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Communication Can Boost Medication Persistence In PAH
Patients continued taking their medications for pulmonary arterial hypertension (PAH) when symptoms appeared to improve, according to a new survey.
Provider instructions and perceived symptom improvement are among the top reasons people with pulmonary arterial hypertension (PAH) persist with taking their medications, according to a new survey. However, the survey also found that many patients get health care information from unreliable sources like social media and that many health care providers (HCPs) overestimate their patients' medication adherence.1
The findings, which were published in the journal Pulmonary Circulation, highlight challenges associated with "treatment persistence"—the duration from initiation to discontinuation of therapy—among patients with PAH.
Both persistent and discontinuing patients listed forgetfulness, fear of adverse effects, and high costs as their top reasons for not taking their medications as prescribed.Image Credit: © fizkes - stock.Adobe.Com
Corresponding author Gabriela Gomez-Rendon, MD, MBA, of Actelion Pharmaceuticals US, and colleagues wrote that treatment persistence is particularly important in PAH because treatment can delay the progression of the debilitating, deadly disease.
"Studies show adherence to and persistence with PAH medication can be suboptimal, yet limited data are available on treatment persistence and its factors among US patients with PAH," they wrote.
A 2022 systematic review and meta-analysis found approximately 4 in 10 patients with PAH discontinue their medications, for reasons ranging from administration frequency to costs to adverse events.2
In the new study, Gomez-Rendon and colleagues focused their attention on 134 adult patients with PAH who took selexipag (Uptravi), macitentan (Opsumit), or both, in the 12 months prior to the survey.1 The investigators also surveyed 23 health care professionals from 23 different clinics. The surveys were conducted between 2019 and 2022.
Among patients who responded to the survey, 112 were considered to be persistent in their medication usage, whereas 22 had discontinued their medication. Eighty-three percent of patients who were persistent reported "always" taking their medication as prescribed, whereas only 45.5% of patients who discontinued said the same.
Overall, symptom improvement, feeling better, instruction from HCPs, and fear of getting sick if they did not take their medications drove patients to continue with their therapies. Both persistent and discontinuing patients listed forgetfulness, fear of side effects, and high costs as their top reasons for not taking their medications as prescribed. However, the reasons were different between the 2 groups; those who were persistent most commonly cited forgetfulness as the reason (13.4%), but the top reason for nonpersistence among people who discontinued was fear of adverse effects (36.4%).
Gomez-Rendon and colleagues said the findings underscore the importance of proactive communication from HCPs.
"Proactive discussion about side effects and their management, as well as patients' fear of side effects, is a key intervention to support treatment persistence," they said.
The investigators found it was not just lack of communication that complicated persistence, but also the type of communication. Greater than 95% of respondents said they received information about PAH from their HCPs, but half of respondents also said they used websites to gain information, and one-third (36.6%) said social media was a source of information. Yet, the investigators said patients who discontinued were more likely than those who were persistent to get information from websites and social media.
Another issue highlighted by the study was the apparent gap between clinicians' perceptions of patient persistence and patients' actual usage patterns.
"At low‐persistence sites (n = 11), HCP perception of persistence differed from actual persistence according to medication shipment rates (85.6% vs <40%, respectively)," the investigators wrote; at high‐persistence sites (n = 13), no such discrepancy existed (88.1% vs ≥80%)."
Gomez-Rendon and colleagues said further research is warranted to understand the reasons for the gap, but they suggested it may be because patients underreport missed medications to their providers, or because of communication delays between pharmacies and providers.
The investigators said they hope their study encourages physicians to seek out ways to improve communication with patients, including the use of social workers and case managers to help answer patients' questions and help connect them with financial resources.
"In summary, our study highlights the importance of continuous patient engagement and improvement of patient-HCP communication, with education on the disease and treatment," they wrote. "Early discussion and management of side effects is important in patients with PAH."
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Understanding Pulmonary Hypertension: Symptoms & Treatments
When most people hear the word hypertension, they think of high blood pressure. While not as common as high blood pressure, pulmonary hypertension, which affects the lungs, can be harder to diagnose, more challenging to treat, and, tragically, often comes with fatal consequences.
Dr. Talal Dahhan, Division Chief for Pulmonary & Critical Care at Baystate Pulmonary explains, "Nearly 3 to 8% of the world's population is affected by pulmonary hypertension (PH). A serious condition brought on by abnormally high pressure in the blood vessels between the lungs and the heart, most cases of PH cannot be cured. But with early detection and proper treatment, it is possible to improve symptoms and slow the disease's progression."
What is Pulmonary Hypertension?While the signs and symptoms of pulmonary hypertension are felt in the lungs, the cause starts in the heart. Dr. Dahhan explains, "In a healthy body, the heart does its job pumping blood around the body and delivering oxygen to organs and tissue. The oxygen gets taken up by the body and the blood cycles back to the heart. When all things are working well, the blood flows back into the heart and travels to the lungs via the pulmonary artery, picks up fresh oxygen, and the whole cycle begins again. However, in cases of PH, the walls of the pulmonary artery become stiff, damaged and narrow and the amount of oxygen going out to the rest of the body drops to dangerously low levels. Sensing the low levels of oxygen, the heart begins to pump harder to push oxygen-low blood to the lungs. Over time, the low level of oxygen in the blood and the added pressure on the heart can lead to serious complications, including heart failure."
What Causes Pulmonary Hypertension?There are a number of conditions and issues that can lead to PH. Once determined, the cause is used to classify a patient's condition by a Group number (Group 1 PH, Group 2 PH, etc.). This grouping information is later used to help identify appropriate treatment options.
Here's a look at the Groups and causes for PH:
Group 1 PH is due to pulmonary arterial hypertension (PAH), a condition in which the tiny arteries in your lung become thickened and narrowed. While PAH can occur due to unknow reasons (what physicians call idiopathic), some common causes include:
Group 2 PH is due to left-sided heart disease, the most common cause of PH in the United States, left-sided heart disease causes blood to back up in the heart, raising the pressure in the pulmonary arteries.
Group 3 PH is due to lung disease, including interstitial lung disease, chronic obstructive pulmonary disease (COPD), certain sleep apneas, pulmonary fibrosis, and emphysema, all of which reduce the flow of blood through the lungs and oxygen uptake.
Group 4 PH is due to blockages in the lungs and arteries, including clots and scars from chronic thromboembolic pulmonary hypertension (CTEPH).
Group 5 PH is due to other diseases and conditions, including blood disorders, kidney disease, inflammatory disorders, and metabolic disorders.
Dr. Dahhan notes that "PH can also be caused by a specific congenital heart called Eisenmenger syndrome. This occurs when there's a hole, or more than one, between the heart chambers, which causes blood to flow incorrectly in the heart, often increasing the blood flow and pressure in the pulmonary arteries."
No matter the cause, Dr. Dahhan notes that PH in all forms is a very serious condition and left untreated can be life threatening.
What Are the Symptoms of Pulmonary Hypertension?According to Dr. Dahhan, "PH symptoms aren't 'all of the sudden' type symptoms. They develop slowly over the years and are often like symptoms of common heart and lung conditions making them easy to miss."
Pulmonary hypertension symptoms include:
Dr. Dahhan adds, "Most people first recognize a problem when they become short of breath when walking. They may be fine on a flat surface but if there's an incline or they're going up stairs, they may find themselves suddenly out of breath. As the disease progresses, they may even feel out of breath when seated. Typically, that's alarming enough to drive someone to seek medical help."
How is Pulmonary Hypertension Diagnosed?As noted, diagnosing PH can be challenging as its symptoms often resemble those of other lung and heart conditions. In addition to performing a physical exam and reviewing your medical history, your doctor may perform the following:
Diagnostic Tests for raising index of suspicion if we have a possibility of Pulmonary HypertensionThe most definite test for diagnosing PH, right heart catheterization measures the blood pressure in the pulmonary arteries. This information identifies where the high pressure exists in your heart and arteries and the specific type (group) of PH the patient has.
What is the Treatment for Pulmonary Hypertension?While there's no cure for most forms of pulmonary hypertension, various treatments are determined by the type of PH. Treatment can help manage symptoms and improve quality of life.
Among the earliest treatments recommended to consider:The next course of treatment options are medications. "There are a number of medication therapies available to help with different types of PH," says Dr. Dahhan, "Depending upon the type of PH a patient has, we may recommend medications to relax or widen blood vessels and arteries, relax muscles in blood vessels, or to increase blood flow. These don't treat PH, but they can treat and help with complications from PH."
A last resort option, surgery may be recommended for patients with CTEPH, however, as Dr. Dahhan notes, not all PH patients are candidates for thromboendarterectomy. But there are procedures to consider in absence of CTEPH. Options include:
Living with pulmonary hypertension can be challenging, but with proper management and care, it is possible to maintain a good quality of life. "Early diagnosis and treatment are crucial," says Dr. Dahhan. "If you are experiencing persistent shortness of breath or other symptoms, reach out to your doctor to schedule an appointment as soon as possible. The more information you can bring to the appointment—how long have you had symptoms, what makes them worse or better, etc.—the easier it will be to identify if PH is behind your issue and the faster you can begin treatment."
GOLD COPD 2025 Report: Key Spirometry And Drug Updates, New Emphasis On CVD, PH
The Global Initiative for Chronic Obstructive Lung Disease (GOLD) released its 2025 updated version of the GOLD Chronic Obstructive Pulmonary Disease (COPD) report earlier this month, just in time for World COPD Day on November 20. Updates to the report, which include new sections on pulmonary hypertension, cardiovascular disease (CVD), and climate change, were detailed at the 2024 GOLD COPD International Conference in Philadelphia, on November 12, by Claus Vogelmeier, MD, GOLD Science Committee chair, who also offered clues on what to expect in the 2026 report.
According to Dr Vogelmeier, key changes to the 2025 GOLD COPD report include:
The science committee sifted through about 3000 research articles, identified through a systematic literature search, to determine updates that were needed to the 2025 report, said Dr Vogelmeier. After performing a double-blind review of the literature, the Science Committee added 164 new references to the report.
No longer included in the GOLD COPD report is a chapter on COVID-19 and COPD that appeared in previous reports.
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If the pre-bronchodilator spirometry does not show obstruction…performing post-bronchodilator spirometry is not necessary unless there is a very high clinical suspicion of COPD.
The 2025 GOLD COPD report, titled "Global Strategy for Prevention, Diagnosis and Management of COPD: 2025 Report," can now be downloaded from the GOLD website, along with a pocket guide to the report and the "2025 GOLD Teaching Slide Set."
Spirometry UpdatesIn the 2025 report, the spirometric criterion for a COPD diagnosis continues to be postbronchodilator ratio of forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) less than 0.7. However, the report authors note that a fixed FEV1/FVC ratio less than 0.7 may lead to overdiagnosis of COPD in elderly patients and underdiagnosis in 1% of young adults, particularly for mild disease, compared with a cutoff based on the lower limit of normal values for FEV1/FVC. For adults aged younger than 50 years with suspected COPD and a repeated fixed ratio of at least 0.7, comparing the ratio with a predicted LLN or with z-scores may aid in managing this group of patients, the report authors stated.
"Importantly, the risk of misdiagnosis and overtreatment of individual patients using the fixed ratio as a diagnostic criterion is limited, as spirometry is only 1 biologic measurement to establish the clinical diagnosis of COPD in the appropriate clinical context (symptoms and risk factors)," the report authors stated. "Diagnostic simplicity and consistency are crucial for the busy clinician. Thus, GOLD favors using the fixed ratio over LLN."
Pre-/Post-Bronchodilator SpirometryIn a major shift, the GOLD science committee now puts greater emphasis on the value of pre-bronchodilator spirometry. "If the pre-bronchodilator spirometry does not show obstruction…performing post-bronchodilator spirometry is not necessary unless there is a very high clinical suspicion of COPD, in which case an FVC [forced vital capacity] volume response may reveal obstruction," the report authors noted. In the event pre-bronchodilator spirometry does show obstruction, a COPD diagnosis should be confirmed with post-bronchodilator spirometry.
Airflow obstruction based on 1 measurement of the post-bronchodilator FEV1/FVC ratio should be confirmed by repeat spirometry during a separate occasion if the measurement is 0.6 to 0.8, the committee advised. If the initial postbronchodilator FEV1/FVC ratio is less than 0.6, it is unlikely to increase spontaneously to greater than 0.7.
GLI Global Reference Equations RecommendedNotably, the 2025 report stresses that "LLN values and z-scores are highly dependent on the choice of valid reference equations." The GOLD Science Committee now recommends the use of Global Lung Function Initiative (GLI) Global reference values, which were developed to be race-neutral. At the same time, report authors acknowledge that these equations have limitations, in that they are based on "a weighted average of racial and ethnic categories, were derived from a population that did not include participants from many countries or global regions, and ignore the observed population differences in body proportions." The report authors noted that "lung function reference values change over time and require periodic revision."
GOLD also continues to advise use of FEV1 as a percentage of the predicted value for staging airflow obstruction severity. The percentage of the predicted GLI-Global equation value may be determined with use of the online GLI calculator.
CVD in Patients With COPDAfter an acute exacerbation of COPD, patients have a 20 times greater risk for a severe CV event or all-cause death within 7 days, said Dr Vogelmeier, in explaining the Science Committee's rationale for including a new section on cardiovascular risk in COPD in the 2025 report.
Cardiovascular disease (CVD) is also increased among patients with clinically stable COPD and is associated with mortality, especially in those with mild to moderate airflow obstruction. CVD, including arterial hypertension, coronary artery disease, heart failure, and arrythmia, should be assessed in patients with COPD and treated according to available guidelines, the committee noted. During exacerbations of COPD (ECOPD), the risk for an acute cardiovascular event increases, especially among patients with severe ECOPD requiring hospitalization.
"Importantly, this risk remains high during the first few weeks after hospital discharge, and it can still remain significantly increased even 1 year later," the committee stated. The GOLD recommendations advise an appropriate differential diagnosis of ECOPD from other conditions such as heart failure and that routine measurement of markers of CVD, including troponin and brain natriuretic peptide levels, may be performed during ECOPD.
Using CT in COPD AssessmentCT can be used to assess the structural and pathophysiologic abnormalities in patients with COPD, and quantitative analysis is increasingly used for emphysema extent and location and can assist with endobronchial valve treatment decision-making. CT also is being used in patients with COPD for assessment of pulmonary nodules identified on chest X-ray or evaluation for concurrent lung disease. "We've learned that imaging is becoming more and more relevant," Dr Vogelmeier noted. Other relevant uses of CT include airway abnormalities such as bronchiectasis, as well as comorbidities such as coronary artery calcium, pulmonary artery enlargement, bone density, muscle mass, interstitial lung abnormalities, hiatal hernias, and liver steatosis.
Pharmacologic COPD TreatmentsFor follow-up pharmacologic treatment in patients with dyspnea for whom the addition of a second long-acting bronchodilator does not improve symptoms, the committee suggests switching inhaler device or molecules, implementing or escalating nonpharmacologic treatment, or considering adding ensifentrine if available.
EnsifentrineEnsifentrine is a first-in-class inhaled dual inhibitor of phosphodiesterase 3 and 4 (PDE3, PDE4) that has anti-inflammatory activity and bronchodilator effects. Phase 3 studies reported that when delivered with a standard jet nebulizer, ensifentrine led to significant improvement in lung function and dyspnea, but its effects on quality of life were inconsistent.
Dupilumab and Other TherapiesAmong patients who continue to have exacerbations after treatment with long-acting beta-2 agonists (LABA) plus long-acting muscarinic antagonists (LAMA) plus ICS, GOLD recommends consideration of dupilumab in those with eosinophil levels 300 cells/µL or more and symptoms of chronic bronchitis. Azithromycin may be considered in patients who are not current smokers, and roflumilast may be administered to patients with FEV1 less than 50%, chronic bronchitis symptoms, and a history of previous severe exacerbations.
Dupilumab is a human monoclonal antibody that blocks the shared receptor component for interleukin-4 and interleukin-13. In a pair of phase 3 trials, patients with COPD, chronic bronchitis, a history of exacerbations despite treatment, and blood eosinophil count of at least 300 cells/µL who were treated with dupilumab had a reduction in exacerbations, improved lung function, and improved health at 1 year. Dupilumab was approved by the US Food and Drug Administration as an add-on maintenance therapy for certain adults with COPD in October.
ICS UseAmong patients who have COPD with no features of asthma treated with LABA/ICS and are well-controlled for symptoms and exacerbations, continuing LABA/ICS in an option, according to GOLD. For patients with further exacerbations, treatment is recommended to be escalated to LABA/LAMA/ICS if the blood eosinophil count is at least 100 cells/µL or switched to LABA/LAMA if less than 100 cells/µL. Patients with major symptoms are advised to change to LABA/LAMA or LABA LAMA/ICS depending on prior ICS response.
Pulmonary Hypertension in COPDThe importance of pulmonary hypertension in COPD "has been underestimated as of yet," said Dr Vogelmeier, in explaining the rationale for the new section on PH in the 2025 report.
Pulmonary hypertension is classified into 5 groups based on pathophysiologic mechanisms, clinical presentation, and management, and all 5 groups can be present in patients with COPD. Diagnosing pulmonary hypertension in patients with COPD requires assessment of the potential mechanisms leading to increased mean pulmonary arterial pressure, and echocardiography is the best noninvasive tool for estimating the probability and severity of pulmonary hypertension, the committee noted. "Patients with pulmonary hypertension–COPD should be referred to a pulmonary hypertension center with experience in respiratory diseases where they will undergo right heart catheterization and multidisciplinary assessment to guide treatment decision," the committee stated. "Long-term oxygen therapy is recommended in hypoxemic patients."
PAH in COPDAmong patients with COPD and pulmonary arterial hypertension (PAH), treatment should adhere to the 2022 European Society of Cardiology/European Respiratory Society guidelines, the committee advises. The preferred treatment in this group of patients is initial monotherapy followed by careful sequential combination therapy if the goals are not achieved. For patients with COPD who are diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), a multidisciplinary CTEPH team evaluation will determine if pulmonary endarterectomy, angioplasty, and/or pulmonary hypertension medications are needed.
PAH medications are not advised in patients with nonsevere pulmonary hypertension–COPD. For patients with severe pulmonary hypertension–COPD, an individualized management approach is recommended, with off-label use of PDE5 inhibitors commonly suggested as treatment. Some research, however, reported that inhaled treprostinil had no clinical efficacy and was associated with potential adverse effects.
"Further well-designed randomized-controlled trials should be encouraged to firmly advise for or against the use of PAH medications in pulmonary hypertension–COPD," the committee stated.
Climate ChangeThe 2025 report addresses climate change in relation to COPD. Research has shown that patients with COPD have an increased mortality risk with exposure to heat and cold, with a higher risk for cold temperatures. Indoor temperatures and high indoor humidity also can affect COPD. Studies have found that higher outdoor temperature is associated with increased dyspnea, and higher indoor temperature is associated with increased symptoms and use of short-acting beta-2 agonists (SABA). Colder indoor and outdoor temperatures have been associated with an increase in cough, sputum, and SABA use and a decrease in FEV1.
"During heatwaves and periods of high temperature, patients should ensure they keep adequately hydrated, keep out of the heat, and try to keep living spaces less than 32°C and sleeping spaces less than 24°C as recommended by the World Health Organization," the committee advised.
Vaccinations and COPDGOLD recommends that patients with COPD receive all recommended vaccinations based on relevant guidelines. Influenza vaccination, for example, can reduce the risk of serious issues such as lower respiratory tract infections that require hospitalization, stroke, and mortality in patients with COPD. Pneumococcal vaccination may be co-administered with influenza vaccination in adults and has been shown to be immunogenic and safe. Researchers have found that respiratory syncytial virus (RSV) was associated with about 8% of outpatient-managed COPD exacerbations and may lead to as many as 10,000 deaths annually in older adults in the United States. Among adults with COPD, the CDC recommends Tdap vaccination in those who were not vaccinated in adolescence, as well as the shingles vaccine. GOLD also advises that patients with COPD receive the COVID-19 vaccine based on national recommendations.
Dysbiosis in COPD DevelopmentDysbiosis involves disruption of the microbiome and may occur in the airways of patients with COPD. The role of dysbiosis in the development and progression of COPD is addressed in a new section of the 2025 report. Dysbiosis has a cross-sectional association with COPD and multiple disease characteristics, including frequency of exacerbations, likely from mucosal defense alterations and stimulation of lung inflammation by immune responses. In addition, the microbiome profile is affected by viral infections and during exacerbations and by antibiotics and oral corticosteroids or ICS. Further research is needed on the effect of dysbiosis in COPD.
Pulmonary Rehab and TelerehabilitationThe COPD report chapter on "Non-pharmacological treatment of stable COPD" includes information about in-person and virtual delivery of pulmonary rehabilitation, education, and self-management. At the recent GOLD COPD conference, Vogelmeier told attendees that this section would undergo major revision in 2026.
The 2025 report states that an effective pulmonary rehabilitation program should include lower extremity endurance exercise training. Telerehabilitation requires appropriately trained staff and infrastructure and should not be misused, the authors noted. "Tele-education and self-management using information communication technology could have the potential to ease the working life of health practitioners and transform the way patients are monitored and health care is delivered," the group stated. "Despite the many advances in self-management, delivering self-management at distance using digital technology still raises many unanswered questions and important limitations." Presumably, these are the questions that may be addressed in next year's report.
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