Optimism for Interstitial Lung Disease–Associated Pulmonary Hypertension? - nejm.org
Optimism for Interstitial Lung Disease–Associated Pulmonary Hypertension? - nejm.org |
- Optimism for Interstitial Lung Disease–Associated Pulmonary Hypertension? - nejm.org
- COVID Vaccine for Patient With Rare Disease? Best Guess Is Yes - MedPage Today
- Previous: Top 10 Pulmonary Hypertension Stories of 2020 - Pulmonary Hypertension News
- New Hope Against Diseases Marked by Progressive Scarring of Lung Tissue - HealthDay News
| Optimism for Interstitial Lung Disease–Associated Pulmonary Hypertension? - nejm.org Posted: 13 Jan 2021 12:00 AM PST  Pulmonary hypertension scares us, and for good reason. Pulmonary hypertension may be caused by an intrinsic pulmonary vasculopathy — termed pulmonary arterial hypertension — or it may complicate other, more common problems such as interstitial lung disease, in which it signals the presence of advanced, often end-stage disease.1 Regardless of its cause, without effective therapy, pulmonary hypertension is associated with worsened dyspnea, mobility, quality of life, and short-term survival. Fortunately, remarkable progress in drug development has transformed a diagnosis of pulmonary arterial hypertension from a disease that is uniformly and rapidly fatal into one that in some patients may be . . .  |
| COVID Vaccine for Patient With Rare Disease? Best Guess Is Yes - MedPage Today Posted: 21 Jan 2021 12:31 PM PST Extrapolating from the limited data from the Pfizer/BioNTech and Moderna COVID-19 vaccine trials, clinicians say the products' benefits likely outweigh the risks for people with pulmonary hypertension and other rare diseases. "The reality is folks with rare diseases will never have enough to do the trials out of the gate," said Tom Maddox, MD, MSc, of BJC HealthCare and Washington University School of Medicine in St. Louis. Without hard data, he said, clinicians and researchers can only postulate why COVID vaccines would work differently in people with pulmonary hypertension. "What do we understand about how the disease works? How the infection works? How the vaccine works? Is there anything that can cause the vaccine to worsen the disease? Any side effects?" "The way mRNA vaccines work, the way pulmonary hypertension works, nothing really occurs to us as problematic," Maddox said in an interview. "I'm not seeing any reason to believe [vaccination] would be dangerous to folks with pulmonary hypertension, also no reason to believe it wouldn't be as efficacious." In general, the primary safety events tied to COVID vaccination are transient flu-like symptoms; severe allergic reactions have been seen but only rarely. Accordingly, both Pfizer/BioNTech and Moderna vaccines include known allergy to vaccine components as contraindications -- not pulmonary hypertension, according to Justin Ortiz, MD, MS, of the University of Maryland School of Medicine in Baltimore and chair of the vaccines and immunization working group of the American Thoracic Society. In fact, the CDC includes people with pulmonary hypertension in phase 1c vaccination prioritization given that pulmonary hypertension -- encompassing several diseases that together affect an estimated 1% of the global population -- is recognized as one of the heart conditions that puts people at increased risk of severe illness from SARS-CoV-2 infection. In 2020, U.S. center directors reported hospitalization and mortality rates of 30% and 12%, respectively, among 50 COVID-19 patients with one of two forms of pulmonary hypertension, namely pulmonary arterial hypertension (PAH; WHO group 1) and chronic thromboembolic pulmonary hypertension (WHO group 4). In comparison, the COVID case fatality rate is just 1.7% in the U.S. general population, according to mortality data from Johns Hopkins. "We absolutely believe that PAH patients are a high-risk group" and "we are recommending vaccination for our patients," said Peter Leary, MD, PhD, of the University of Washington Medical Center, in an email to MedPage Today. Nevertheless, the mRNA vaccine data specific to the pulmonary hypertension population are sparse, if not nonexistent. For example, Pfizer/BioNTech's vaccine showed 95% efficacy in preventing COVID-19. The phase III trial enrolled more than 2,900 people with chronic pulmonary disease in the 43,548-person study, according to co-author Stephen Thomas, MD, of SUNY Upstate Medical University in Syracuse. Yet, no participant had pulmonary hypertension, a Pfizer representative confirmed. About 5% of participants in Moderna's 30,000-person trial, which reported 94.1% vaccine efficacy, had significant cardiac disease. Moderna did not clarify how many, if any, pulmonary hypertension patients participated in that trial, though probably no more than a few of those 5%. "In general, it makes sense for pulmonary hypertension patients to get the vaccine, but they certainly should talk to their physicians," Maddox cautioned. "Patients on blood thinning medicines or on medicines that affect their immune system are encouraged to discuss with their healthcare providers before they are vaccinated; however, in my opinion, in the vast majority of situations the benefits of vaccination will greatly outweigh the risks," said Ortiz. Last Updated January 21, 2021  |
| Previous: Top 10 Pulmonary Hypertension Stories of 2020 - Pulmonary Hypertension News Posted: 04 Jan 2021 05:00 AM PST Throughout 2020, Pulmonary Hypertension News brought daily coverage of groundbreaking research, treatment advancements, and clinical trial developments related to pulmonary hypertension (PH). As we anticipate bringing you more PH news this year, we take a look back at our top 10 most-read stories of 2020, with a summary of their significance for the PH community. No. 10 — "PAH Orenitram Drug More Cost-effective Than Uptravi, Study Suggests" A study found that in people with pulmonary arterial hypertension (PAH), treatment with Orenitram (oral treprostinil) is more cost-effective than Uptravi (selexipag). Both common treatments for PAH, the medications mimic the action of prostacyclin, a hormone that lowers blood pressure by opening blood vessels. Healthcare costs associated with Orenitram, marketed by United Therapeutics (which funded and participated in this study), were found to be 51.4% lower than those associated with Uptravi, and pharmacy costs 68.2% lower. A presentation at the European Society of Cardiology Congress reported that the Phase 3b TRITON clinical trial found the triple combination of oral Uptravi, Opsumit (macitentan), and tadalafil not to be more effective than Opsumit plus tadalafil at reducing pulmonary vascular resistance or other clinical disease markers in newly diagnosed PAH patients. The triple combination therapy, however, was more effective at slowing disease progression. No. 8 — "Imbalance in Gut Bacteria Composition May Play Key Role in PAH" A study in a rat disease model reported that a poorer gut microbiome diversity — a phenomenon called dysbiosis — may be involved in PAH onset and progression. Using a combination of antibiotics to alter gut bacteria composition, the researchers found that antibiotic-treated rats with PAH had lower strain and blood pressure in the right ventricle that pumps blood from the heart to the lungs. Antibiotic treatment seemed to prevent the enlargement of the heart's right ventricle, which tends to happen over time as PAH progresses, and lessened blood vessel narrowing and obstruction in the animals' lungs, another key hallmark of PAH. No. 7 — "PhaseBio Reports Positive Effects of PB1046 Therapy in PAH Patient in Case Study" A case study detailed a PAH patient who was successfully treated with PB1046, being developed by PhaseBio. The therapy is a combination of the company's proprietary elastin-like polypeptide biopolymer and vasoactive intestinal peptide — a hormone secreted by nerve cells that increases blood flow by opening blood vessels. The patient experienced a reduction in mean blood pressure of the pulmonary artery, and in blood flow resistance in the lungs. The treatment also increased the patient's stroke volume — the amount of blood the heart pumps out to the body in one heartbeat — and his cardiac output, or the amount of blood pumped by the heart in one minute, without raising his heart rate. No. 6 — "At-home System of Inhaled Nitric Oxide Treats PAH Patient Infected With COVID-19 " In a case report, a 34-year-old woman with PAH complicated by COVID-19 infection was successfully treated with an at-home inhaled nitric oxide tankless delivery system called Genosyl, developed by Vero Biotech, to lower blood pressure. The patient received inhaled nitric oxide for 12–14 hours each day for 17 days, before being weaned off treatment without incident. No. 5 — "PAH Candidate Sotatercept Granted FDA's Breakthrough Therapy Status" In April, Pulmonary Hypertension News reported that the FDA had designated sotatercept, an investigational molecule developed by Acceleron Pharma to treat PAH by improving pulmonary artery health and function, a breakthrough therapy. The designation conveys regulatory and approval incentives to potential treatments for serious or life-threatening conditions. No. 4 — "Pulmonary Hypertension Device by Aria CV Gets FDA Breakthrough Status" The FDA months earlier ad designated a medical product developed by Aria CV to treat pulmonary hypertension a breakthrough device. The system is designed to restore damaged arteries in the lungs, reduce blood pressure, and increase blood flow. It consists of a balloon that is implanted in the pulmonary artery, which inflates and deflates in sync with the heart cycle. The company believes the device will improve quality of life in patients with PH. No. 3 — "Case Report Suggests Lack of Vitamin C as PAH Cause" A case report described a man in his 60s with PAH brought on by severe vitamin C deficiency. The patient, who was hospitalized with dangerously high blood pressure along with a rash, sore joints, and difficulty breathing, had low vitamin C levels likely caused by a poor diet. He was treated with vitamin C and D supplements, which alleviated his symptoms over the next five months. PAH resolved following repletion of vitamin C. The case highlights the importance of checking vitamin C levels in patients experiencing PAH, its researchers suggested, and supports "the potential therapeutic role of vitamin C supplementation in patients who are deficient." In March, Vero Biotech released early details from the case described in article No. 6 on this list. The patient, a 34-year-old female physician, was the first person with PAH complicated by COVID-19 to receive the at-home Genosyl inhaled nitric oxide treatment. She was treated under the terms of an emergency investigational new drug application approved by the FDA. No. 1 — "Study Identifies Gut Bacteria That May Be Linked to PAH" A study found that the composition of the gut microbiome is unique in patients with PAH, and could serve as a diagnostic marker for the disease. PAH patients had a distinct gut bacterial profile, with less diversity and a low number of beneficial bacterial species compared with healthy controls. This specific bacterial profile was able to predict the presence or absence of PAH with 83% accuracy. According to the research team, the identification of specific gut microorganisms may lead to earlier, less invasive diagnoses, as well as new avenues for treatment. *** Pulmonary Hypertension News hopes our reporting in 2021 will continue to inform and improve the lives of everyone affected by PH. Wishing all our readers a happy and bright 2021.  Aisha Abdullah received a B.S. in biology from the University of Houston and a Ph.D. in neuroscience from Weill Cornell Medical College, where she studied the role of microRNA in embryonic and early postnatal brain development. Since finishing graduate school, she has worked as a science communicator making science accessible to broad audiences. × Aisha Abdullah received a B.S. in biology from the University of Houston and a Ph.D. in neuroscience from Weill Cornell Medical College, where she studied the role of microRNA in embryonic and early postnatal brain development. Since finishing graduate school, she has worked as a science communicator making science accessible to broad audiences. Latest Posts  |
| New Hope Against Diseases Marked by Progressive Scarring of Lung Tissue - HealthDay News Posted: 15 Jan 2021 09:05 AM PST  FRIDAY, Jan. 15, 2021 (HealthDay News) -- An inhaled medication might make every day physical activity a bit easier for patients with serious scarring of the lungs, a new clinical trial finds. The study, published online Jan. 13 in the New England Journal of Medicine, involved patients with high blood pressure in the lungs caused by interstitial lung disease (ILD). ILD is a broad term for progressive scarring of the tissue surrounding the lungs' air sacs and blood vessels. It can have a range of causes, from smoking, to occupational exposure to toxins like asbestos, as well as autoimmune diseases like rheumatoid arthritis. Sometimes, no cause can be found. A potential, and serious, complication of that scarring is pulmonary hypertension, in which the vessels that supply blood to the lungs become hard and narrow. Once pulmonary hypertension arises, patients can become so short of breath they have difficulty walking, and often need to use more supplemental oxygen. The complication may also shorten their lives. Right now, no medication is approved specifically for pulmonary hypertension caused by ILD, said Dr. Steven Nathan, senior researcher on the new trial. He's director of the Advanced Lung Disease and Lung Transplant Program at Inova Fairfax Hospital in Falls Church, Va. There are, however, drugs for another form of pulmonary hypertension, known as pulmonary arterial hypertension. Those medications are vasodilators, which means they help blood vessels in the lungs relax and widen. And Nathan's team found that one of them -- an inhaled medication called Tyvaso (treprostinil) -- improved exercise capacity in patients with ILD. Over 16 weeks, patients randomly assigned to use the drug were able to improve their performance on a six-minute treadmill walk. Beyond that, their lung disease remained more stable. About 23% showed a worsening, compared to 33% of patients randomly assigned to take a placebo (an inactive inhaled solution). The trial is the first to "unequivocally" show benefits of vasodilator medication for patients with pulmonary hypertension caused by ILD, according to Nathan. The study period was short, however, (16 weeks) and it's unclear what the long-term effects would be, including whether the therapy can help extend patients' lives. But current medications for pulmonary arterial hypertension have been approved on the basis of even shorter trials, Nathan pointed out. He said the new findings should be enough for the U.S. Food and Drug Administration to approve Tyvaso for ILD patients. "It's enough evidence for me to prescribe it for my patients," Nathan added. (Doctors are allowed to prescribe FDA-approved medications for unapproved uses when they believe it's medically appropriate.) Dr. Albert Rizzo, chief medical officer of the American Lung Association, described the results as encouraging. "These patients are very impaired," he said, and an improvement on the six-minute walk test is important. In part, that's because walking is a key part of managing the lung disease. "We want patients to stay active," Rizzo said, "and walking is the best exercise for them." A broader goal, he said, is to improve patients' quality of life. In this trial, patients using Tyvaso did not rate their quality of life any higher than placebo patients did. But the study's primary goal was to assess exercise capacity, which is an initial step, Rizzo said. Larger, longer studies are needed to answer questions about quality of life and survival, he added. The trial, funded by Tyvaso maker United Therapeutics, involved 326 patients. Half were randomly assigned to use the inhaler drug every day for 16 weeks; the other half received an identical-looking placebo. During that time, patients on the drug improved their walking distance on treadmill tests, while placebo patients generally declined. On average, Tyvaso patients managed to go about 100-feet farther. The main side effects were cough and throat irritation from using the inhaler. If Tyvaso is approved for ILD patients, Nathan foresees it as being another tool doctors can prescribe, along with oxygen therapy and pulmonary rehabilitation (which involves supervised exercise). Even though doctors can prescribe the drug without an official approval, Rizzo said patients should be aware that their insurance plan might balk at covering it. And the drug is costly, topping $100,000 a year, according to CVS Health. Rizzo noted that many patients with serious, progressive lung disease may not know whether they have pulmonary hypertension. He suggested they ask their doctors whether they've been tested for it, and if they do have the condition, whether there are any additional therapies they should be getting. More information The American Lung Association has more on interstitial lung disease. SOURCES: Steven Nathan, MD, director, Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, Va.; Albert Rizzo, MD, chief medical officer, American Lung Association, Chicago; New England Journal of Medicine, Jan. 13, 2021, online  |
![author['full_name']](https://clf1.medpagetoday.com/media/images/author/nicoleLou_188.jpg)
| You are subscribed to email updates from "pulmonary hypertension causes" - Google News. To stop receiving these emails, you may unsubscribe now. | Email delivery powered by Google |
| Google, 1600 Amphitheatre Parkway, Mountain View, CA 94043, United States | |
Comments
Post a Comment