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Lung Scarring: What To Know
Lung scarring, called pulmonary fibrosis, is a serious, permanent condition, but treatments are available.
Medically reviewed by Sanja Jelic, MDMedically reviewed by Sanja Jelic, MD
Lung scarring changes the normally thin tissue in the lungs into thick, scarred tissue. This makes it difficult for oxygen to reach your bloodstream, leaving you short of breath. Eventually, the illness can contribute to early death, although many people live years with the disease. Healthcare providers refer to lung scarring as "pulmonary fibrosis"—pulmonary means "lung," and fibrosis means "scarring." There are more than 100 conditions that can lead to lung scarring. Together, they are called interstitial lung disease. Oftentimes, the cause of lung scarring isn't clear, so healthcare providers call it idiopathic pulmonary fibrosis, meaning the cause isn't known.
Continue reading to learn more about pulmonary fibrosis symptoms, pulmonary fibrosis treatment, and how to live well with lung scarring.
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What Causes Lung Scarring?It's normal for the lungs to become damaged from environmental toxins, medications, lung disease, or illnesses, including COVID-19. In most cases, the body heals the damage, and the lungs function normally. However, in the case of more serious damage, scar tissue forms. Healthcare providers aren't sure why scar tissue forms in response to some lung damage, but not others, or why scarring worsens with time.
Any condition that causes inflammation and scarring in the lungs falls under the umbrella of interstitial lung disease, which covers more than 100 conditions. The scarring itself is called pulmonary fibrosis. Unfortunately, the scarring gets worse with time, which is why pulmonary fibrosis is a progressive disease.
Types of Lung ScarringThere are many different types of lung scarring. Pulmonary fibrosis is the term used to refer to more than 200 different diseases with similar symptoms and causes. Getting the most accurate diagnosis can help you get the most impactful treatments. Here is an overview of some of the most common.
Idiopathic pulmonary fibrosis (IPF)Idiopathic pulmonary fibrosis is the most common type of lung scarring. And yet, it may be such a big grouping because this term is used when healthcare providers don't know what caused the scarring. The word "idiopathic" means "of unknown cause."
There are certain risk factors for idiopathic pulmonary fibrosis, including:
Occupational lung diseasesOccupational lung diseases are illnesses caused by exposure to hazardous materials at work. Being exposed to dangerous materials on construction sites, mines, factories, and other workplaces can lead to lung scarring even years later. Some examples of occupational lung disease include:
COVID-19-related pulmonary fibrosisLung scarring is one of the potential long-term side effects of COVID-19 infection. It's more common in people who had severe COVID infection, as well as in males and older people.
Autoimmune diseasesAutoimmune diseases, including arthritis, sarcoidosis, and systemic sclerosis, can all cause lung scarring.
Drug-induced lung diseasesCertain medications can cause pulmonary fibrosis, including:
Chemotherapy drugs like bleomycin
Drugs used to treat abnormal heart rhythms, including amiodarone
Drugs used to treat inflammatory conditions, including methotrexate
Nitrofurantoin, an antibiotic used to treat urinary tract infections
The first symptoms many people with lung scarring notice are shortness of breath and a dry cough. Other symptoms of pulmonary fibrosis include:
Fatigue
Chest pain or discomfort
Anxiety, including from shortness of breath
Unexplained weight loss
Muscle aches
Widening and rounding of the fingers and toes (known as clubbing)
Lung scarring is permanent. Once you've been diagnosed, you'll be living with a chronic, progressive, life-limiting disease. For most people, the ability to breathe becomes more difficult over time. Still, medications and treatment can help. The most serious complication from lung scarring is death, but working closely with your healthcare team can help you stay active for longer.
How Is Lung Scarring Diagnosed?Healthcare providers will use a physical exam and extensive testing to diagnose lung scarring. Since lung scarring can be caused by things in your past (such as workplace hazards), it's important to provide the healthcare provider with a picture of your health over your whole life, not just now.
After taking your health history, a healthcare provider will often listen to your lungs and measure your oxygen level using a pulse oximeter. They'll then use tests and imaging, including:
Sometimes, your healthcare provider might ask you to exercise, like walking on a treadmill, to see how your lungs function during movement.
How Are Scars on the Lungs Treated?There's no way to cure lung scarring, but there are treatments for pulmonary fibrosis that can slow the progression of the disease and help you feel better. Your treatment goals will be based on your age, health, activity level, and daily activities that you wish to maintain. It's important to be open and honest with your healthcare provider about the impact of certain treatments and any side effects.
MedicationsDepending on the type of lung scarring you have, medications can help:
Antifibrotic agents, which slow the spread of scarring, are approved for use in people with idiopathic pulmonary fibrosis. The two medications are nintendanib (Ofev) and pirfenidone (Esbriet).
Steroids. Some types of lung scarring respond to steroids that reduce inflammation.
Immunosuppressant drugs can reduce inflammation if an autoimmune disease is contributing.
Cough medications, including over-the-counter and prescription options.
Anti-acid medications, since acid reflux is common in people with lung scarring.
Non-medication treatments can also help with the symptoms of lung scarring. These treatments include:
Oxygen therapy: Delivers concentrated oxygen via a nose cannula. This can help more oxygen reach your bloodstream and reduce symptoms like shortness of breath and fatigue.
Pulmonary rehabilitation: A type of therapy focused on improving lung function.
Lifestyle changes: Exercising, eating well, and reducing stress can all help fight symptoms of lung scarring.
A lung transplant is a good treatment option for some people with lung scarring. It's important to get evaluated for a lung transplant soon after diagnosis, even if you're not sure you would want to choose this treatment.
Lifestyle Tips to Find ReliefGetting the diagnosis of lung scarring can be really frightening. Living a healthy lifestyle and nurturing your physical and emotional health can help. Here are some tips:
Connect with other patients: Support groups can help you find people who are in a similar situation.
Talk about mental health: Getting diagnosed with a chronic disease can increase your risk for depression and anxiety. Talk with your healthcare provider and get mental health support any time you need it.
Keep exercising: Exercise can make you feel much better. It reduces shortness of breath by strengthening the heart and lungs.
Eat well: Nutrition plays a role in overall wellness as you live with lung scarring. Ask your healthcare provider for a referral to a nutritionist.
Take care of your lungs: Reduce your exposure to smoke, irritants, and pollution. Avoid high altitudes if possible.
The average life expectancy for someone with lung scarring is three to five years. However, some people live much longer than that. Early diagnosis and treatment can help prolong your life. For the most accurate prognosis, have an honest conversation with your healthcare provider.
SummaryLung scarring, known medically as pulmonary fibrosis, can make it difficult to breathe in enough oxygen. When that happens, you may experience symptoms including shortness of breath, fatigue, and coughing. There is no cure for lung scarring, but treatments, including medication, oxygen therapy, and rehabilitation, can help you maintain your quality of life for longer while living with this chronic disease.
Read the original article on Verywell Health.
Alarming Findings: COVID-19 Triggers Lethal Lung Disease In Previously Unaffected Populations
A University of Leeds study found a sharp increase in a rare autoimmune disease during the COVID-19 pandemic, primarily impacting previously unaffected groups. The study, which found a significant correlation with COVID-19 exposure, underscores the need for awareness and rapid treatment to mitigate severe outcomes like lung disease and death. Credit: SciTechDaily
Researchers discovered that during the COVID-19 pandemic, there was a surge in a rare autoimmune disease among previously unaffected demographic groups, leading to the deaths of nine patients.
The disease is an autoimmune response where the immune system mistakenly attacks its own sentinel proteins, which are help detect viruses. It is predominantly observed in East Asian females and is typically very uncommon in the UK. However, researchers from the University of Leeds and Leeds Teaching Hospitals NHS Trust observed an unprecedented 60 cases in Yorkshire during the pandemic, mainly among white men and women. Their findings were published in The Lancet eBioMedicine.
COVID-19 as a Trigger for Autoimmune ResponseThe rise in cases is believed to be triggered by exposure to the COVID-19 virus. This condition manifests through distinctive skin rashes, pneumonia, and interstitial lung disease—a rapidly progressing type of lung inflammation that is often fatal.
"Whilst we very occasionally see this disease in the UK, this surge in cases was completely new and very different," said Principal Investigator Dennis McGonagle, Professor of Investigative Rheumatology at the University of Leeds.
"It is important that physicians understand the symptoms so that patients can be quickly referred for treatment and have the best chance of a rapid and full recovery. Lives could undoubtedly be saved but there is a great need for research to try and slow or stop the rapid lung progression that occurs in some patients," he adds.
Understanding MDA5 and Autoimmune ResponseThe immune system contains a protein called MDA5, which helps detect RNA viruses like COVID-19. Normally, this protein helps trigger an immune response in the body, where more of the protein is produced to help fight off the virus. However, sometimes the immune system releases antibodies that mistakenly attack this protein, leading to MDA5 autoimmune diseases such as the rare disease described in this study. The exact cause is not well understood, but scientists believe the virus itself could trigger the response. Autoimmune diseases seen after viral infections have similar features, such as fatigue, joint pain and swelling, skin rashes, and digestive issues.
During the COVID-19 pandemic, physicians saw a rare condition in children called multisystem inflammatory syndrome in children (MIS-C) where there was no evidence of active viral infection of the lungs. This MIS-C syndrome affects multiple systems in the body, including the heart, kidneys, brain, skin, eyes, and digestive organs, but typically not the lungs. The disease seen in Yorkshire was dubbed MIP-C (pronounced "mipsy") because of its similarities to MIS-C in that it occurred around the time of the pandemic but where active infection was usually absent.
Case Study and AnalysisThe 60 patients in the study presented at their GPs or to A&E with a range of symptoms including shortness of breath, muscle pain, rashes, and reduced blood flow to the fingers, known as Raynaud's disease. These symptoms are associated with autoimmune disease.
The patients were referred to rheumatology specialists for further testing and all were diagnosed with the condition. Of the 60 patients, 35 had received COVID-19 vaccinations, and 15 had previously tested positive for COVID-19. However, patients were not systematically tested for COVID-19, and some may have been infected, but were asymptomatic at the time. Such cases could have developed MDA5 disease due to an overreaction of their immune system to minimal exposure to the virus.
Twenty-five out of the 60 patients (41.7%) developed interstitial lung disease, and despite treatment with immunosuppressant medication, eight of those patients died. A ninth patient, who did not have interstitial lung disease, died from sepsis.
To understand the increase in cases of this rare condition, researchers collected data on the number of these tests between January 2018 and December 2022, alongside data on COVID-19 infections and vaccination, and information about each patient's symptoms. Analysis showed that just six cases of the rare condition had been diagnosed between 2018 and 2019. However, in the 3 years after 60 new cases appeared, eight cases were diagnosed in 2020; 35 cases were diagnosed in 2021 and 17 cases were diagnosed in 2022. Very few cases have occurred since then.
Patient Demographics and ObservationsPatients ranged in age from 43 to 71, with 36 of them being female.
Ethnicity:
Researchers noted that there was a strong overlap in 2021 between vaccination rates in Yorkshire and the surge in MDA5 disease cases. This peak, though, also occurred shortly after a community wave of coronavirus infection occurred in late 2021. A smaller overlap was seen between confirmed COVID-19 infection and MDA5. However, it was interesting to note that almost half (42%) of patients were not documented to have been vaccinated against COVID-19 before they tested positive for MDA5. Four children who tested positive for MDA5 were unvaccinated, pointing to the idea of an overreacting immune response after exposure to a virus.
McGonagle said: "We know that vaccines can trigger an immune overreaction, but given that not all of these patients were vaccinated against COVID-19, and the increase in cases occurred when the COVID-19 virus was circulating in significant numbers, the evidence strongly suggests that the increase in cases of this rare disease is linked to exposure to the virus."
This work was done in collaboration with Dr Pradipta Ghosh and her team at the University of California, San Diego, who showed that the MDA5 protein was found in higher amounts in patients with COVID-19 and in patients with other diseases involving MDA5. They discovered that this increase was linked to an abnormal immune response that slows the virus's ability to multiply and spread. Additionally, they found that higher levels of the MDA5 protein were associated with increased levels of Interleukin-15, a protein that activates T-cells, or immune cells. This interaction could be contributing to the autoimmune response.
Impact and Significance of ResearchThe study highlights the critical need for awareness among doctors about this condition to ensure prompt diagnosis and treatment, which could mitigate lung damage and save lives. This is especially so in subjects with suspected pneumonia not getting better on therapy and where the rashes, muscle involvement, and other disease features are absent.
"We think that this large, unprecedented outbreak of MDA5 disease in Yorkshire will help advance the field to better appreciate the role between viruses and autoimmunity," said Dr Paula David, the lead author of the study.
Dr. Saptarshi Sinha, the co-first author of the paper and the interim director of PreCSN, added: "Here at PreCSN, we enjoy the ability to help researchers such as the McGonagle group dive into big data and find patterns rapidly with precision that allows us to connect the dots. In this case, we are glad we could find a connection between a clinical presentation of autoimmune disease in the backdrop of COVID-19 at a molecular level."
For more on this study, see Entirely New COVID-Related Syndrome Discovered.
Reference: "MDA5-autoimmunity and interstitial pneumonitis contemporaneous with the COVID-19 pandemic (MIP-C)" by Paula David, Saptarshi Sinha, Khizer Iqbal, Gabriele De Marco, Sahar Taheri, Ella McLaren, Sheetal Maisuria, Gururaj Arumugakani, Zoe Ash, Catrin Buckley, Lauren Coles, Chamila Hettiarachchi, Emma Payne, Sinisa Savic, Gayle Smithson, Maria Slade, Rahul Shah, Helena Marzo-Ortega, Mansoor Keen, Catherine Lawson, Joanna Mclorinan, Sharmin Nizam, Hanu Reddy, Omer Sharif, Shabina Sultan, Gui Tran, Mark Wood, Samuel Wood, Pradipta Ghosh and Dennis McGonagle, 8 May 2024, eBioMedicine.DOI: 10.1016/j.Ebiom.2024.105136
The data was generated by Dr Paula David and Dr Gabriele DeMarco from the University of Leeds' School of Medicine and the Leeds Teaching Hospitals NHS Trust, with their Trust colleague Dr Khizer Iqbal.
Study: ENV-101 Improves IPF Lung Function, Reverses Fibrosis
Endeavor BioMedicines' ENV-101 improved lung function and reversed key signs of lung scarring in people with idiopathic pulmonary fibrosis (IPF), according to data from a completed Phase 2a trial.
The results, which also showed the treatment had an acceptable tolerability profile, were presented in a late-breaking oral presentation at the American Thoracic Society 2024 (ATS 2024) International Conference in San Diego.
Based on the results from the Phase 2a trial (NCT04968574), the company intends to start another Phase 2 trial of ENV-101 called WHISTLE-PF (NCT06422884), which is expected to include up to 320 adults with IPF and progressive pulmonary fibrosis (PPF).
"There is a tremendous need for IPF treatments that do more than slow the inevitable decline of lung function," Toby M. Maher, MD, PhD, professor and director of interstitial lung disease at the University of Southern California, said in a company press release. "These preliminary signs of clinical and antifibrotic activity suggest that ENV-101 has the potential to change the trajectory of this otherwise relentless disease."
In IPF, the accumulation of fibrosis, or scarring, in the lungs causes them to become stiffer and makes it harder to breathe, leading to symptoms like shortness of breath and cough.
Lung scarring in IPF is driven by the excessive activity of myofibroblasts, cells that are involved in wound healing and scar tissue formation. Myofibroblast formation and activation is driven by a signaling cascade called the Hedgehog pathway. ENV-101 is an oral therapy that blocks this pathway, which should reduce scar tissue formation and reverse fibrosis over time.
Lung function gains seen with ENV-101The Phase 2a trial, which was recently completed, assessed the safety and efficacy of ENV-101 in 41 adults with IPF. The patients were randomly assigned to receive 200 mg tablets of the treatment, or a placebo, once daily for 12 weeks, or about three months.
According to the presented results, patients treated with ENV-101 saw a significant improvement in lung function, with a mean increase of 1.9% in percent predicted forced vital capacity (ppFVC) and a mean increase of 200 mL in total lung capacity (TLC), over the start of the study, that is, its baseline. The amount of air that can be forcibly exhaled after a deep breath expressed as a percentage of normal is what ppFVC indicates. TLC measures the total volume of air in the lungs after a deep breath.
The patients on a placebo had a mean decline of 1.3% in ppFVC and a mean drop of 56 mL in TLC over the baseline.
Also, 80% of the patients taking ENV-101 saw an improvement in TLC, while 70% of those on a placebo had a TLC decrease.
The trial also assessed the effects of treatment on key measures of lung fibrosis as assessed by high-resolution computed tomography (HRCT). Results showed treatment with ENV-101 significantly decreased the absolute percent of quantitative interstitial lung disease — which measures the extent of lung fibrosis — by 9.4%, compared with a 1.1% increase in the placebo.
The most commonly reported side effects related to ENV-101 were changes in taste (57%), hair loss (52%), and muscle spasms (43%), all mild to moderately severe. No treatment-related serious side effects or clinically meaningful safety findings were reported.
"These trial results suggest ENV-101 could have transformational clinical benefits for individuals with IPF, who are in need of therapies that change treatment expectations — from slowing disease progression to potentially reversing it," said Paul A. Frohna, MD, PhD, Endeavor's chief medical officer. "We look forward to further evaluating ENV-101's potential in the upcoming WHISTLE-PF clinical trial."
The company recently announced the completion of $132.5 million in funding to support the clinical development of ENV-101 in IPF and PPF.
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