Materiały źródłowe

• #1 Pulmonary fibrosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/symptoms-causes/syc-20353690

  Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it harder for the lungs to work properly. Pulmonary fibrosis worsens over time. Some people can stay stable for a long time, but the condition gets worse faster in others. As it gets worse, people become more and more short of breath. […] Symptoms of pulmonary fibrosis may include: Shortness of breath. Dry cough. Extreme tiredness. Weight loss that’s not intended. Aching muscles and joints. Widening and rounding of the tips of the fingers or toes, called clubbing. […] How fast pulmonary fibrosis worsens over time and how severe the symptoms are can vary greatly from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.

• #2 Pulmonary Fibrosis: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis

  Pulmonary fibrosis is scarring in your lungs. It can make it hard to expand your lungs and get enough oxygen to your body. Pulmonary fibrosis usually gets worse over time, but how quickly it gets worse is different for everyone. […] You might feel short of breath or get tired with everyday activities. […] Hard, stiff lung tissues dont expand as well as they should. If you have pulmonary fibrosis, you may get short of breath during daily tasks that never seemed tiring before. Pulmonary fibrosis is permanent and usually gets progressively worse over time (called progressive pulmonary fibrosis). […] Pulmonary fibrosis symptoms include: Shortness of breath, especially during or soon after exertion; Breathing in short, shallow spurts; Dry cough that doesnt go away; Fatigue; Unexplained weight loss; Clubbed fingers; Bluish, gray or white skin around your lips, eyes or nails (cyanosis).

• #3 What is pulmonary fibrosis? | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/what-pulmonary-fibrosis

  Pulmonary fibrosis is the buildup of scar tissue in your lungs. It makes breathing increasingly difficult. […] Most forms of pulmonary fibrosis tend to get worse over time. The likelihood of this happening and how quickly it happens varies with the type of pulmonary fibrosis, but also from person to person. This makes it difficult to predict exactly how fast symptoms may progress and change. […] All kinds of pulmonary fibrosis cause scarring in your lungs that make breathing more difficult. Scarring makes your lungs stiffer and less elastic so they’re less able to move and take oxygen from the air you breathe. […] If you have pulmonary fibrosis, scarring affects the air sacs in your lungs. The air sacs are supported by the interstitium, a network of supporting tissue. Scarring happens in the gaps between and around the air sacs and limits the amount of oxygen that gets into the blood. […] As scarring increases, your lungs are less able to expand to allow you to take deep breaths and the level of oxygen in your blood can start to drop. Breathing may feel like harder work and you can feel breathless from everyday activities like walking.

• #4 Pulmonary Fibrosis Progression and Exacerbation | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/living-well-with-pulmonary-fibrosis/progression-and-exacerbation

  Pulmonary fibrosis is a progressive disease that naturally gets worse over time. This worsening is related to the amount of fibrosis (scarring) in the lungs. As this occurs, a person’s breathing becomes more difficult, eventually resulting in shortness of breath, even at rest. […] Patients with pulmonary fibrosis experience disease progression at different rates. Some patients progress slowly and live with PF for many years, while others decline more quickly. […] Some patients will experience a sudden worsening of their pulmonary fibrosis, which is referred to as an acute exacerbation. This occurs when there is a triggering event that results in a sudden increase in the processes that lead to scarring. As the lung scarring gets worse, patients have a harder time breathing. The damage to the lungs during an acute exacerbation is permanent.

• #5 Pulmonary Fibrosis – What Is Idiopathic Pulmonary Fibrosis? | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/idiopathic-pulmonary-fibrosis

  Idiopathic pulmonary fibrosis (IPF) is a serious chronic (long term) disease that affects the tissue surrounding the air sacs, or alveoli, in the lungs. The most common symptoms of IPF are shortness of breath and cough. Some people may not have symptoms at first, but symptoms can develop and get worse as the disease progresses. The way that IPF progresses varies from person to person, and scarring may happen slowly or quickly. In some people, the disease stays the same for years. In other people, the condition quickly gets worse. Many people with IPF also experience what are known as acute exacerbations, where symptoms suddenly become much more serious. […] There is currently no cure for IPF. However, certain treatments may slow the progression of IPF and help your lungs work better. This may extend the lifespan and improve the quality of life of people who have the disease.

• #6

  https://www.pulmonaryfibrosis.org/understanding-pff/about-pulmonary-fibrosis/symptoms

  The most common symptoms of pulmonary fibrosis are dry, persistent cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating. […] Other common symptoms of pulmonary fibrosis include: Fatigue and weakness, Discomfort in the chest, Loss of appetite, Unexplained weight loss.

• #7 Pulmonary Fibrosis Symptoms | Temple Health

  https://www.templehealth.org/services/conditions/pulmonary-fibrosis/symptoms

  Pulmonary fibrosis symptoms tend to develop over time. They may be very mild early on and can worsen as more scar tissue develops in the lungs. How fast the symptoms of pulmonary fibrosis develop may differ significantly from person to person. Pulmonary fibrosis symptoms can come on quickly or may take years to develop. At first, you may not even notice that you have symptoms, or may simply shrug off your symptoms as a common condition. But over time, scarring in your lungs causes your lungs to become stiff. This makes it hard for your body to get the oxygen it needs. When your body has low oxygen, you may have symptoms such as cough and breathlessness. As the stiffness and scarring worsen, your symptoms may progress. […] The most common symptoms of pulmonary fibrosis are shortness of breath with exercise or exertion and dry cough that does not seem to get better. At first breathlessness, or shortness of breath, gets worse during exercise. As your condition worsens, you may feel breathless during normal everyday activities such as taking a shower, talking on the telephone or eating.

• #8 Early Symptoms | ILD Collaborative

  https://www.ildcollaborative.org/resources/ipf-patient-journey/early-symptoms

  Idiopathic pulmonary fibrosis (IPF) mainly affects people who are over the age of 50. IPF often starts with vague, annoying symptoms that do not seem very serious. People with early IPF may become breathless when walking upstairs, or climbing a hill. They may develop a nagging, dry cough. They may feel tired, even after a full nights sleep. Many people with early symptoms think that their symptoms are just a normal part of aging. […] It is important to take these early symptoms of IPF seriously. Feeling breathless or having a nagging cough are not a normal part of aging. Although IPF has no cure, treatments are available that can slow down lung damage, improve symptoms, and improve quality of life. These treatments work best when the condition is diagnosed early. […] There are three main early symptoms of idiopathic pulmonary fibrosis (IPF). These are: Breathlessness a feeling of being short of breath or out of breath. This is often more noticeable during exercise, such as walking upstairs or uphill. In early IPF, breathlessness may only happen during exercise. Later, patients may feel breathless even while resting. Cough usually dry and nagging. Coughing often comes in episodes for people with IPF. Fatigue a feeling of tiredness or weariness that is not relieved by sleeping. It may feel physical, emotional, or both. Fatigue may be worse after coughing episodes. For people with IPF, fatigue seems to worsen over time.

• #9 Idiopathic Pulmonary Fibrosis Symptoms | What to Look For

  https://www.nationaljewish.org/conditions/ipf/ipf-overview/symptoms

  The most common symptom of IPF is shortness of breath, also known as dyspnea. Many people describe this as a feeling of breathlessness. […] The earliest symptom of IPF is shortness of breath with exertion or strenuous activity. Many people ignore this symptom or ascribe it to being old or out of shape and simply reduce the amount of physical activity they do. For example, earlier in the course of IPF, you may notice shortness of breath only when running or hurrying to catch a bus. Later in the course of IPF, you may notice shortness of breath when casually walking across the street. […] The hallmark symptom or main symptom of IPF is shortness of breath. It comes on very quietly or gradually, typically. It sneaks up on patients, so that they notice shortness of breath maybe when theyre walking up a slight incline that they once walked up without any difficulty.

• #10 7 Common Symptoms of Pulmonary Fibrosis | Pulmonary Fibrosis NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-social-clips/7-common-symptoms-of-pulmonary-fibrosis/

  Pulmonary fibrosis is a progressive chronic lung disease where the tissue in the lungs becomes thick and stiff, making it increasingly difficult to breathe and leading to a low level of oxygen in the blood. In the early stages of the disease, the symptoms can mimic many other illnesses which can make it difficult to diagnose, and often the symptoms are ignored until they become more severe. […] Shortness of breath (dyspnea) is the most commonly associated symptom of pulmonary fibrosis. It begins with shortness of breath during physical exertion but as the disease progresses, patients will find they suffer shortness of breath even while resting. […] A persistent cough is one of the most commonly reported symptoms of pulmonary fibrosis, but because coughing is associated with many different lung diseases, it is not automatically linked to PF. However, many other lung diseases have a cough which produces a lot of mucus, whereas PF patients experience a dry cough.

• #11 Early Symptoms | ILD Collaborative

  https://www.ildcollaborative.org/resources/ipf-patient-journey/early-symptoms

  Breathlessness and a nagging, dry cough can start years before a person is diagnosed with IPF. The study found that patients with IPF were telling their primary care doctors about their troubles with breathlessness and cough for up to five years before they were actually diagnosed with IPF. […] People with IPF breathe more deeply than healthy people do. Even at rest, a person with IPF will breathe in a bigger volume of air than a healthy person does. They breathe in more air to try to make up for the slow and difficult gas exchange. […] The combination of slow and difficult gas exchange and harder working muscles creates the feeling in the brain that we know as breathlessness. Breathlessness is a neurological response in the brain that happens when the brain senses that breathing is hard work, and even with all that hard work not enough oxygen is getting into the bloodstream.

• #12 Pulmonary Fibrosis – Symptoms | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/idiopathic-pulmonary-fibrosis/symptoms

  The symptoms of IPF develop over time. Symptoms may be different between people and may develop slowly or quickly. […] The most common symptoms are: […] Shortness of breath that gets worse over time. At first, you may be short of breath only during exercise. Over time, you may feel breathless even at rest. […] A dry cough that gets worse. You may have repeated bouts of coughing that you cannot control. […] Other symptoms may include: […] Aching muscles and joints […] Clubbing, which is a widening and rounding of the tips of the fingers or toes […] Extreme tiredness […] Gradual, unintended weight loss […] Generally feeling unwell […] Rapid, shallow breathing.

• #13 Pulmonary Fibrosis Symptoms | Temple Health

  https://www.templehealth.org/services/conditions/pulmonary-fibrosis/symptoms

  Other symptoms of pulmonary fibrosis include: Fatigue, Unexplained weight loss, Rapid, shallow breathing, Clubbing widening and rounding of the tips of the fingers or toes, Bluish discoloration of fingers or toes, caused by low blood oxygen levels, Swelling in the feet or legs. […] Pulmonary fibrosis symptoms tend to worsen over time, from mild to moderate and severe to very severe. As your condition progresses, you may develop other symptoms such as: Blue discoloration of the fingers or toes, Rapid, shallow breathing, Swelling in the feet and legs, Tiredness, Unexplained weight loss, Widening or rounding of the fingertips or toes, a condition called clubbing. […] Sometimes pulmonary fibrosis, including idiopathic pulmonary fibrosis, leads to other serious problems, such as: Blood clots in the lungs, Lung infections, Lung cancer. […] At its most severe, pulmonary fibrosis can also lead to conditions such as: Respiratory failure, Pulmonary hypertension, Heart failure.

• #14 What are the stages of pulmonary fibrosis? | Action for Pulmonary Fibrosis

  https://www.actionpf.org/information-support/pulmonary-fibrosis-stages

  youre experiencing unintended weight loss […] youve seen a change in your pattern of breathing (e.g. rapid, shallow breathing) […] there are any issues that seem to be triggered by your medication. […] Currently, there is no cure for pulmonary fibrosis. As the fibrosis progresses, the lungs can fail and it is likely you will eventually die from pulmonary fibrosis or a complication of it. […] My advice to patients with IPF, from my own experience, is don’t assume that any deterioration in your condition is normal and just the progression of the disease. Talk to your medical team.

• #14 What are the stages of pulmonary fibrosis? | Action for Pulmonary Fibrosis

  https://www.actionpf.org/information-support/pulmonary-fibrosis-stages

  There are no formally recognised stages of pulmonary fibrosis. Every person’s lung disease is different. For many people, their pulmonary fibrosis is likely to get worse over time. […] Your medical team will monitor your pulmonary fibrosis progression. They may describe your lung disease as mild, moderate or severe based upon your symptoms, lung function tests, six-minute walk test and amount of fibrosis present on CT chest scan. […] Make sure you tell your medical team if your symptoms seem to be getting worse. Look out for the following: […] youre becoming increasingly breathless, especially with mild physical activity or when you’re sitting or resting […] your cough is getting worse or has changed in nature (for example, a dry cough becomes productive of sputum) […] youre suffering from increased tiredness or generally feeling low

• #15 7 Common Symptoms of Pulmonary Fibrosis | Pulmonary Fibrosis NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-social-clips/7-common-symptoms-of-pulmonary-fibrosis/

  As the body’s oxygen levels begin to decline, this causes pain in the joints and muscles as they aren’t getting enough oxygen-rich blood. In addition, rheumatoid arthritis patients can develop pulmonary fibrosis due to the spread of inflammation, which can make joints and muscles hurt even more. […] Digital clubbing, or clubbing of the fingers or toes, occurs due to a lack of oxygen in the blood. It tends to appear in the latter stages of the disease so should be reported to a doctor as soon as possible. The tips of the fingers or toes become rounded and enlarged, resembling drumsticks.

• #16 7 Common Symptoms of Pulmonary Fibrosis | Pulmonary Fibrosis NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-social-clips/7-common-symptoms-of-pulmonary-fibrosis/

  Fatigue is much more than just feeling tired and cannot usually be remedied by a good night’s sleep. Fatigue is a common symptom in many chronic illnesses and can be seriously debilitating and affect the quality of life for those living with pulmonary fibrosis. […] Weight loss in pulmonary fibrosis patients tends to occur as the disease progresses. A general lack of appetite and feelings of nausea are often to blame which may be caused by the medications used to treat the disease, rather than the disease itself. Depression, which is common for people living with pulmonary fibrosis, can also lead to weight loss. […] Shallow or fast breathing (tachypnea) is a common symptom of pulmonary fibrosis and as the disease progresses, low oxygen levels in the blood can lead to difficulty in breathing and cause patients to breathe rapidly. Typically, a healthy person will take between 12 and 20 breaths per minutes, whereas someone with pulmonary fibrosis will take more than 20 breaths in the same time frame.

• #17 Pulmonary fibrosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/diagnosis-treatment/drc-20353695

  Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs. […] The lung scarring and thickening that occurs in pulmonary fibrosis cannot be repaired. And no current treatment has proved effective in stopping the disease from getting worse over time. Some treatments may improve symptoms for a time or slow how fast the disease worsens. Others may help improve quality of life. […] When symptoms suddenly get worse, called an acute exacerbation, you may need more supplemental oxygen. In some cases, you may need mechanical ventilation in the hospital. […] A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. But a lung transplant can involve complications such as rejection and infection.

• #18 Idiopathic Pulmonary Fibrosis – Pulmonary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pulmonary-disorders/interstitial-lung-diseases/idiopathic-pulmonary-fibrosis

  Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, causes progressive pulmonary fibrosis. Symptoms and signs develop over months to years and include exertional dyspnea, cough, and fine (Velcro) crackles. […] Symptoms and signs of idiopathic pulmonary fibrosis typically develop over 6 months to several years and include dyspnea on exertion and nonproductive cough. […] The classic sign of IPF is fine, dry, inspiratory crackles (Velcro crackles) at both bases. Clubbing is present in about 50% of cases. […] Most patients have moderate to advanced clinical disease at the time of diagnosis and deteriorate despite treatment. The mortality rate is estimated to be 50 to 70% at 5 years. […] Causes of acute deterioration include infections, pulmonary embolism, pneumothorax, and heart failure. Also, acute exacerbations without an identifiable cause may occur. All acute exacerbations have a high morbidity and mortality.

• #19 Pulmonary Fibrosis Progression and Exacerbation | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/living-well-with-pulmonary-fibrosis/progression-and-exacerbation

  During an acute exacerbation, many patients require hospitalization. If your exacerbation is severe, you may require ventilator support. This means that you are placed in a machine that acts as your lungs. Patients who are placed on a ventilator during an acute exacerbation are at an increased risk of dying.

• #20 Pulmonary fibrosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/symptoms-causes/syc-20353690

  In people with pulmonary fibrosis, especially idiopathic pulmonary fibrosis, shortness of breath can suddenly get worse over a few weeks or days. This is called an acute exacerbation. It can be life-threatening. The cause of an acute exacerbation may be another condition or an illness, such as a lung infection. But usually the cause is not known.

• #21 Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease | Thorax

  https://thorax.bmj.com/content/66/3/226

  In idiopathic pulmonary fibrosis (IPF) the distribution and spatial-temporal progression of fibrotic changes may be influenced by general or locoregional conditions. […] Patients with AIPF did not differ from controls in global fibrosis score and forced vital capacity, but carbon monoxide transfer factor was less decreased (5219% vs 4313%, p=0.009). […] The rate of gastro-oesophageal reflux and acute exacerbations was significantly higher in patients with AIPF (62.5% vs 31.3%, p=0.006 and 46.9% vs 17.2%, p=0.004, respectively). […] Although the global fibrosis score worsened significantly in all 23 patients with AIPF with serial high-resolution CT scans (p0.0001), pulmonary fibrosis remained asymmetrical in all except three. […] During follow-up, 15 patients with AIPF experienced 18 acute exacerbations. […] AIPF may be related to locoregional factors including gastro-oesophageal reflux which may be responsible for both disease expansion and the occurrence of acute exacerbations.

• #22 Prognosis of Pulmonary Fibrosis

  https://www.clinicbarcelona.org/en/assistance/diseases/pulmonary-fibrosis/progression-of-the-disease

  The worst prognosis is given when the idiopathic pulmonary fibrosis progresses towards respiratory failure in an average of approximately four years after the initial diagnosis. This progression of the disease has a mean survival that varies between two and five years. […] In any case, there is a wide variability in the course of the disease in each patient that will depend on factors like the appearance of acute exacerbations in the progression of the disease, or the presence of other diseases. […] During the long-term progression of these diseases, acute exacerbation episodes can occur, characterised by a rapid deterioration of the disease from at least one month of onset, or a respiratory deterioration that leads to significant mortality rates for the patients. […] Several studies have established possible risk factors for the development of acute exacerbations, like the maximum amount of air you can forcibly exhale from your lungs after fully inhaling (Forced Vital Capacity); the increased lung capacity (ILC); low lung diffusing capacity (DLCO); low distance covered in the walk test; deficient oxygenation; baseline level of dyspnoea increased, as well as having presented previous exacerbation episodes or have comorbidities such as pulmonary hypertension, emphysema, or coronary artery disease.

• #23 Stages of Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/stages-of-pulmonary-fibrosis

  Pulmonary fibrosis often gets worse over time. No one can predict how fast a patient’s PF will progress. In some people, PF progresses very quickly while others live with the disease for many years. […] Discussing symptoms with your doctor is key to determining the severity of your PF. You might be asked about how frequently you become breathless or how often you cough. Worsening breathlessness and cough don’t always mean the disease has progressed. But knowing your symptoms helps your doctor better understand your particular disease. […] A scoring system was recently developed called the GAP Model which uses Gender, Age and Physiology (and includes your FVC score and diffusing capacity) to stage PF. Not all physicians use this staging system. Science is always advancing so ask your doctor to explain your stage and the tests you may need.

• #24 How Quickly Does Pulmonary Fibrosis Progress? | PulmonaryFibrosisNow.org

  https://pulmonaryfibrosisnow.org/2019/05/22/how-quickly-does-pulmonary-fibrosis-progress/

  You have just been diagnosed with pulmonary fibrosis(PF), a family of more than 200 serious lung diseases characterized by scarring and thickening of the lung tissue. Undoubtedly, you’ll have many questions about this disease and how it will affect you and your family. Chief among those questions will be “How quickly will this disease progress?” and “What should I expect to happen?” […] Most people diagnosed with PF will experience a progression of the disease over time. In this context, “progression” means your lungs are developing more scar tissue and your symptoms are worsening. It is important to note that there is no typical rate of progression, and the rate will vary depending on a number of factors. […] The rate at which PF progresses can differ significantly from one person to the next. Some people may experience mild to moderate symptoms that worsen slowly over the course of several years; whereas, others may experience “acute exacerbation” in which their symptoms worsen quickly over the course of days or weeks. Still, others may experience extended periods of stability followed by periods of more rapid disease progression.

• #25 Idiopathic Pulmonary Fibrosis Symptoms | What to Look For

  https://www.nationaljewish.org/conditions/ipf/ipf-overview/symptoms

  Other symptoms include a dry, hacking cough and fatigue is a really under recognized symptom by the medical community. Its very common in patients with IPF. […] Other common symptoms include a dry, hacking cough and fatigue. Symptoms of IPF may develop and worsen gradually or, less commonly, they may also worsen rapidly. Instances of acute IPF worsening occur in about 10 percent of people. This is called an acute exacerbation of IPF. The cause of acute exacerbations is unknown, but lung infections may play a role. […] Idiopathic pulmonary fibrosis is typically a life-shortening condition. Studies have shown that the median survival among people with IPF is 3-5 years from the time of diagnosis. This means that, in those studies, by 3-5 years after diagnosis, 50 percent of people with IPF have died and 50 percent are alive.

• #26 Stages of Pulmonary Fibrosis | Temple Health

  https://www.templehealth.org/services/conditions/pulmonary-fibrosis/stages

  The most common pulmonary fibrosis symptoms are breathlessness and dry cough. But you may experience other symptoms such as tiredness, unexplained weight loss or swelling in the feet and legs. […] Understanding how severe your condition is helps doctors select a treatment plan tailored to your specific needs. […] While not a formal staging system, these levels of disease severity help your doctor monitor you for worsening of the disease over time.

• #27 Pulmonary Fibrosis Life Expectancy & Prognosis – Responsum Health

  https://responsumhealth.com/conditions/pulmonary-fibrosis/prognosis/

  Increased breathlessness, or dyspnea, has proven to be an important predictor of survival after accounting for disease severity and other disease factors. […] Declining lung function is linked to a worse prognosis and seen as a decrease in forced vital capacity (FVC), total lung capacity (TLC), and diffusing capacity of the lungs for carbon monoxide (DLCO). […] Greater fibrosis on a high-resolution computed tomography (HRCT) scan signals IPF progression, with a UIP pattern (usual interstitial pneumonia) generally linked to worse life expectancy.

• #28 Prognosis of Pulmonary Fibrosis

  https://www.clinicbarcelona.org/en/assistance/diseases/pulmonary-fibrosis/progression-of-the-disease

  The development of pulmonary hypertension causes a deterioration in the quality of life of the patients, higher exertion dyspnoea, reduction in the diffusing capacity for carbon monoxide (DLCO) in respiratory function tests, and less exercise capacity, which is demonstrated by a lower distance covered, and a lower desaturation (when the arterial oxygen saturation decreases by 4% or more) in the walking test. […] Furthermore, it is also associated with a lower survival with a mortality at one year of 28% in patients with pulmonary hypertension compared to 5.5% in those that do not have it. […] Several studies have documented a high prevalence (66-87%) of gastro-oesophageal reflux in patients with pulmonary fibrosis, which is asymptomatic in the majority of them. […] There is also a higher risk of developing lung cancer in patients with pulmonary fibrosis. The prevalence is from 5-10% and increases with the development of the disease in time and in patients with associated emphysema. […] Pneumothorax (collapsed lung) can also be a cause for the worsening of the pulmonary fibrosis, with an approximate incidence of 11%.

• #29 What are the stages of idiopathic pulmonary fibrosis?

  https://www.antidote.me/blog/what-are-the-stages-of-idiopathic-pulmonary-fibrosis

  The early symptoms of IPF can sometimes include fatigue, shortness of breath, and a distinctive, dry cough, but some people do not experience symptoms at all in this first phase. […] As IPF advances, it becomes more common for patients to experience coughing more frequently, in addition to shortness of breath with activity. […] In the third stage, patients will feel shortness of breath with activity and will experience low oxygen levels at rest. […] Eventually, a portable, lightweight oxygen delivery system will no longer be sufficient for a patients needs, and doctors will then recommend high-flow oxygen delivery.

• #30 How Quickly Does Pulmonary Fibrosis Progress? | PulmonaryFibrosisNow.org

  https://pulmonaryfibrosisnow.org/2019/05/22/how-quickly-does-pulmonary-fibrosis-progress/

  Life expectancy also varies among those suffering from PF. Different life expectancies may be associated with varying types of PF, so the proper diagnosis of the disease by a pulmonary specialist is critical to determining your prognosis. In general, however, most people with IPF rarely survive more than three to five years following their diagnosis. […] Like any serious disease, PF will affect you physically as well as emotionally. Having been diagnosed with PF by your doctor, you are already aware of some of its symptoms. These include shortness of breath, fatigue, hacking cough, painful joints and muscles and sometimes clubbing (rounding and widening) of your fingers and toes. As the disease progresses, you may experience a worsening of some or all of these symptoms. Pulmonary fibrosis may also lead to serious complications such as pulmonary hypertension, partial heart failure, lung infections, lung cancer and ultimately, respiratory failure.

• #31 End of life with pulmonary fibrosis | Action for Pulmonary Fibrosis

  https://www.actionpf.org/information-support/end-of-life

  Everybody is different but over time, your lung function is likely to drop and you may need oxygen to support your breathing. You may become a lot more fatigued and it will be harder to breathe as your pulmonary fibrosis progresses. […] As you lose more lung function you will probably want to spend more time in bed. Towards the end, you may be sleepy or unconscious much of the time. […] You may also lose interest in eating and drinking. Your breathing pattern may change and eventually, your skin may become pale and moist, and you will become very drowsy.

• #32 13 Pulmonary Fibrosis Stage 4 Symptoms (and Finding Relief)

  https://lunginstitute.com/pulmonary-fibrosis-stage-4-symptoms/

  Pulmonary fibrosis is a condition in which the tissue of the lungs becomes damaged and scarred. […] Stage 4 pulmonary fibrosis is very painful, often requiring high-flow oxygen. While this can be a difficult adjustment, it’s not a death sentence. […] Many symptoms accompany stage 4 pulmonary fibrosis, but there are ways to find relief from these symptoms. […] Chest pain is a common side effect of end stage pulmonary fibrosis. […] For patients with stage 4 pulmonary fibrosis, shifting from portable oxygen to high-flow oxygen can trigger or increase anxiety and depression. […] Along with increased depression, stage 4 pulmonary fibrosis can often lead to increased anxiety. […] A lack of appetite from pulmonary fibrosis can be caused by many things, such as medications, depression, or not feeling well.

• #33 13 Pulmonary Fibrosis Stage 4 Symptoms (and Finding Relief)

  https://lunginstitute.com/pulmonary-fibrosis-stage-4-symptoms/

  Coughing is a common symptom of end stage pulmonary fibrosis and can lead to discomfort and chest pain. […] For many patients, moving into stage 4 of pulmonary fibrosis means they will need high-flow oxygen. […] Pulmonary fibrosis’s characteristic damage and lung scarring often result in reduced lung function. […] Fluid retention, also called edema, is a side effect of your body not getting enough oxygen. […] Many of the symptoms of pulmonary fibrosis can disrupt your sleep. […] Many patients with stage 4 pulmonary fibrosis will need intensive home support. […] Patients with stage 4 pulmonary fibrosis symptoms may deal with increasing flare-ups and hospitalizations. […] As your pulmonary fibrosis worsens, so will your shortness of breath. […] Patients with pulmonary fibrosis often lose weight. […] Stage 4 pulmonary fibrosis can be a difficult diagnosis. While there are medications that can slow the progress of the disease, there is no cure. In some cases with severe stage 4 pulmonary fibrosis, patients may be eligible for a lung transplant, which can relieve many of the above symptoms.

• #34 End Stage Lung Disease – Newport Cardiac & Thoracic Surgery

  https://newportcts.com/thoracic-surgery/lung-cancer-surgery/end-stage-lung-disease/

  Pulmonary fibrosis results in scarring of the lung tissue that restricts the ability of the lung to fully expand with breathing decreasing lung function and leading to shortness of breath. […] As the lungs become more scarred, patients have worsening shortness of breath, decreased oxygen levels, and a chronic dry cough. […] The signs and symptoms depend on the underlying cause and the levels of oxygen and carbon dioxide in your bloodstream. COPD and emphysema can result in wheezing while patients with pulmonary fibrosis can have a chronic dry cough. […] Low levels of oxygen in your blood due to end-stage lung disease can cause shortness of breath (feeling like you cannot get enough air in your lungs) and fatigue eventually limiting walking and other physical activity. […] For patients with end-stage lung disease, lung transplant may be considered when other treatment options are no longer effective. […] While there is no specific cure for idiopathic pulmonary fibrosis, newer medications may be useful in mild idiopathic pulmonary fibrosis in preventing further scarring and decline in pulmonary function.

• #35 What are the signs of end stage pulmonary fibrosis? | PulmonaryFibrosisNow.org

  https://pulmonaryfibrosisnow.org/2019/11/27/what-are-the-signs-of-end-stage-pulmonary-fibrosis-2/

  No one wants to hear how bad it could get, yet everyone wants to know! Not only for their own sake, but also for their families and their caregivers. […] Most long-term lung conditions gradually worsen with time. As people reach the end stage of long-term lung disease, physical changes typically appear. However, the progression to the end is different for people. Some people progress slowly while others progress rapidly. It is not easy to predict when life will come to an end. […] Each person’s experience at their end of life varies. We must remember that the presence of one or more of these symptoms does not necessarily mean someone is close to death. They could have had any of these symptoms for months or even years before. Below, we discuss some common end-stage pulmonary fibrosis signs, and offer suggestions to help alleviate these uncomfortable symptoms.

• #36 Interstitial Lung Disease: Stages, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17809-interstitial-lung-disease

  Since there are many kinds of interstitial lung diseases, any one case could be mild or very serious. Most kinds of ILD cause irreversible lung damage. The most serious ILDs are progressive, where your condition continues to worsen over time. […] In severe cases of interstitial lung disease, you can have life-threatening complications, including: High blood pressure in your lungs (pulmonary hypertension). […] For people with the most severe and rapidly worsening forms of interstitial lung disease, life expectancy is around 3-5 years after diagnosis.

• #37 Complications of Idiopathic Pulmonary Fibrosis & Progression

  https://pulmonaryfibrosismd.com/complications-of-idiopathic-pulmonary-fibrosis/

  Worsening shortness of breath is a predictable feature of Idiopathic Pulmonary Fibrosis as the disease progresses. […] Hypoxia (low oxygen levels) is the rule not the exception as IPF advances. […] Cough is a vexing and common symptom of IPF. […] Flares or exacerbations are discreet episodes of increased respiratory symptoms that evolve over a few days to a couple of weeks. Patients note increased shortness of breath and often cough. Worsening oxygenation and declining pulmonary function tests are also seen. These episodes should be taken very seriously.

• #38 Pulmonary Fibrosis Causes, Symptoms, Stages & Life Expectancy

  https://www.medicinenet.com/pulmonary_fibrosis/article.htm

  The prognosis of pulmonary fibrosis is poor with patients typically surviving for less than 5 years. […] The progress of the disease varies from person to person, but often people diagnosed with idiopathic pulmonary fibrosis live about three to five years following their diagnosis. […] Signs and symptoms of pulmonary fibrosis include: Shortness of breath (dyspnea), including shortness of breath with everyday activities, Coughing (chronic, dry, hacking cough), Diminished exercise tolerance, Fast, shallow breathing, Fatigue/tiredness, Weakness, Chest discomfort, Muscle and joint aches, Loss of appetite, Unexplained/unintended weight loss, „Clubbing” of the tips of the fingers and toes (thickening of flesh under the nails, causing a widening and rounding appearance). […] Symptoms vary depending on the cause of pulmonary fibrosis. The severity of symptoms and the progression (worsening) of symptoms over time can vary.

• #39 Prognosis and monitoring of idiopathic pulmonary fibrosis – UpToDate

  https://www.uptodate.com/contents/prognosis-and-monitoring-of-idiopathic-pulmonary-fibrosis

  Prognosis and monitoring of idiopathic pulmonary fibrosis […] The natural history of IPF is most often described as one of insidious decline in lung function resulting in progression to respiratory failure and death on average within approximately four to five years after the initial diagnosis. […] However, there is great variability in disease course among individual patients with IPF, and survival is influenced by several factors including variable progression rates, occurrence of acute exacerbations, and comorbid disease. […] Mortality — The median survival of IPF has been reported to range from two to five years. This estimate reflects the range of average life expectancies observed in cohorts of IPF patients, rather than the limits of an individual patient’s life expectancy. This nuance is important, as the actual range of survival of individual IPF patients is quite broad, with up to 20 to 25 percent of patients living beyond 10 years.

• #40 Pulmonary Fibrosis: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis

  No, there isnt a cure for pulmonary fibrosis. Treatment focuses on easing symptoms, slowing down progression, and improving your quality of life. […] Talk to a healthcare provider if you have shortness of breath or get tired more easily with everyday tasks than you used to. Early diagnosis and treatment can help slow down the progression of lung disease. […] Lung scarring is nearly always permanent (unless caused by a medication and caught early). If you have an underlying disease, management of it might help prevent further damage. […] Healthcare providers cant easily predict how pulmonary fibrosis will progress. Your symptoms may get worse very slowly, over years. In some cases, the disease may lead to severe symptoms quickly (over months). […] The life expectancy of someone with the most common form, idiopathic pulmonary fibrosis, is three to five years. But life expectancies for people with PF have been getting longer in recent years.

• #41 What Are the Stages of Pulmonary Fibrosis? – Responsum Health

  https://responsumhealth.com/conditions/pulmonary-fibrosis/stages/

  Pulmonary fibrosis (PF) is a chronic disease that progresses, or worsens, over time though how quickly or slowly it progresses for each individual varies. […] Common symptoms of pulmonary fibrosis include shortness of breath (dyspnea) and a dry cough. Other symptoms include achy muscles and joints, fatigue, unexplained weight loss, and clubbing (widening and rounding) of the tips of your fingers and/or toes. […] Early detection plays a large role in a patients prognosis. The sooner PF or IPF is diagnosed, the better ones chance of survival. Disease severity at the time of diagnosis is directly related to ones life expectancy. […] While there is no cure for pulmonary fibrosis, two anti-fibrotic drugs, or anti-scarring treatments, have been approved by the U.S. Food and Drug Administration (FDA) and have clinically proven to slow PF progression. […] Lung function may decline faster in some patients than others. While many live past three to five years, others experience respiratory failure before the three-year mark, and some become very ill within months.

• #42 Pulmonary Fibrosis Life Expectancy & Prognosis – Responsum Health

  https://responsumhealth.com/conditions/pulmonary-fibrosis/prognosis/

  Upon diagnosis, pulmonary fibrosis life expectancy can vary depending on many different factors. All types of PF involve progressive scarring and inflammation. As scarring builds, so does breathlessness, and lung failure can eventually develop. The average life expectancy of pulmonary fibrosis is said to be three to five years after diagnosis, but those figures are now outdated. […] Disease progression can be impacted, both positively and negatively, by a number of different factors. […] Early detection plays a large role in a patients prognosis. The sooner PF or IPF is diagnosed, the better ones chance of survival. Disease severity at the time of diagnosis is directly related to ones life expectancy. […] While there is no cure for pulmonary fibrosis, two anti-fibrotic drugs, or anti-scarring treatments, have been approved by the U.S. Food and Drug Administration (FDA) and have clinically proven to slow PF progression.

• #43 Progressive Pulmonary Fibrosis | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/nurses/understanding-PF/PPF

  Patients with a range of ILDs may develop progressive pulmonary fibrosis. […] Patients with progressive fibrosing ILD may experience irreversible lung function decline, reduced health-related quality of life, and early mortality. […] Patients should be made aware that regularly monitoring of their symptoms can help discussions with their doctor about whether their pulmonary fibrosis is worsening. […] Changes in the symptoms your patients experience, including dry cough, dyspnea, fatigue, and weight loss may indicate that their ILD has become progressive. […] Patients should be made aware that regular check-ups with their doctor are important to detect worsening of their disease as soon as possible. […] Pulmonary fibrosis is unpredictable and regular doctor visits can enable detection of progressive disease; patients should expect further testing including lung imaging, lung function tests, as well as monitoring of their symptoms. […] Early detection of PPF with regular tests can help doctors adjust treatment, recommend lifestyle changes and provide additional support.

• #44

  https://www.nhs.uk/conditions/idiopathic-pulmonary-fibrosis/treatment/

  There’s currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve the symptoms as much as possible and slow down its progression. […] IPF can cause levels of oxygen in your blood to fall, which can make you feel more breathless. […] Pulmonary rehabilitation is used for many long-term lung conditions. […] A lung transplant can significantly improve life expectancy in people with IPF, although it’s a major procedure that puts a great strain on the body.

• #45 Pulmonary Fibrosis: What to Know About Symptoms and Treatment

  https://www.alto.com/blog/post/pulmonary-fibrosis-symptoms-and-treatment

  Pulmonary fibrosis is a progressive, long-term lung disease in which the lungs become scarred, making it difficult to breathe. […] As the disease progresses, you may experience shortness of breath more frequently during everyday activities. […] While the severity of pulmonary fibrosis symptoms vary from one person to another, there are some common symptoms: Shortness of breath, A dry cough, Fatigue, Unexplained weight loss, Aching muscles and joints, Widening and rounding of the tips of the fingers or toes. […] Some people with pulmonary fibrosis experience acute flare-ups in which symptoms such as shortness of breath intensify for several days up to several weeks. […] There are two primary medications that slow the progression of lung scarring and preserve lung function: pirfenidone (Esbriet) and nintedanib (OFEV). Both medications are similarly effective at treating symptoms and improving lung capacity.

• #46 Pulmonary Fibrosis: What to Know About Symptoms and Treatment

  https://www.alto.com/blog/post/pulmonary-fibrosis-symptoms-and-treatment

  Oxygen therapy transports more oxygen into your lungs, increasing your tolerance for physical activity and helping you stay active. […] Pulmonary rehabilitation is a program of education and exercise classes, typically in a group setting, aimed at improving lung function and reducing symptoms. […] A lung transplant, in which a damaged lung is replaced with a healthy lung from a donor, is a treatment option for some individuals with pulmonary fibrosis.

Zobacz więcej