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. […] The scarring that happens in pulmonary fibrosis can be caused by many things. […] Damage to the lungs that results in pulmonary fibrosis may be caused by many different things. Examples include long-term exposure to certain toxins, radiation therapy, some medicines and certain medical conditions. In some cases, the cause of pulmonary fibrosis is not known. […] Many substances and conditions can lead to pulmonary fibrosis. Even so, in many people, the cause is never found. But risk factors such as smoking or exposure to air pollution could be related to the condition, even if the cause cannot be confirmed. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.

• #2 Lung Scarring: Types, Causes, Symptoms, and Treatment Options

  https://www.verywellhealth.com/pulmonary-fibrosis-types-symptoms-diagnosis-6891013

  Lung scarring, called pulmonary fibrosis, is a serious, permanent condition, but treatments are available. […] 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. […] Its normal for the lungs to become damaged from environmental toxins, medications, lung disease, or illnesses, including COVID-19. […] However, in the case of more serious damage, scar tissue forms. […] 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.

• #3

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

  It can be challenging for doctors to figure out what causes PF. […] When the cause of the PF is unknown, it is called idiopathic. […] There are five main categories of pulmonary fibrosis that have identifiable causes. They are: Drug-induced, Radiation-induced, Environmental, Autoimmune, and Occupational. […] In the United States, Environmental and Autoimmune causes seem to be the most common types of PF of known cause. […] Some medications can cause PF. […] Radiation to the chest for lymphoma; Hodgkins disease; or breast, lung, and other cancers; can also injure the lung and cause fibrosis. […] Inhaled environmental substances can cause a form of PF called hypersensitivity pneumonitis (HP) or chronic hypersensitivity pneumonitis. […] Substances found in occupational settings can cause a form of PF called pneumoconiosis.

• #4 Types, Causes and Risk Factors of Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/types-causes-and-risk-factors

  Pulmonary fibrosis (PF) is a form of interstitial lung disease that causes scarring in the lungs. There are over 200 different types of PF and in most cases, there’s no known cause. […] The most common type of PF is IPF, which stands for idiopathic pulmonary fibrosis. This means this type of PF has no known cause. […] Some cases of PF are caused by autoimmune diseases like rheumatoid arthritis, scleroderma or Sjogren’s syndrome. Certain viral infections and gastroesophageal reflux disease (GERD) are also risk factors for PF. […] PF can be caused by exposure to hazardous materials. Examples include occupational exposures such as asbestos or silica. Some cases of PF are caused by breathing in bird or animal droppings. Radiation treatments and certain types of medications can cause PF. Cigarette smoking also increases a person’s risk of developing PF.

• #5

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

  It can be challenging for doctors to figure out what causes PF. […] When the cause of the PF is unknown, it is called idiopathic. […] There are five main categories of pulmonary fibrosis that have identifiable causes. They are: Drug-induced, Radiation-induced, Environmental, Autoimmune, and Occupational. […] In the United States, Environmental and Autoimmune causes seem to be the most common types of PF of known cause. […] Some medications can cause PF. […] Radiation to the chest for lymphoma; Hodgkins disease; or breast, lung, and other cancers; can also injure the lung and cause fibrosis. […] Inhaled environmental substances can cause a form of PF called hypersensitivity pneumonitis (HP) or chronic hypersensitivity pneumonitis. […] Substances found in occupational settings can cause a form of PF called pneumoconiosis.

• #6 CAUSES OF PULMONARY FIBROSIS | Madeksho Law

  https://www.madeksholaw.com/post/causes-of-pulmonary-fibrosis

  This is called familial pulmonary fibrosis (FPF) or familial interstitial pneumonia (FIP). […] A number of genes and genetic deviations have been identified that are associated with PF, but genetic tests are seldom performed when PF is diagnosed, thus we are only just beginning to understand the importance of these genetic abnormalities. […] Pulmonary fibrosis can develop after high exposure to a wide range of inorganic dusts, such as asbestos, silica, coal dust, beryllium, and hard metal dusts. […] Another cause of some types of PF is organic dusts, such as animal proteins, bacteria, and molds. […] Diseases caused by inhaled organic dusts are often called hypersensitivity pneumonitis. […] Exposure to radiation can also contribute to the risk of PF. […] Some medications have been linked to the development of PF, including drugs used to treat infections (nitrofurantoin, sulfasalazine), heart disease (amiodarone, propranolol), seizures (phenytoin), and cancer (methotrexate, bleomycin, oxaliplatin, radiation therapy).

• #7 Lung Scarring: Types, Causes, Symptoms, and Treatment Options

  https://www.verywellhealth.com/pulmonary-fibrosis-types-symptoms-diagnosis-6891013

  The causes of interstitial lung disease are mostly unknown and, hence, difficult to prevent. […] 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. […] Occupational lung diseases are illnesses caused by exposure to hazardous materials at work. […] Lung scarring is one of the potential long-term side effects of COVID-19 infection. […] Autoimmune diseases, including arthritis, sarcoidosis, and systemic sclerosis, can all cause lung scarring. […] Certain 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, and nitrofurantoin, an antibiotic used to treat urinary tract infections.

• #8 Pulmonary fibrosis – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_fibrosis

  Some typical connective tissue diseases such as rheumatoid arthritis, ankylosing spondylitis, SLE and scleroderma. […] Certain medications, e.g. amiodarone, bleomycin (pingyangmycin), busulfan, apomorphine, and nitrofurantoin. […] Hypersensitivity pneumonitis, most often resulting from inhaling dust contaminated with bacterial, fungal, or animal products.

• #8 Pulmonary fibrosis – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_fibrosis

  Pulmonary fibrosis may be a secondary effect of other diseases. Most of these are classified as interstitial lung diseases. Examples include autoimmune disorders, viral infections, and bacterial infections such as tuberculosis that may cause fibrotic changes in the lungs’ upper or lower lobes and other microscopic lung injuries. But pulmonary fibrosis can also appear without any known cause. In that case, it is termed „idiopathic”. […] Causes include environmental pollution, certain medications, connective tissue diseases, infections, and interstitial lung diseases. […] Diseases and conditions that may cause pulmonary fibrosis as a secondary effect include: Inhalation of environmental and occupational pollutants, such as metals in asbestosis, silicosis, and exposure to certain gases. […] Cigarette smoking can increase the risk or make the illness worse. Smoking is a known cause of some types of lung fibrosis, such as smoking-related interstitial fibrosis (SRIF).

• #9 Idiopathic Pulmonary Fibrosis Causes | NSIP Lung | PF NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-causes/

  Certain antibiotics and anti-inflammatory medications, like nitrofurantoin and sulfasalazine, also can cause PF. Medications for heart conditions, like amiodarone, also can trigger the onset of the disease, as can the anti-seizure medication phenytoin. […] While a person’s genetic makeup typically does not cause PF outright, it may make an individual more susceptible to the disease. […] For example, mutations in the genes TERC and TERT are tied to an increased risk of IPF and other types of PF. These genes provide instructions for making components of telomerase, an enzyme that maintains telomeres — structures found at the end of chromosomes to stabilize and protect DNA. It is thought that lower telomerase function, as a result of these mutations, could cause telomeres to become abnormally shorter, which in turn may cause lung cells to stop dividing or die prematurely.

• #9 Idiopathic Pulmonary Fibrosis Causes | NSIP Lung | PF NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-causes/

  PF also can be caused by viral infections, such as COVID-19, which can damage the lungs and cause scar tissue to develop. Certain muscle diseases, including polymyositis and anti-synthetase syndrome, also can cause PF, typically as a consequence of muscle inflammation (myositis) in the muscles found around the lungs that are required for breathing. […] Occupational and environmental exposure to certain toxic substances can cause irritation and damage to the lungs and lead to scar tissue formation. Some of these toxic substances include asbestos fibers, silica dust, coal dust, and hard metal dusts. Other irritants, such as cigarette smoke or animal droppings, also may result in the onset of PF. […] Several medications and treatments used in clinical practice can cause PF as a side effect. For example, people who undergo certain forms of radiation therapy may develop PF, depending on how much their lungs were exposed to radiation and the total amount of radiation used. Similarly, some forms of chemotherapy that are designed to kill cancer cells can cause damage to lung tissue, leading to the onset of PF.

• #10 What is drug-induced pulmonary fibrosis? | Action for Pulmonary Fibrosis

  https://www.actionpf.org/information-support/what-is-drug-induced-pulmonary-fibrosis

  Certain medications can cause inflammation and scarring or fibrosis of the lungs. This usually affects older people. […] Drugs that can cause pulmonary fibrosis include: the antibiotic nitrofurantoin, anti-cancer drugs such as bleomycin, cardiac medications such as amiodarone and biological therapies used for treating cancer and autoimmune diseases. […] Drug-induced pulmonary fibrosis can be an idiosyncratic reaction. This means it may occur quickly after starting the drugs and is not associated with the dose of treatment. […] If drug-induced pulmonary fibrosis is diagnosed quickly and the drugs stopped, most people make a good recovery. […] But if it is not identified early, drug-induced pulmonary fibrosis can progress and be life-limiting. The prognosis depends on the drug, the extent of pulmonary fibrosis and the overall health of the patient.

• #11 Causes of Pulmonary Fibrosis

  https://www.clinicbarcelona.org/en/assistance/diseases/pulmonary-fibrosis/causes-and-risk-factors

  The mechanisms that trigger these diseases are not the same as in the development of each one of them. […] Diffuse interstitial lung diseases (DILD) can occur without a known cause, which is called idiopathic, or due to other causes such as, for example: […] In autoimmune diseases like rheumatoid arthritis, lupus, vasculitis, scleroderma the immune system attacks its own respiratory system. […] Inhalation of foreign substances by the body, like certain types of dust, fungi, or contact with birds, which produce inflammation of the lungs that finally ends up as fibrosis (pneumonitis due to chronic hypersensitivity). […] Drugs, like sulphamides, nitrofurantoin, amiodarone, methotrexate, some monoclonal antibodies, or some types of chemotherapy, like bleomycin, which can initially inflame the lung (drug-induced-pneumonitis).

• #11 Causes of Pulmonary Fibrosis

  https://www.clinicbarcelona.org/en/assistance/diseases/pulmonary-fibrosis/causes-and-risk-factors

  Radiotherapy, in some areas of the chest. […] Occupational exposure, (to work exposed to asbestos, coal dust, cotton dust, wood dust, silica dust, dust from hard metals, etc.), that may lead to inflammation of the lung and that, after a prolonged time, can lead to lung fibrosis. […] Furthermore, it should be taken into account that smoking increases the risk of developing any diffuse interstitial lung diseases and could worsen the prognosis of the disease. […] Some factors that increase the risk of developing a fibrosing interstitial lung idiopathic diseases are: […] Around 5% of IPF can be familial. […] Some fibrosing DILD are more common in smokers, such as IPF, or the combination of pulmonary fibrosis-emphysema. […] Some fibrosing DILD associated with autoimmune diseases are more common in women between 30-40 years-old. On the other hand, the majority of patients with IPF are men over 55 years-old. […] The duration of any exposures should be taken into account, and whether breathing protection has been used (masks). […] There are many types of drugs capable of triggering a fibrosing DILD. […] Some autoimmune diseases predispose the development of some fibrosing DILDs.

• #12 Pulmonary Fibrosis: Symptoms, Causes and Treatment | MedPark Hospital

  https://www.medparkhospital.com/en-US/disease-and-treatment/pulmonary-fibrosis

  Pulmonary fibrosis is a respiratory disorder with scarring of lung tissues between the air sacs (alveoli). Various factors can trigger lung damage, including prolonged exposure to specific toxins, certain medical conditions, radiation therapy, and certain medications. […] Long-term exposure to toxins and pollutants such as coal dust, grain dust, asbestos fibers, silica dust, and animal droppings in the environment or workplace. […] Lung cancer or breast cancer radiotherapy. The extent of the harm depends on how much of your lungs received the radiation, the dosage of the radiation, and whether there is concurrent chemotherapy. […] Chemotherapy drugs, such as bleomycin, methotrexate, and cyclophosphamide. […] Heart arrhythmia medication such as amiodarone. […] Antibiotics such as nitrofurantoin.

• #12 Pulmonary Fibrosis: Symptoms, Causes and Treatment | MedPark Hospital

  https://www.medparkhospital.com/en-US/disease-and-treatment/pulmonary-fibrosis

  Anti-inflammatory medicines, such as rituximab or sulfasalazine. […] Medical conditions, such as dermatomyositis, mixed connective tissue disease, rheumatoid arthritis, pneumonia, polymyositis, sarcoidosis, scleroderma, and systemic lupus erythematosus. […] Certain types of pulmonary fibrosis, called idiopathic pulmonary fibrosis, usually affect seniors, about 70 to 75 years old, and may run in the family; the causes are not clear. According to research, GERD may also be a factor in the progression of pulmonary fibrosis, but more study about its association is required.

• #13 6 Pulmonary Fibrosis Causes and Risk Factors | Pulmonary Fibrosis NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-social-clips/6-pulmonary-fibrosis-causes-risk-factors/

  Both people who smoke and those who have quit are more at risk of developing PF than those who have never smoked. People with lung diseases associated with smoking such as COPD also have an elevated risk. […] Radiation therapy for breast cancer or lung cancer can increase the risk of pulmonary fibrosis. The risk will rise depending on how much radiation therapy the chest area received during cancer treatment and may be further increased if the patient also had chemotherapy. […] Some medications have been found to increase the risk of developing pulmonary fibrosis, namely chemotherapy medications, heart medications, some anti-inflammatory drugs and some antibiotics. […] Idiopathic pulmonary fibrosis (IPF) is the term given to the disease when doctors cannot attribute a single cause to the development of the disease. However, being male, over the age of 50, a smoker or ex-smoker and having a family history of the disease are all risks. Up to 20 percent of IPF patients share the disease with another close family member. […] In addition, it’s thought that patients who suffer from gastroesophageal reflux disease (GERD) may have an increased risk of PH due to inhaling some of the stomach acid that comes up the esophagus causing damage to the lung tissue.

• #13 6 Pulmonary Fibrosis Causes and Risk Factors | Pulmonary Fibrosis NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-social-clips/6-pulmonary-fibrosis-causes-risk-factors/

  Pulmonary fibrosis is a lung disease where the tissue which surrounds the air sacs (called alveoli) become scarred, making it difficult for oxygen to get into the bloodstream. Since there are various causes and risk factors for pulmonary fibrosis, we’ve compiled a list of some of the main causes and risks of the condition using information from the The Mayo Clinic and the Lung Association of Canada. […] Those who work in occupations where they might inhale various toxic dust, powders or chemicals will be more at risk of developing pulmonary fibrosis. Some of these include silica fibers, bird or animal droppings, asbestos fibers, coal dust, heavy metal dust, and grain dust. […] Certain medical conditions can leave patients vulnerable to developing PF, these include connective tissue diseases such as rheumatoid arthritis, lupus, and scleroderma.

• #14 GERD & Other Pulmonary Fibrosis Causes You Need to Know

  https://blog.uvahealth.com/2023/05/11/pulmonary-fibrosis-causes/

  Pulmonary fibrosis is more common in men but on the rise in women. Know the causes. […] Turns out there are lots of pulmonary fibrosis causes. GERD falls into a maybe category. […] The cause can range from environmental exposures such as mold or having birds indoors to autoimmune diseases like scleroderma or rheumatoid arthritis. Or ILD can be idiopathic where we don’t know the cause. […] In some cases, we cant find a cause. We call this idiopathic pulmonary fibrosis. But, in addition to GERD, its important to know about things that can inflame and scar your lungs. […] Many toxins and pollutants can damage your lungs over the long run. […] Some people who receive radiation therapy for lung or breast cancer may show signs of lung damage months or sometimes years after the first treatment.

• #14 GERD & Other Pulmonary Fibrosis Causes You Need to Know

  https://blog.uvahealth.com/2023/05/11/pulmonary-fibrosis-causes/

  Many drugs can damage your lungs, especially any of these medications you might need to take: […] Many medical conditions can damage the lungs. […] Paul sees a lot of pulmonary fibrosis patients with GERD. Experts are looking to see if pulmonary fibrosis changes a persons anatomy, making them more likely to have GERD. […] Its important to control GERD when present and symptomatic in pulmonary fibrosis patients, Paul says. […] No matter the pulmonary fibrosis cause, ILD is challenging to diagnose and manage, Paul notes.

• #15

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

  In people with IPF, the tiny air sacs in the lungs (alveoli) become damaged and increasingly scarred. […] The reason this happens is not clear. Idiopathic means the cause is unknown. […] IPF has been linked to: exposure to certain types of dust, such as metal or wood dust, viral infections, a family history of IPF around 1 in 20 people with IPF has another family member with the condition, acid reflux, smoking. […] But it’s not known whether some of these factors directly cause IPF.

• #16 What Is Pulmonary Fibrosis?

  https://www.webmd.com/lung/pulmonary-fibrosis-symptoms-causes-and-treatments

  Your doctor will call it idiopathic pulmonary fibrosis, or IPF, if theyre not sure why it happened. […] It can be hard to figure out the reason for your pulmonary fibrosis. The possible causes scientists do know about include: […] Autoimmune diseases. Your doctor might call these rheumatologic or connective tissue diseases. Autoimmune conditions that may lead to pulmonary fibrosis include: […] Environmental causes. These might be natural substances like mold spores, bacteria, or even dust from animal poop, especially birds. […] Tobacco smoke is another possible environmental cause of lung fibrosis.

• #17 Pulmonary fibrosis – treatment, causes and symptoms | healthdirect

  https://www.healthdirect.gov.au/pulmonary-fibrosis

  Pulmonary fibrosis is a lung disease where the tissue around the air sacs in the lungs becomes damaged. […] Most of the time, the cause of pulmonary fibrosis is not known. […] In some people, a cause can be found. Some causes include: certain medicines, having radiation treatment in the past, breathing in harmful dust or chemicals at a workplace or in the environment, like asbestos, silica, metal dusts, and coal dust, having an autoimmune disease like rheumatoid arthritis or a connective tissue disease, having certain inflammatory conditions, such as sarcoidosis. […] You are more at risk than others of developing pulmonary fibrosis if you: are over 60 years old, smoke or used to smoke, work or live in an environment where you breathe in smoke or dust from wood, metal, stone, coal or sand, have family members who have pulmonary fibrosis.

• #18 Idiopathic Pulmonary Fibrosis (Lung Hardening): What It Is, Symptoms, and Treatment | Anadolu Sağlık Merkezi

  https://www.anadolumedicalcenter.com/health-guide/idiopathic-pulmonary-fibrosis-lung-hardening-what-it-is-symptoms-and-treatment

  Idiopathic pulmonary fibrosis (IPF) is a disease characterized by thickening and hardening of the lungs with an unknown cause. […] Idiopathic pulmonary fibrosis causes damage and thickening of the tissue between the alveoli, the air sacs in the lungs. […] Idiopathic pulmonary fibrosis is a disease with an unknown cause that is rarely seen. […] The causes mentioned above, along with many other unlisted factors, can contribute to the development of idiopathic pulmonary fibrosis. Additionally, viruses and microbial diseases can also be considered as potential causes. Even in the absence of smoking, being in an environment where smoking occurs can trigger IPF.

• #19

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

  Autoimmune diseases are also called connective tissue diseases, collagen vascular diseases, or rheumatologic diseases. […] Examples of autoimmune diseases that can cause PF include: Rheumatoid arthritis; Scleroderma (also called systemic sclerosis); Sjgrens syndrome; and Polymyositis, dermatomyositis, and antisynthetase syndrome. […] Idiopathic pulmonary fibrosis (IPF) is scarring of the lung where the cause is not known. […] Although IPF is considered to be a disease of unknown cause, we do know some factors that increase the risk of getting IPF, including aging (IPF is rare before age 50), cigarette smoking, and having certain genetic predispositions. […] There have been several genes identified that appear to increase the risk of developing PF when an abnormal form of the gene is inherited.

• #20 Pulmonary Fibrosis, including Idiopathic (IPF): Symptoms, Causes and Stages

  https://patient.info/signs-symptoms/breathlessness-and-breathing-difficulties-dyspnoea/pulmonary-fibrosis

  Pulmonary fibrosis is a serious lung disease where the tiny air sacs of the lungs (the alveoli) and the lung tissue next to the alveoli become damaged and scarred, resulting in lung fibrosis. The cause is often not known but pulmonary fibrosis can be caused by a variety of underlying conditions. […] The main causes of pulmonary fibrosis are: […] Unknown (called idiopathic pulmonary fibrosis, or IPF) – see below for more information. […] Hypersensitivity pneumonitis: This happens when something you breathe into your lungs causes a reaction, which causes inflammation. Over a period of time this gradually causes scarring (fibrosis). […] Occupational interstitial lung disease (pneumoconiosis): These lung diseases are caused by breathing in certain dusts when at work. […] Pulmonary fibrosis associated with autoimmune diseases: An autoimmune disease means that your immune system, which normally attacks germs (bacteria, viruses, etc), attacks part of your body.

• #20 Pulmonary Fibrosis, including Idiopathic (IPF): Symptoms, Causes and Stages

  https://patient.info/signs-symptoms/breathlessness-and-breathing-difficulties-dyspnoea/pulmonary-fibrosis

  Sarcoidosis often affects the lungs. It can sometimes cause pulmonary fibrosis. […] Certain medicines may occasionally cause pulmonary fibrosis as a side-effect. […] The current thinking is that somehow the cells that line the alveoli are damaged in some way. The cells then try to heal themselves. But, this healing process becomes out of control, causing thickening and damage to the walls of the alveoli, and scarring of the alveoli and lung tissue. […] Various things have been suggested as potential causes, or triggers, of the initial damage to the cells lining the alveoli. These include: Cigarette smoking is the main risk factor.

• #21 Pulmonary Fibrosis: Definition and Patient Education

  https://www.healthline.com/health/pulmonary-fibrosis

  Researchers currently believe that a combination of exposure to lung irritants like certain chemicals, smoking, and infections, along with genetics and immune system activity, play key roles in pulmonary fibrosis. […] It was once thought that the condition was caused by inflammation. Now scientists believe that there is an abnormal healing process in the lungs that leads to scarring. The formation of significant lung scarring eventually becomes pulmonary fibrosis. […] The causes of pulmonary fibrosis can be divided into several categories: autoimmune diseases, infections, environmental exposure, medications, idiopathic (unknown), and genetics. […] Autoimmune diseases cause your body’s immune system to attack itself. Autoimmune conditions that can lead to pulmonary fibrosis include rheumatoid arthritis, lupus erythematosus, scleroderma, polymyositis, dermatomyositis, and vasculitis.

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

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

  The treatment options for idiopathic pulmonary fibrosis are very limited. There is no evidence that any medications can help this condition since scarring is permanent once it has developed. […] The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. […] Idiopathic pulmonary fibrosis has an increased frequency in cigarette smokers. The cause of idiopathic pulmonary fibrosis is unknown, and therefore prevention is difficult.

• #23 Pulmonary Fibrosis | University of Michigan Health

  https://www.uofmhealth.org/conditions-treatments/pulmonary/pulmonary-fibrosis

  Pulmonary fibrosis may result from a variety of injuries to the lung. Some of these injuries include occupational and environmental exposures, drugs, poisons and certain connective tissue diseases. […] In most cases, there is no known cause and when a cause cant be determined, the disease is called idiopathic pulmonary fibrosis.

• #24 Environmental Causes of Idiopathic Pulmonary Fibrosis

  https://www.mdpi.com/1422-0067/24/22/16481

  Idiopathic pulmonary fibrosis (IPF), the most common and severe of the idiopathic interstitial pneumonias, is a chronic and relentlessly progressive disease, which occurs mostly in middle-aged and elderly males. […] Several occupational and environmental exposures, including metal dust, wood dust and air pollution, as well as various lifestyle variables, including smoking and diet, have also been associated with an increased risk of IPF, probably through interaction with genetic factors. […] Most research in this field has primarily concentrated on treatments and the influence of specific comorbidities. Conversely, less emphasis has been placed on investigating the consequences of potentially preventable and avoidable environmental and occupational hazards as potential causes of IPF. […] Despite being a diagnosis of exclusion, the risk of developing IPF increases in those occupationally exposed to vapors, dusts, gases, and fumes (VGDF), as demonstrated by multiple retrospective case–control studies of IPF registries.

• #24 Environmental Causes of Idiopathic Pulmonary Fibrosis

  https://www.mdpi.com/1422-0067/24/22/16481

  Organic dust, notably agricultural and wood dust, has been found to be associated with an increased risk of IPF based on multiple meta-analyses. […] Studies utilizing mineralogical analysis techniques have found metal dusts (e.g., nickel, iron, and aluminum) as well as silica in the lymph node and lung tissue of patients with pulmonary fibrosis. […] The recent literature on inhalational injuries from conflict-related disasters has focused on the first responders in the World Trade Center (WTC) attacks. […] Exposure to many of these inorganic and organic materials, specifically silica, asbestos fibers, heavy metals, and wood dust, have been associated with an increased risk of diffuse interstitial changes, such as those seen in IPF. […] A multitude of occupational/environmental exposures, including, among others, respirable dust exposure in mining, construction, and manufacturing, has been associated with an increased risk of IPF, particularly among racial and ethnic minority populations and in low–middle income countries wherein safety standards may be suboptimal.

• #25 Pulmonary Fibrosis | Saint Luke’s Health System

  https://www.saintlukeskc.org/health-library/pulmonary-fibrosis

  Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease. […] Most of the time, healthcare providers dont know what causes lung tissue to become thick and stiff and form scarring in the lungs. Things that can increase your risk of getting IPF are: […] Being older (risk increases after age 50) […] Smoking […] Being male […] Certain viral or bacterial infections […] Long periods of exposure to asbestos fibers, pollution, silica, grain or metal dusts, bacteria, radiation, bird droppings, and gases […] Certain cancer treatments […] Certain types of heart medicines or antibiotics […] Family history (genetics). More than one family member may have pulmonary fibrosis. […] Gastroesophageal reflux disease (GERD). Researchers are trying to find the link between GERD and IPF.

• #26 What causes IPF? | Asthma + Lung UK

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

  Idiopathic means the cause is not known, but researchers now believe that the body creates fibrosis (scarring) in response to an injury to the lung. […] The initial injury to the lungs might be from: […] acid reflux from the stomach […] viruses – in some studies, IPF has been linked to certain viruses, including herpes, hepatitis C and the Epstein Barr virus, which causes glandular fever […] environmental factors – breathing in certain kinds of dust from wood, metal, textiles or stone, or from cattle or farming […] cigarette smoke exposure. […] It is not known if these factors directly cause IPF. […] Some people may also get IPF if its in their family history. But this link is only found in a minority of cases. According to a 2021 survey by the British Thoracic Society, 5.7% of patients were found to have a known relative with IPF.

• #27 Idiopathic pulmonary fibrosis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-pulmonary-fibrosis?lang=us

  Idiopathic pulmonary fibrosis (IPF) is a clinical syndrome and considered the most common and the most lethal form of pulmonary fibrosis corresponding to the histologic and imaging pattern of usual interstitial pneumonia. […] It is more common in elderly men and diagnosed by: […] absence of alternative causes such as drug toxicity, environmental exposure (e.g. asbestos) or collagen vascular disease (e.g. scleroderma, rheumatoid arthritis). […] IPF, as the name states, is idiopathic, however, there is an association with concurrent or previous history of smoking in 60% of patients and genetic factors. Up to 520% of patients with IPF have a family history of interstitial lung disease (ILD) or pulmonary fibrosis. […] The rs35705950 single-nucleotide polymorphism (SNP) a promoter site of an airway mucin gene (MUC5B) is strongly associated with IPF and familial pulmonary fibrosis and not seen in other secondary causes of lung fibrosis.

• #28 Pulmonary Fibrosis: Causes, Symptoms & Treatment

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

  Pulmonary fibrosis is scarring in your lungs. It can be idiopathic (without a known cause) or result from autoimmune diseases, environmental factors or medications. […] Pulmonary fibrosis usually gets worse over time, but how quickly it gets worse is different for everyone. […] Experts think pulmonary fibrosis happens when your lungs dont heal properly from damage or inflammation. Specific causes include: […] In most cases, the specific cause is unknown. […] Idiopathic pulmonary fibrosis is lung scarring without a known cause. Most cases of pulmonary fibrosis are idiopathic. […] Specific tests and procedures providers might use to diagnose pulmonary fibrosis include: […] Treatments for pulmonary fibrosis could include: […] Many causes of pulmonary fibrosis arent preventable. […] Lung scarring is nearly always permanent (unless caused by a medication and caught early). […] 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.

• #29 Idiopathic Pulmonary Fibrosis (IPF): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/301226-overview

  The etiology of idiopathic pulmonary fibrosis (IPF) remains undefined; however, in the current hypothesis regarding the pathogenesis of idiopathic pulmonary fibrosis, exposure to an inciting agent (eg, smoke, environmental pollutants, environmental dust, viral infections, gastroesophageal reflux disease, chronic aspiration) in a susceptible host may lead to the initial alveolar epithelial damage. […] This damage may lead to activation of the alveolar epithelial cells, which provokes the migration, proliferation, and activation of mesenchymal cells with the formation of fibroblastic/myofibroblastic foci, leading to the exaggerated accumulation of extracellular matrix with the irreversible destruction of the lung parenchyma. […] The following is a summary of possible inciting factors: Occupational dust or fume exposure (eg, hairdressers, ranchers, farmers, stonecutters, metal workers), Older age (approximately two thirds of patients are 60 years at diagnosis), Smoking history, Male sex (higher prevalence in men vs women), Genetics.

• #29 Idiopathic Pulmonary Fibrosis (IPF): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/301226-overview

  Other potential causes of idiopathic pulmonary fibrosis have been recognized through the study of familial pulmonary fibrosis. Familial pulmonary fibrosis, affecting two or more members of the same primary biological family, accounts for 10-20% of total patients with idiopathic pulmonary fibrosis. […] Genetic mutations in serum surfactant protein C have been discovered in some individuals with familial pulmonary fibrosis. […] Additionally, a common variant in the putative promoter of the gene encoding mucin 5B (MUC5B) has been associated with the development of both familial interstitial pneumonia and sporadic pulmonary fibrosis. […] Finally, mutant telomerase is associated with familial idiopathic pulmonary fibrosis. […] Pulmonary fibrosis in patients with short telomeres is provoked by a loss of alveolar epithelial cells. […] Respiratory viruses have been considered a particularly likely cause of AE-IPF based on the similarities in clinical and radiologic presentation between and AE-IPF and viral pneumonitis and the poor sensitivity of standard methods of viral detection.

• #30 Causes of Pulmonary Fibrosis in the Elderly

  https://www.mdpi.com/2076-3271/6/3/58

  Idiopathic pulmonary fibrosis (IPF) is the most common and most lethal type of idiopathic interstitial pneumonia. […] Although its etiology is unknown, several pathogenic pathways have been described that could explain this process, involving aging, environmental factors, genomic instability, loss of proteostasis, telomere attrition, epigenetic changes, mitochondrial dysfunction, cell senescence, and altered intercellular communication. […] However, the causes that lead to the uncontrolled fibrogenesis characteristic of the disease are still unknown, and it is difficult to identify the possible risk factors, given that patients are typically diagnosed at an advanced stage of the disease. […] Epidemiological studies have shown that one of the main predictors of IPF diagnosis is indeed age.

• #30 Causes of Pulmonary Fibrosis in the Elderly

  https://www.mdpi.com/2076-3271/6/3/58

  Aging is a complex multifactorial process characterized by a progressive loss of physiological integrity, accumulating deleterious changes of tissues, and cell damage, which are responsible for increased vulnerability and risk of diseases and death. […] It has been suggested that IPF is a consequence of accelerated aging of the lungs, or that pathogenic mechanisms develop there more easily after a certain age. […] It has been postulated that in genetically predisposed subjects, diverse exogenous factors such as environmental and occupational exposure or infection attack the alveolar epithelium, generating the fibrotic process. […] Several epidemiological studies support the conclusion that environmental factors have an etiologic role in IPF. […] Cigarette smoking, especially of more than 20 pack-years, is the most strongly associated environmental risk factor.

• #30 Causes of Pulmonary Fibrosis in the Elderly

  https://www.mdpi.com/2076-3271/6/3/58

  Other factors such as metal and wood dust exposure have also been associated with an increased risk of IPF. […] In addition to these external factors, endogenous factors also influence the development of IPF. […] Some studies suggest that pulmonary fibrosis is genetically determined. […] Aging increases the quantity of mutations while simultaneously decreasing the body’s ability to repair damaged DNA. […] General genetic mutations and epigenetic methylations accumulating with age release a process that involves excess generation of fibrous tissue and overall damage to the pulmonary system. […] There are pathogenic telomerase variants causing dysfunction of telomerase activity leading to accelerated telomere shortening. […] In summary, these studies demonstrate the possible therapeutic potential of addressing telomeric shortening in pulmonary fibrosis.

• #31 Idiopathic Pulmonary Fibrosis (IPF) | Canadian Lung Association

  https://www.lung.ca/lung-health/idiopathic-pulmonary-fibrosis-ipf

  Idiopathic means the cause is not known. […] No one knows what causes idiopathic pulmonary fibrosis or why some people get it. […] We do know some common risk factors for IPF. These include: Age: Almost all patients with IPF are over the age of 50 years. Cigarette smoking: Approximately 75% of people with IPF currently smoke tobacco or did smoke in the past. Acid reflux (gastroesophageal reflux disease [GERD]): About 75% of people with IPF have symptoms of acid reflux or heartburn. Sex: About 75% of patients with IPF are male. Genetics (family history): Up to 20% of people with IPF have another family member with an interstitial lung disease.

• #32 Idiopathic pulmonary fibrosis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/idiopathic-pulmonary-fibrosis/

  Researchers have also examined environmental risk factors that could contribute to idiopathic pulmonary fibrosis. These factors include exposure to wood or metal dust, viral infections, certain medications, and cigarette smoking. Some research suggests that gastroesophageal reflux disease (GERD) may also be a risk factor for idiopathic pulmonary fibrosis; affected individuals may breathe in (aspirate) stomach contents, which over time could damage the lungs.

• #33 Pulmonary Fibrosis – Causes and Risk Factors | NHLBI, NIH

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

  IPF is a type of interstitial lung disease. It is caused by lung tissue becoming thick and stiff and eventually forming scar tissue within the lungs. The scarring, or fibrosis, seems to result from a cycle of damage and healing that occurs in the lungs. Over time, the healing process stops working correctly and scar tissue forms. What causes these changes in the first place is unknown. […] Your risk for IPF is higher if a first-degree relative, such as a parent or sibling, has IPF. The specific genes you inherit may make you more likely to develop IPF, especially if those genes contain certain changes, or mutations. To date, mutations in more than 10 different genes have been linked to an increased risk for IPF. […] Scientists have found that mutations in certain genes are common among people who have IPF. Some of these genes help the body make surfactant and mucus, which are important for healthy lung function. One gene, called MUC5B, makes a mucus protein that helps clear harmful substances, such as Bacteria, from the lungs. Having a mutated MUC5B gene increases your risk for IPF more than other genes.

• #33 Pulmonary Fibrosis – Causes and Risk Factors | NHLBI, NIH

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

  Mutations in the TERT and TERC genes are also common in people who have IPF. These genes help protect the DNA in our cells as they divide over our lifespans. The genes do this by producing an enzyme called telomerase. More research must be done to understand why these mutations contribute to the development of IPF.

• #34

• #34 Idiopathic pulmonary fibrosis – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_pulmonary_fibrosis

  The cause is unknown, hence the term idiopathic. […] Risk factors include cigarette smoking, gastroesophageal reflux disease, certain viral infections, and genetic predisposition. […] The cause of IPF is unknown but certain environmental factors and exposures have been shown to increase the risk of getting IPF. Cigarette smoking is the best recognized and most accepted risk factor for IPF, and increases the risk of IPF by about twofold. […] Other environmental and occupation exposures such as exposure to metal dust, wood dust, coal dust, silica, stone dust, biologic dusts coming from hay dust or mold spores or other agricultural products, and occupations related to farming/livestock have also been shown to increase the risk for IPF. […] There is some evidence that viral infections may be associated with idiopathic pulmonary fibrosis and other fibrotic lung diseases.

• #35 Pulmonary fibrosis: pathogenesis, etiology and regulation

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2675823/

  Alleviating symptoms is the primary concern of patients presenting pulmonary fibrosis. Understanding the etiology of pulmonary fibrosis can provide long-term symptomatic relief and possible reversal of the disease. […] When all known causes of interstitial lung disease and fibrosis have been ruled out, the condition is referred to as idiopathic (of unknown origin) pulmonary fibrosis (IPF). Despite an unknown etiology, there are a number of conditions and risk factors associated with the disease including; smoking, farming, and occupational hazards and viral, and bacterial infections. […] The pathogenesis of IPF has been debated for many years with two different schools of thought. One group suggests an inflammatory stimulus is involved, with recurring inflammation leading to immunopathology, tissue destruction and the propagation of a wound-healing response.

• #35 Pulmonary fibrosis: pathogenesis, etiology and regulation

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2675823/

  Continuous chemokine and cytokine production in diagnosed IPF patients indicates that damage and subsequent inflammation may be ongoing. […] Collectively, a cascade of failed regulatory mechanisms and hyper-secretion of cytokines, chemokines and growth factors culminates in an out-of-control fibroblast-mediated wound-healing response.

• #35 Pulmonary fibrosis: pathogenesis, etiology and regulation

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2675823/

  Pulmonary fibrosis and architectural remodeling of tissues can severely disrupt lung function, often with fatal consequences. The etiology of pulmonary fibrotic diseases is varied, with an array of triggers including allergens, chemicals, radiation and environmental particles. However, the cause of one of the most common pulmonary fibrotic conditions, idiopathic pulmonary fibrosis (IPF), is still unclear. […] A tightly regulated repair response following tissue injury is therefore critical. […] The prevalence and incidence of pulmonary fibrotic diseases are hard to estimate, given the vast array of clinical conditions. […] The nature of the insult or causative agent often dictates the character of the ensuing inflammatory response. […] The mechanisms leading to pulmonary fibrosis are diverse, with immeasurable genetic, environmental and immunological interactions regulating the entire process.

• #36 Idiopathic pulmonary fibrosis: pathogenesis and management | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-018-0730-2

  Ageing is a physiological progression to the death, through loss of function and increasing weakness. […] The damage to alveolar structure and the loss of AECs, with disruption of basement membrane, involves alveolar vessels and leads to increased vascular permeability. […] The contribution of mesenchymal cells, and particularly of fibroblasts and myofibroblasts is crucial for IPF pathogenesis; these cells are recruited, activated and induced to differentiate, trans-differentiate and proliferate by the abnormal biochemical environment created by activated epithelial and endothelial cells. […] Fibrocytes are circulating bone-marrow derived mesenchymal cell progenitors, co-expressing CD45 or CD34 with type-1 collagen. […] The activated epithelium recruits them exposing CXCL12, CCL2 and secreting TGF-1, a strong fibrocytes activator that induces SMA production.

• #36 Idiopathic pulmonary fibrosis: pathogenesis and management | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-018-0730-2

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease characterized by the aberrant accumulation of fibrotic tissue in the lungs parenchyma, associated with significant morbidity and poor prognosis. […] Over the last two decades much has been clarified about the pathogenic pathways underlying the development and progression of the lung scarring in IPF. […] Several environmental and microbial exposures have been proposed as playing roles in IPF pathobiology that might be far from collateral, making the concept of idiopathic less compelling. […] Individual genetic and epigenetic factors remain the most important for the development of the fibrotic process, although the contribution of the variants so far identified, or their interaction with the putative external factors has yet to be clarified.

• #36 Idiopathic pulmonary fibrosis: pathogenesis and management | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-018-0730-2

  Fibroblasts are tissue mesenchymal cells committed to re-establish a normal and well-structured ECM in wound healing repair process. […] The most important stimulating factor for trans-differentiation is TGF-1, but also PDGF plays a significant role. […] The pathobiology of IPF is leaded by aberrant epithelial-mesenchymal crosstalk, but the inflammation may play an important role. […] The role of lymphocytes is still debated; certain lymphocytic cytokines are considered profibrotic, with direct effects on fibroblasts and myofibroblasts activity. […] The prevalence of COPD in IPF ranges from 6% to 67% across 23 different studies. […] Pulmonary hypertension (PH), usually defined as mean pulmonary arterial pressure (mPAP) 25 mmHg, is one of the conditions more frequently associated to IPF and has been widely demonstrated to increase mortality in this population. […] The coexistence of coronary artery disease deserves attention is observed in up to 30% of patients with IPF and increases mortality. […] Anxiety-depressive disorder considerably affects patients with IPF with a prevalence of 1150%.

• #37 Causes and Risks of Pulmonary Fibrosis | Temple Health

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

  Familial pulmonary fibrosis (FPF) occurs when 2 or more members of the same family develop pulmonary fibrosis. Some 2% of FPF cases are linked to idiopathic pulmonary fibrosis. Research suggests family history and exposure to certain environmental factors such as cigarette smoke may increase your risk of developing IPF. […] Idiopathic pulmonary fibrosis (IPF) is diagnosed when the cause of pulmonary fibrosis is unknown. IPF is usually diagnosed between the ages of 50 and 70, and is the most common type of pulmonary fibrosis. As with other types of pulmonary fibrosis, IPF causes thickening and scarring of the lungs resulting in a dry, hacking cough. While the cause is unknown, studies suggest a variant in the MUC5B gene may be the most important risk factor.

• #38

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

  When a person is diagnosed with pulmonary fibrosis (PF), sometimes there is a clear association with another illness, or the lung scarring (fibrosis) is the result of a side effect from a medication, radiation treatments to the chest, or an environmental or occupational exposure known to cause PF. […] Pulmonary fibrosis that is associated with another disease, such as scleroderma or rheumatoid arthritis, would be referred to as pulmonary fibrosis secondary to scleroderma or secondary to rheumatoid arthritis. […] When the cause of the disease is not known, the term idiopathic is used. […] There are multiple forms of ILD that are idiopathic, but idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic types of ILD. […] There are also some patients who have a form of PF that runs in families, which affects from 3-20% of people who are diagnosed with PF.

• #39 Idiopathic Pulmonary Fibrosis Causes | NSIP Lung | PF NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-causes/

  In rare cases, PF can run in families. Familial pulmonary fibrosis is usually defined when two people in the same biological family develop IPF and/or any other form of idiopathic interstitial pneumonia. Previous studies reported this particular form of PF has been identified in about 100 families. Mutations in TERC and TERT account for roughly 15% of all cases of familial pulmonary fibrosis, which appears to be inherited in an autosomal dominant pattern, meaning that a single copy of a faulty gene is sufficient to trigger its onset.

• #39 Idiopathic Pulmonary Fibrosis Causes | NSIP Lung | PF NewsEnvelope icon

  https://pulmonaryfibrosisnews.com/pulmonary-fibrosis-causes/

  Certain antibiotics and anti-inflammatory medications, like nitrofurantoin and sulfasalazine, also can cause PF. Medications for heart conditions, like amiodarone, also can trigger the onset of the disease, as can the anti-seizure medication phenytoin. […] While a person’s genetic makeup typically does not cause PF outright, it may make an individual more susceptible to the disease. […] For example, mutations in the genes TERC and TERT are tied to an increased risk of IPF and other types of PF. These genes provide instructions for making components of telomerase, an enzyme that maintains telomeres — structures found at the end of chromosomes to stabilize and protect DNA. It is thought that lower telomerase function, as a result of these mutations, could cause telomeres to become abnormally shorter, which in turn may cause lung cells to stop dividing or die prematurely.

• #40

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

  There are rare genetic forms of PF that can affect both children and adults when just a single gene is inherited in an abnormal form (usually from both parents). […] In the case of pulmonary fibrosis, there are currently no treatments or preventive care options based on genetic testing, but this is the goal for the future.

• #41 Who Gets Idiopathic Pulmonary Fibrosis? What are the Causes?

  https://pulmonaryfibrosismd.com/gets-idiopathic-pulmonary-fibrosis/

  The term idiopathic literally means there is no known cause. We dont yet understand what causes IPF. Recent advances have identified a few hopeful leads that suggest that IPF may be due to accelerated aging of the lung tissue. The DNA repair system has been implicated as being defective in some patients. The implicated genes are called TERT and TERC. In other patients, the genes that code for a proteins that lubricates the lung tissue has been found to be abnormal (SFTPA2, SFTPC). There is much active research in this area. […] There are a very small number of patient who have familial idiopathic pulmonary fibrosis. These families have multiple family members that are affected. If you have no other family members with IPF or with similar symptoms, then it is extremely unlikely that your children will be affected.

• #42

  https://www.jci.org/articles/view/74368

  The cellular and molecular mechanisms that were hardwired to generate fibrotic responses to infectious pathogens may have been co-opted to respond to noninfectious stressors in our modern environment. […] Autoimmunity is defined by the presence of specific receptor-mediated lymphocyte responses to distinct, particular autologous peptides. […] The overwhelming preponderance of these secondary autoimmune responses appear to be clinically benign, but they are highly pathogenic in an important minority of cases, including carditis, nephritis, neurologic dysfunction associated with group A streptococcal and other microbial infections, paraneoplastic syndromes linked to malignancies, and myriad other tissue-specific autoimmune disorders. […] Genetic studies in familial cases of IPF indicate a number of pathways related to protein folding and/or trafficking that may adversely affect the regenerative capacity of the epithelium.

• #42

  https://www.jci.org/articles/view/74368

  There is growing recognition that aging is a risk factor for fibrosis. […] The switch from regeneration to fibrosis as the primary repair response may represent slow, stochastic changes in cells and/or tissues driven by the accumulation of cellular or molecular damage. […] The concept of antagonistic pleiotropy was proposed by Williams in 1957 to explain senescence and aging; however, this concept may even more aptly apply to a number of complex, age-related diseases. […] We have proposed the concept of antagonistic pleiotropy to explain the role of the ROS-generating enzyme NADPH oxidase 4 (NOX4), which mediates myofibroblast differentiation and normal wound healing in young subjects, but promotes persistent fibrosis with aging. […] The mechanisms that account for the detrimental roles of developmental genes and/or pathways in age-related diseases may involve the permissive effect of specific aging phenotypes, including loss of proteostasis, metabolic reprogramming, immune dysregulation, and alterations in stem cell niches.

• #43 Understanding PF | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/nurses/understanding-PF/what-is-PF

  Pulmonary fibrosis occurs commonly in a wide range of interstitial lung diseases (ILDs), it can cause irreversible lung damage and is associated with increased mortality. […] Pulmonary fibrosis causes irreversible lung damage and sustained fibrosis leads to early mortality. […] Support your patients by knowing that pulmonary fibrosis occurs in a wide range of ILDs and is associated with an increased mortality. […] While each patient with ILD is unique, common pathogenic pathways to pulmonary fibrosis are shared. […] Patients with ILDs develop pulmonary fibrosis via common pathogenic pathways, irrespective of the underlying diagnosis or trigger. […] In the early stages of ILD pathogenesis, inflammation and fibrosis can coexist and lead to the development of pulmonary fibrosis. […] As ILD progresses, it may develop into progressive pulmonary fibrosis (PPF), leading to increased, self-sustaining fibrosis. […] Importantly, patients should understand that pulmonary fibrosis causes irreversible lung damage and sustained fibrosis may lead to early mortality. […] Patients with a range of ILDs may develop PPF, which is associated with high mortality.

• #44 Interstitial lung disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/symptoms-causes/syc-20353108

  Interstitial lung disease can have many causes, including long-term exposure to hazardous materials such as asbestos. […] Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. […] Interstitial lung disease seems to occur when an injury to your lungs causes a healing response that isn’t proper. […] Tissue in and around the lungs’ air sacs, called alveoli, becomes inflamed, scarred and thickened. […] Certain diseases or conditions may lead to interstitial lung disease. […] These include autoimmune diseases, such as rheumatoid arthritis, scleroderma and mixed connective tissue disease. […] The list of substances and conditions that can lead to interstitial lung disease is long. […] Conditions without a known cause are grouped together under the label of idiopathic interstitial pneumonias. […] Idiopathic pulmonary fibrosis, also called IPF. […] IPF is a typically progressive lung disease that occurs when lung tissue becomes damaged and scarred what’s known as fibrosis.

• #45 Underlying and immediate causes of death in patients with idiopathic pulmonary fibrosis | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-018-0642-4

  The most common cause of death in IPF patients has been reported to be the disease itself followed by cardiac disorders and lung cancer. […] A rapid deterioration of the disease may be caused by pulmonary embolism, pneumothorax, infections or heart failure. […] The main underlying causes of death were IPF, ischemic heart disease and lung cancer. The most common immediate causes of death were IPF, pneumonia and ischemic heart disease. […] An acute exacerbation was observed in 10 (12.7%) and a suspected acute exacerbation of IPF in 28 (35.4%) patients with IPF as the underlying cause of death. […] More patients with a rapid disease progression died from an acute exacerbation compared to their counterparts with a moderate and slow disease progression. […] Even though the overall mortality was higher in males, the disease-specific mortality for IPF was higher in females, with males having more commonly comorbidities as the underlying cause of death. […] An acute exacerbation of IPF may be associated with smoking and gender since females and non-smokers were less likely to develop pneumonia, an illness that is known to trigger acute exacerbations.

• #46 Pulmonary & Sleep of Tampa Bay | Pulmonary Fibrosis: Examine the Causes, Symptoms, and Treatment Approaches – Pulmonary & Sleep of Tampa Bay

  https://pulmonaryandsleephealth.com/pulmonary-fibrosis-examine-the-causes-symptoms-and-treatment-approaches/

  Pulmonary fibrosis refers to a group of lung disorders that cause the tissue in the lungs to become thick and stiff, making it harder for the lungs to function properly. The scarring (fibrosis) can be a result of various causes, and the exact mechanisms differ between different types of pulmonary fibrosis. The most common type is idiopathic pulmonary fibrosis (IPF), where the cause is unknown. […] Environmental Factors: Exposure to environmental toxins, such as asbestos, silica dust, and other occupational hazards, can contribute to lung scarring and the development of pulmonary fibrosis. […] Autoimmune Diseases: Conditions like rheumatoid arthritis and lupus can lead to autoimmune-related pulmonary fibrosis, where the body’s immune system mistakenly attacks healthy lung tissue. […] Genetic Factors: Some cases of pulmonary fibrosis have a hereditary component, which means they can run in families.

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