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. Often, doctors and other healthcare professionals cannot pinpoint what’s causing the problem. When a cause cannot be found, the condition is called idiopathic pulmonary fibrosis. […] 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. […] 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.

• #2

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

  Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult. […] It’s not clear what causes it, but it usually affects people who are around 70 to 75 years old, and is rare in people under 50. […] 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.

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

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

  Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). It causes lung scarring, which, over time, results in reduced oxygen intake. […] 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.

• #4 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. […] In many cases, the exact cause of pulmonary fibrosis in unknown. When this is the case, the condition is called idiopathic pulmonary fibrosis (IPF).

• #5 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. But the cause isn’t known sometimes. ILD can have many causes, so treatment varies. […] Interstitial lung disease seems to occur when an injury to your lungs causes a healing response that isn’t proper. Ordinarily, your body creates just the right amount of tissue to repair damage. But in ILD, the repair process doesn’t work properly. Tissue in and around the lungs’ air sacs, called alveoli, becomes inflamed, scarred and thickened. This makes it harder for oxygen to pass into your bloodstream. […] Certain diseases or conditions may lead to interstitial lung disease. For example: Connective tissue diseases. These include autoimmune diseases, such as rheumatoid arthritis, scleroderma and mixed connective tissue disease. These diseases result in an immune response that isn’t proper and may cause tissue inflammation and scarring in the body, including in the lungs.

• #6 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. […] Following injury it is paramount that tissue architecture is restored to regain normal organ function. […] However, common to most fibrotic conditions is the presence of a persistent irritant, which can be known agents, such as allergens, toxic chemicals, radiation, or other persistent irritants or unknown factors that trigger idiopathic pulmonary fibrosis (IPF). […] The prevalence and incidence of pulmonary fibrotic diseases are hard to estimate, given the vast array of clinical conditions. IPF affecting 30 in 100 000 with 34 000 new cases annually and allergic asthma, affecting one in five in the United States; although not always leading to airway remodeling and fibrosis, which are two of the most common pulmonary fibrotic diseases.

• #7 What is idiopathic pulmonary fibrosis? | Action for Pulmonary Fibrosis

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

  IPF is the commonest form of pulmonary fibrosis and it is estimated that there are 6,000 new cases of this condition each year in the UK. […] We do not know what causes IPF. Research suggests that fibrosis occurs in response to damage of the cells that line the air sacs (alveoli). It is likely due to a combination of genetic and environmental factors, such as smoking, dusts from metal, wood and agricultural work. […] IPF may run in families: it has been estimated that 5-20% of patients have a relative with the disease. This is termed familial pulmonary fibrosis.

• #8

  https://www.pulmonaryfibrosis.org/understanding-pff/types-of-pulmonary-fibrosis/idiopathic-pulmonary-fibrosis

  A family history of pulmonary fibrosis is also a risk factor, as are certain genes, such as MUC5B, TERT, TERC, DKC1, RTEL1, AKAP13, DSP, FAM13A, DPP9, and TOLLIP. Some evidence suggests that certain viral infections, air pollution, and some exposures in the workplace may also be risk factors for IPF. […] There are also conditions, such as gastroesophageal reflux disease (acid reflux, heartburn, or GERD), sleep apnea, or pulmonary hypertension (PH) that are often present in people who are diagnosed with IPF. These conditions are called comorbid conditions or comorbidities. The presence of risk factors and comorbidities provide clues that may be helpful to a physician who suspects that an ILD is present in a patient. […] Diagnosing an ILD involves many different types of information, and making an accurate diagnosis can be complicated. Some patients disease is easier to diagnose because the cause and type of disease are clear. For other patients a cause cannot be found, and they may not fit easily into any particular category of the disease. In addition to IPF, there are many ILDs of unknown cause meaning that there are many idiopathic ILDs other than IPF.

• #9 Idiopathic pulmonary fibrosis – Wikipedia

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

  The cause is unknown, hence the term idiopathic. […] 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. […] The fibrosis in IPF has been linked to cigarette smoking, environmental factors (e.g. occupational exposure to gases, smoke, chemicals or dusts), other medical conditions including gastroesophageal reflux disease, or to genetic predisposition (familial IPF). […] Despite extensive investigation, the cause of IPF remains unknown. […] Familial IPF accounts for less than 5% of the total of patients with IPF and is clinically and histologically indistinguishable from sporadic IPF. […] Genetic associations include mutations in pulmonary surfactant proteins A1, A2, C (SFTPA1, SFTPA2B) and mucin (MUC5B).

• #10 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.

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

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

  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. Telomere shortening also occurs with aging and can also be acquired. This telomere shortening could promote the loss of alveolar epithelial cells, resulting in aberrant epithelial cell repair, and therefore should be considered as another potential contributor to the pathogenesis of idiopathic pulmonary fibrosis.

• #12 Idiopathic pulmonary fibrosis: MedlinePlus GeneticsLock

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

  Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. […] The cause of idiopathic pulmonary fibrosis is unknown. The fibrosis that builds up in the lungs is thought to develop as a result of abnormal tissue repair following tissue damage. This abnormal repair response probably results from a combination of genetic and environmental factors. It is likely that genetic changes increase a person’s risk of developing idiopathic pulmonary fibrosis, and then exposure to certain environmental factors triggers the disease. […] Changes in several genes have been suggested as risk factors for idiopathic pulmonary fibrosis, while it is likely that other genetic influences have yet to be discovered. […] However, mutations in genes known as TERC and TERT have been found in about 15 percent of all cases of familial pulmonary fibrosis and a smaller percentage of cases of sporadic idiopathic pulmonary fibrosis.

• #13 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.

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

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

  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. […] 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. These mutations in serum surfactant protein C may damage type II alveolar epithelial cells.

• #15 Pulmonary fibrosis: Symptoms, treatment, and outlook

  https://www.medicalnewstoday.com/articles/pulmonary-fibrosis

  Idiopathic pulmonary fibrosis (IPF) has no known cause this is called idiopathic PF (IPF). […] Medical experts do not know the direct causes of IPF. […] Certain gene mutations are common among people with IPF. […] Researchers estimate that variations in genetics account for about one-third of an individual’s risk for developing IPF.

• #16 Overview of Idiopathic Pulmonary Fibrosis (IPF) and Evidence-Based Guidelines

  https://www.ajmc.com/view/overview-of-idiopathic-pulmonary-fibrosis-ipf-and-evidence-based-guidelines-article

  While the genome-wide association studies have identified the common genetic variants accounting for almost one-third of risk factors of development of IPF, they are limited by a lack of definite causal relationship. […] The understanding of the complex pathogenesis of IPF has evolved significantly over the past 2 decades. It is now generally agreed that the pathogenesis of IPF is related to epithelial injury from endogenous or exogenous events, which results in widespread fibrosis, which replaces the normal lung parenchyma. […] There is some evidence that GERD may be a causative factor of IPF, with animal studies documenting worsening pulmonary parenchymal damage with exposure to decreasing pH, and damage to the lung periphery from gastric acid.

• #17 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. […] Although IPF is by definition “idiopathic”, multiple factors have been reported to increase disease risk, aging being the most prominent one. 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. […] The factors that trigger the plethora of profibrotic pathways involved in IPF pathogenesis remain unknown, but advancing age, cigarette smoking, viral infection, chronic aspiration of gastric content and genetic factors are all associated with an increased risk of IPF, probably by causing an alveolar epithelial barrier injury.

• #18 Occupational and environmental risk factors of idiopathic pulmonary fibrosis: a systematic review and meta-analyses | Scientific Reports

  https://www.nature.com/articles/s41598-021-81591-z

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease of unknown cause. […] Any occupational or environmental exposure to metal dust (OR 1.83, 95% CI 1.15-2.91, I2=54%), wood dust (OR 1.62, 95% CI 1.04-2.53, I2=5%) and pesticide (OR 2.07, 95% CI 1.24-3.45, I2=0%) were associated with an increased risk of IPF. […] Moreover, smoking increased IPF risk with an odds ratio of 1.39 (95% CI 1.01-1.91, I2=29%). […] According to the 2015 Korean National Health and Nutrition Survey, those exposed to occupational and environmental dust were diagnosed with IPF at a younger age and had a longer period of symptomatic symptoms at the time of diagnosis. […] Exposure to metal dust, wood dust, pesticide, occupational history of farming or agriculture and ever smoking increased the risk of IPF.

• #19 Idiopathic pulmonary fibrosis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-8

  Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. […] Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). […] The term „idiopathic” suggests there are no known causes for IPF. […] However, an environmental etiology for IPF is supported by evidence from several sources. […] Cigarette smoking is consistently associated with IPF. […] A multi-center case-control study conducted in the United States included 248 patients with IPF and 491 matched control subjects. This study demonstrated significant associations between IPF and a) cigarette smoking (OR, 1.6; 95% CI, 1.12.4); b) silica exposure (OR, 3.9; 95% CI, 1.212.7); and c) exposure to livestock (OR, 2.7; 95% CI, 1.35.5).

• #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. […] 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:

• #21 Idiopathic Pulmonary Fibrosis Mortality by Industry and Occupation — United States, 2020–2022 | MMWR

  https://www.cdc.gov/mmwr/volumes/74/wr/mm7407a1.htm

  Idiopathic pulmonary fibrosis (IPF), a progressive lung disease characterized by scarring and worsening lung function, has a poor prognosis. An estimated 21% of IPF deaths might be attributable to occupational exposures. […] Although the exact IPF etiology remains unknown, studies have indicated associations with cigarette smoking, genetic mutations, viral infections (e.g., Epstein-Barr virus and hepatitis C), and occupational exposures to pesticides, and wood (pine) and metal (brass, lead, and steel) dust. […] The proportion of IPF cases associated with occupational exposures (the population attributable fraction) has been previously estimated to be 21%. […] Based on an estimate that 21% of IPF deaths might be related to occupational exposures, during this 3-year period, 8,340 IPF deaths in males and 5,908 deaths in females might have resulted from occupational exposures.

• #22 Occupational and environmental risk factors of idiopathic pulmonary fibrosis: a systematic review and meta-analyses | Scientific Reports

  https://www.nature.com/articles/s41598-021-81591-z

  Although smoking has not been established as a causative agent, it has been shown to increase the risk of IPF. […] A longer occupational exposure period is known to increase IPF risk. […] In conclusion, meta-analysis of patient-control studies revealed that exposure to pesticides, metal dust, and wood dust increases the risk of IPF. Additionally, the risk of IPF was more in agricultural workers. Lastly, smoking also increased the risk of IPF.

• #23 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.

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

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

  Cigarette smoking is the main risk factor. The disease is more common in people who smoke or have been smokers at some time, and continuing to smoke after diagnosis is associated with poorer chances of survival. […] IPF seems to run in some families. However, in about 4 out of 5 cases, there is no family history of the disease.

• #25 Idiopathic pulmonary fibrosis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-8

  Several intriguing reports suggest the involvement of herpesvirus and/or hepatitis C virus in the etiology of IPF. […] The currently accepted paradigm proposes that injury to the alveolar epithelium is followed by a burst of pro-inflammatory and fibroproliferative mediators that invoke responses associated with normal tissue repair. […] For unclear reasons, these repair processes never resolve and progressive fibrosis ensues.

• #26 Idiopathic Pulmonary Fibrosis | RT

  https://respiratory-therapy.com/disorders-diseases/chronic-pulmonary-disorders/pulmonary-fibrosis/idiopathic-pulmonary-fibrosis/

  Pulmonary fibrosis is a component of 200 interstitial lung diseases, many of which have no known cause. […] Idiopathic pulmonary fibrosis (IPF) makes up a large subset of idiopathic interstitial pneumonia that has been the focus of much research. […] Even though the cause(s) of IPF are unknown, six potential risk factors have been identified through research. […] The pathogens involved include Epstein-Barr virus, influenza, cytomegalovirus, hepatitis C virus, parainfluenza and parainfluenza 3 viruses, HIV-1, measles virus, herpesvirus 6, Mycoplasma, and Legionella. […] Apparently, when IPF occurs, the normal process is disrupted, and several detrimental changes occur in the lung. […] As IPF develops, normal steps in the coagulation process appear to go awry, and fibrin deposits begin to build up in the lung.

• #27 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. […] The cytokine profile from biopsy or BAL-derived cells or BAL fluid of IPF patients is rich in pro-inflammatory cytokines; IL-1, IL-8, IL-18, TNF, MCP-1 as well as Type-2 cytokines, and their receptors. […] 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.

• #28 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.

• #29 Idiopathic Pulmonary Fibrosis: Causes, Signs, Treatment

  https://www.verywellhealth.com/idiopathic-pulmonary-fibrosis-7484192

  Idiopathic pulmonary fibrosis is a severe, chronic lung disease. […] The causes of this illness are not fully known. As a result, this disease is called idiopathic, meaning it has no known cause. […] While the cause is unknown, idiopathic pulmonary fibrosis often occurs in people who have one of the following comorbidities (simultaneous disease): pulmonary hypertension, ischemic heart disease, lung cancer, emphysema/chronic obstructive pulmonary disease (COPD), gastroesophageal reflux disease (GERD), sleep apnea, and depression. […] Having one or more of the following factors can increase your chances of developing idiopathic pulmonary fibrosis with or without another disease present: male sex, being in your 60s or 70s, smoking, a first-degree relative, such as a parent or sibling, with idiopathic pulmonary fibrosis, and exposure to hazardous materials such as asbestos or silica. […] The causes of this disease are not fully known, though having certain risk factors can make you more likely to develop it.

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

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

  Most of the time, the cause of pulmonary fibrosis is not known. […] If this is the case, it is called idiopathic pulmonary fibrosis (idiopathic means the cause is unknown). […] 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. […] Having idiopathic pulmonary fibrosis can increase your chance of developing: lung cancer, pulmonary hypertension, where the blood pressure in the arteries that carry blood from the heart to the lungs is higher than normal, lung infections, anxiety, depression.

• #31

  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. […] 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.

• #32 IPF (Idiopathic Pulmonary Fibrosis)

  https://www.svhlunghealth.com.au/conditions/ipf-idiopathic-pulmonary-fibrosis

  Idiopathic Pulmonary Fibrosis (IPF) is a common type of Interstitial Lung Disease (ILD) that causes thickening and scarring (fibrosis) of the lung tissue. […] We dont fully understand what causes IPF. The term idiopathic means no known cause. […] However, there are some factors that can increase your risk of IPF. Common factors can include: A family history of IPF called familial pulmonary fibrosis, Smoking or having previously smoked, Gastro-oesophageal reflux disease (GORD) may contribute to progression of disease. […] IPF also tends to be more common in men aged over 50.

• #33 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. […] Even though the overall mortality was higher in males with IPF, the disease-specific mortality for IPF was higher in females i.e. in males, comorbidities were more often the underlying causes of death. […] The main underlying causes of death were IPF, ischemic heart disease and lung cancer. […] 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. […] 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.

• #34 Pulmonary fibrosis: pathogenesis, etiology and regulation

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

  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. […] IPF is characterized by usual interstitial pneumonitis and progressive interstitial fibrosis caused by excessive extracellular matrix deposition. […] 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. Others suggest a slightly different pathogenic mechanism in which an initial or absent inflammatory stage is quickly followed by an uncontrolled wound-healing response.

• #35

  https://link.springer.com/article/10.1007/s12016-010-8211-5

  Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease (ILD) affecting the pulmonary interstitium. […] Familial cases of IPF also exist, suggesting a genetic predisposition; telomerase mutations have been observed to occur in familial IPF, which may also explain the increase in IPF with advancing age. […] Alveolar epithelial cells are believed to be the primary target of environmental agents that have been putatively associated with IPF. These agents may include toxins, viruses, or the autoantibodies found in collagen vascular diseases. […] The mechanism of disease is still unclear in IPF, but aberrations in fibroblast differentiation, activation, and proliferation may play a role. […] It is becoming increasingly clear that the pathogenesis of IPF is indeed a complex and convoluted process that involves numerous cell types and humoral factors.

• #36 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 has been shown that fibroblasts in this process demonstrate neoplastic or neoproliferative characteristics. […] 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.

• #37 Idiopathic Pulmonary Fibrosis | RT

  https://respiratory-therapy.com/disorders-diseases/chronic-pulmonary-disorders/pulmonary-fibrosis/idiopathic-pulmonary-fibrosis/

  The concept of multiple hits may explain some of the causes for the spiraling deterioration in the lung parenchyma and continuing buildup of a fibrous, stiff extracellular matrix. […] Despite efforts to treat this disease, the mean survival time ranges from 2 to 4 years after diagnosis. […] Possible future therapeutic strategies include agents that inhibit the negative (fibrotic) effects of cytokines, proteases, or fibroblast growth factors.

• #38 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, identified histologically as usual interstitial pneumonia, accounts for most cases of idiopathic interstitial pneumonia. […] A combination of environmental, genetic, and other unknown factors probably contribute to alveolar epithelial cell dysfunction or reprogramming, which leads to abnormal fibroproliferation in the lung. […] Current or former cigarette smoking is most strongly associated with the disorder. There is some genetic predisposition; familial clustering occurs in up to 20% of cases. […] 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.

• #39 Idiopathic Pulmonary Fibrosis (IPF): Causes, Symptoms, and Treatment

  https://continentalhospitals.com/blog/idiopathic-pulmonary-fibrosis-ipf-causes-symptoms-and-treatment/

  Idiopathic pulmonary fibrosis is a type of interstitial lung disease (ILD). This group of diseases results in the inflammation and scarring of lung tissue. While the specific cause of the disease remains unknown, there are several predisposing factors. […] While the exact etiology remains unknown, some factors may indicate a predisposition to developing IPF. […] However, most cases have no known cause, making the disease „idiopathic.” […] The exact cause of IPF is unknown. However, it is believed to result from a combination of genetic and environmental factors.

• #40 What is IPF? | Idiopathic Pulmonary Fibrosis Information

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

  The diagnosis of idiopathic pulmonary fibrosis is made only after other causes of symptoms have been ruled out. […] As of January 2025, the U.S. Food and Drug Administration (FDA) has approved two medications for treatment of idiopathic pulmonary fibrosis (IPF) — Nintedanib (Ofev) and Pirfenidone (Esbriet). […] Clinical trials help determine new treatment options for diseases and conditions. Patients with idiopathic pulmonary fibrosis have access to clinical trials and should speak with their physician to determine what trials might work best for them.

• #41

  https://www.pulmonaryfibrosis.org/understanding-pff/types-of-pulmonary-fibrosis/idiopathic-pulmonary-fibrosis

  If a cause for the PF, such as an autoimmune disease or environmental exposure, is identified, then the diagnosis is not IPF. […] The Pulmonary Fibrosis Foundation is leading the fight by funding promising research and we need your help. […] The more individuals who join and provide responses, the closer we come to a cure.

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