the disease, making it more severe and harder to manage².

On the one hand, a qualitative research carried out by clinical experts, the Pulmonary Hypertension Association Europe (PHA Europe) and the European Pulmonary Fibrosis Federation (EU-PFF) representatives, ORPHA Strategy Consulting, and Ferrer, a B Corp-certified international pharmaceutical company, has highlighted for the first time in Europe the lived experiences, perspectives, priorities, and viewpoints of patients and carers living with PH-ILD¹.

The study, published in the peer-reviewed journal Pulmonary Circulation, gathers insights from people living with this debilitating disease and finds that early recognition of PH-ILD symptoms could be life-changing. Patients and carers reported major difficulties and barriers in navigating the healthcare system and obtaining adequate information to reduce their considerable uncertainties.

As often occurs in rare conditions, there is a lack of standardized approaches for the diagnosis of PH-ILD in routine clinical practice. “It takes an average of 2-3 years and at least 3 doctors for individuals to receive an accurate diagnosis of PH-ILD due to the complex nature of this condition,” says Hall Skaara from PHA Europe.

Despite current management, the burden of the disease remains high, affecting the patients' daily wellbeing and the unmet medical needs persist given the lack of satisfactory treatments, poor prognosis, and decreased rates of survival. This confirms the substantial challenges that rare and complex conditions such as PH-ILD pose for routine clinical practice beyond PH expert centres. “Time matters and early detection can lead to better outcomes,” emphasizes Dr Lucilla Piccari, a pulmonologist from Hospital del Mar in Barcelona (Spain) and co-author of the study.

“Symptoms – especially shortness of breath – and physical limitations were identified as the most critical impacts for people living with PH-ILD,” says Prof Gabor Kovacs from the University of Graz (Austria). Reduced mobility was the biggest physical limitation, impacting patients' ability to perform daily activities, hobbies, sports, and to enjoy travel. “Expectations for PH‐ILD treatments –a cure aside – thus concentrated on an improvement of patients’ quality of life, particularly related to disease symptoms and physical functioning,” explains Steve Jones, a representative from EU-PFF.

The participants in the study were patients with a confirmed diagnosis of PH-ILD and their spouses, relatives, or close friends with the role of primary carer.

The results of the study are similar to those obtained by a previous one carried out in the United States³. While specific treatments are approved in the US for PH-ILD, none is currently available in Europe.

The challenges in PH-ILD management

A recent questionnaire-based survey has also unveiled insights into the clinical perspective of PH-ILD in Europe, specifically in France, Germany, Italy, Spain, and the UK⁴. Despite observed country-specific differences, the study highlights considerable unmet needs in the management of this rare disease, including the demand for robust screening and diagnostic protocols, and the need for effective and approved therapies across Europe.

The participants were experienced clinical pulmonologists, cardiologists and rheumatologists who currently manage a median of 110 ILD, 73 PH and 20 PH-ILD patients annually.

Most clinicians highlighted a lack of efficacy of off-label therapies that they prescribed to manage the PH component as a key gap in PH-ILD management. Regarding approach to ILD treatment, clinicians reported using immunosuppressant and antifibrotic medications in a number of their PH-ILD patients. These and other findings were recently published in an article by Prof. David Montani and other experts in the peer-reviewed journal ERJ Open Research.

The clinicians’ comments reinforce the notion that efforts should be focused on education, the multidisciplinary collaboration of experts and generating more evidence on PH-ILD and its subtypes. This will lead to the implementation of diagnostic and treatment guidelines and improved outcomes for this severe patient group.

Researchers said that additional qualitative research to expand on these findings could support the patient-centred design of future clinical trials, effectively incorporating what matters most to patients and carers.

References

1. Piccari L, Kovacs G, Jones S, et al. The European Voice of the Patient living with pulmonary hypertension associated with interstitial lung disease: Diagnosis, symptoms, impacts, and treatments. Pulm Circ. 2024 Jun 22;14(2):e12405.
2. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension [published correction appears in Eur Heart J. 2023 Apr 17;44(15):1312]. Eur Heart J. 2022;43(38):3618-3731
3. DuBrock HM, Nathan SD, Reeve BB, et al. Pulmonary hypertension due to interstitial lung disease or chronic obstructive pulmonary disease: a patient experience study of symptoms and their impact on quality of life. Pulm Circ. 2021;11(2):20458940211005641.
4. Montani D, Cifrián JM, Rojo RP, et al. Unmet need in pulmonary hypertension-associated interstitial lung disease (PH-ILD): a clinician survey of real-world management of PH-ILD in Europe. ERJ Open Res. 2024;10(4):00039-2024.

About Ferrer

At Ferrer we use business to fight for social justice. We have long been a company that looks to do things differently; instead of maximizing shareholder returns, we reinvest much of our profit in initiatives that give back to society. Back where it belongs. We go beyond compliance and are guided by the highest standards of sustainability, ethics and integrity. As such, since 2022, we are a B Corp.

Founded in Barcelona in 1959, Ferrer offers transformative solutions for life-threatening diseases in more than one hundred countries. In line with our purpose, we have an increasing focus on pulmonary vascular and interstitial lung diseases and rare neurological disorders. Our 1,800-strong team is driven by a clear conviction: our business is not an end in itself, but a way to change lives.

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