Materiały źródłowe

• #1

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

  Idiopathic pulmonary fibrosis (IPF) can be hard to diagnose because its symptoms are similar to other lung conditions, such as chronic obstructive pulmonary disease (COPD). […] A GP can refer you to hospital specialists for a number of tests to help rule out other conditions and confirm the diagnosis. […] Lung function tests (also called pulmonary function tests) assess how well your lungs work and can help indicate what the problem may be. […] A commonly used lung function test is spirometry. During the test, you breathe into a mouthpiece that’s attached to a monitor. […] If IPF is suspected, the chest X-ray will be followed by a CT scan. […] This can help your doctor spot signs of scarring in your lungs. […] If doctors are still not sure what the problem is after these tests, they may suggest having a bronchoscopy. […] If other tests are not conclusive, a lung biopsy may need to be carried out. […] This involves keyhole surgery to remove a small sample of lung tissue so it can be analysed for signs of scarring.

• #2 How Is Pulmonary Fibrosis Diagnosed? | American Lung Association

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

  Pulmonary fibrosis (PF) may be difficult to diagnose as the symptoms of PF are similar to other lung diseases. […] If your doctor suspects you might have PF, it is important to see a specialist to confirm your diagnosis. This will help ensure you are treated for the exact disease you have. […] A high-resolution computed tomography scan, or HRCT scan, is an X-ray that provides sharper and more detailed pictures than a standard chest X-ray and is an important component of diagnosing PF. […] If more sampling is required to make a diagnosis, your doctor might perform a biopsy.

• #3 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/pulms/evaluation-diagnosis-of-ILDs/diagnosis-of-ILDs

  HRCT is the gold standard diagnostic tool for ILDs. […] In the absence of other known causes, HRCT, in discussion with an MDT, is necessary to confirm an IPF diagnosis. […] MDTs reach an IPF diagnosis more frequently and with higher confidence than individual clinicians. […] Diagnosis of IPF is confirmed from either HRCT, BAL or surgical lung biopsy. […] If a specific diagnosis is not made or no potential cause for ILD is identified, then clinical findings and appropriate combination of HRCT and histopathological patterns are considered during multidisciplinary discussion to either ascertain or exclude the diagnosis of IPF. […] Patients clinically suspected of having IPF are those with unexplained symptomatic or asymptomatic patterns of bilateral pulmonary fibrosis on a chest radiograph or chest computed tomography, bibasilar inspiratory crackles, and age greater than 60 years.

• #4 CHEST Annual Meeting

  https://www.healio.com/news/pulmonology/20221025/time-lost-is-lung-lost-early-diagnosis-critical-in-pulmonary-fibrosis

  Delayed diagnosis of pulmonary fibrosis is an unfortunate reality for many patients, but experts are seeking to address this problem by closing knowledge gaps and increasing awareness of the disease. […] There are more than 200 causes for pulmonary fibrosis and the diagnostic process is fairly complex in the sense of understanding the demographic and clinical presentation of patients and investigations required. […] For physicians who may not specialize in ILD, the complicated nature of pulmonary fibrosis, vague symptoms and need for more extensive testing can prove challenging. […] On average, delayed diagnosis can delay treatment as much as 1 to 5 years. […] However, we can really slow down the progression of the disease and give the patient a much better quality of life. […] Additionally, we have medications to treat the disease, lung transplant for those with severe pulmonary fibrosis and ongoing drug trials in which patients can potentially participate.

• #5 Idiopathic pulmonary fibrosis in the United States: time to diagnosis and treatment | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-023-02565-7

  The average time to a diagnosis of IPF was 2.7 years after initial respiratory diagnosis. […] Notably, we found that only 4,649 or 10.3% of patients with IPF were treated with an anti-fibrotic during their disease course. […] The prognosis for IPF from this real-world patient cohort was similar to other cohorts described, with a mean 2.88-year prognosis following clinical diagnosis. […] Despite increasing knowledge regarding the risk factors and available treatments for IPF, delays in the diagnosis of this disorder and the initiation of therapy persist.

• #6 Delayed Diagnosis | ILD Collaborative

  https://www.ildcollaborative.org/resources/ipf-patient-journey/delayed-diagnosis

  Many people with idiopathic pulmonary fibrosis (IPF) experience a long journey before they are correctly diagnosed. Right now, the average delay in the United States between the start of a persons earliest symptoms of breathlessness and accurate diagnosis of IPF is a little over two years. However, an accurate diagnosis can take as long as four or five years. […] A survey study of 600 people with IPF found that over half (55%) had at least one wrong diagnosis before they were correctly diagnosed. […] The longer the delay in correct diagnosis, the more risk that a person will die from complications of IPF in the five years after diagnosis. Early treatment is better for survival. […] IPF is difficult for primary care doctors to recognize in the early stages for two reasons. First, IPF is very rare. Because IPF is a rare disease, some primary care doctors have never seen a patient with IPF.

• #7 Delayed Diagnosis | ILD Collaborative

  https://www.ildcollaborative.org/resources/ipf-patient-journey/delayed-diagnosis

  Many people with idiopathic pulmonary fibrosis (IPF) experience a long journey before they are correctly diagnosed. Right now, the average delay in the United States between the start of a persons earliest symptoms of breathlessness and accurate diagnosis of IPF is a little over two years. However, an accurate diagnosis can take as long as four or five years. […] A survey study of 600 people with IPF found that over half (55%) had at least one wrong diagnosis before they were correctly diagnosed. […] The longer the delay in correct diagnosis, the more risk that a person will die from complications of IPF in the five years after diagnosis. Early treatment is better for survival. […] IPF is difficult for primary care doctors to recognize in the early stages for two reasons. First, IPF is very rare. Because IPF is a rare disease, some primary care doctors have never seen a patient with IPF.

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

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

  To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. […] During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs. […] A high-resolution CT scan can be helpful in diagnosing pulmonary fibrosis and in finding out how much lung damage has occurred. […] In addition to showing whether you have pulmonary fibrosis, imaging and lung function tests can be used to check your condition over time and see how treatments are working. […] If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed. This is called a biopsy. The biopsy sample is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. […] A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life.

• #9 Diagnosing Idiopathic Pulmonary Fibrosis | NYU Langone Health

  https://nyulangone.org/conditions/idiopathic-pulmonary-fibrosis/diagnosis

  Idiopathic pulmonary fibrosis is a rare and serious condition in which the tiny air sacs within the lungs and the surrounding tissue become thick and scarred. […] The symptoms of idiopathic pulmonary fibrosis can mimic several other lung conditions. To diagnose this condition, an NYU Langone doctor takes a medical history and performs a physical exam, during which he or she listens to your lungs with a stethoscope. If there is a crackling sound, the doctor may order one or more tests to rule out other lung conditions. […] Because evidence of idiopathic pulmonary fibrosis may not be evident on chest X-rays, further testing is often required. […] If the X-ray does not provide enough information, your doctor may order a CT scan, in which cross-sectional, three-dimensional images of the body are produced using a series of X-ray images and a computer. A CT scan can reveal the extent of scarring in the lungs.

• #10 Pulmonary Fibrosis – Diagnosis | NHLBI, NIH

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

  Your doctor will diagnose IPF based on your symptoms, your medical and family history, your risk factors, and the results from tests and procedures. […] To help determine whether you have IPF and rule out other possible causes of lung problems, your doctor may ask about your medical history and possible risk factors. […] To diagnose IPF, your doctor may order some of the following tests and procedures. […] This is used to see if your lung tissue shows signs of inflammation, scarring, or other changes. This procedure is sometimes used to verify the diagnosis of IPF. […] Your doctor may perform some tests and procedures to help rule out other conditions that may cause lung disorders. […] These tests help assess the severity of your lung disease, and they can help monitor whether your condition is stable or worsening over time. […] This is used to see if you have mutations, or changes in your gene, that can raise your risk of IPF.

• #11 Idiopathic pulmonary fibrosis – Wikipedia

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

  An earlier diagnosis of IPF is a prerequisite for earlier treatment and, potentially, improvement of the long-term clinical outcome of this progressive and ultimately fatal disease. […] If IPF is suspected, diagnosis can be challenging but a multidisciplinary approach involving a pulmonologist, radiologist and pathologist expert in interstitial lung disease has been shown to improve the accuracy of IPF diagnosis. […] A Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS) in 2000 proposed specific major and minor criteria for establishing the diagnosis of IPF. […] Currently, a diagnosis of IPF requires: Exclusion of known causes of interstitial lung disease (ILD), e.g., domestic and occupational environmental exposures, connective tissue disorders, or drug exposure/toxicity.

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

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

  To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. […] During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs. […] A high-resolution CT scan can be helpful in diagnosing pulmonary fibrosis and in finding out how much lung damage has occurred. […] In addition to showing whether you have pulmonary fibrosis, imaging and lung function tests can be used to check your condition over time and see how treatments are working. […] If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed. This is called a biopsy. The biopsy sample is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. […] A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life.

• #13 Diagnosis – Lung Foundation Australia

  https://lungfoundation.com.au/patients-carers/living-with-a-lung-disease/pf/diagnosis/

  Many people with Pulmonary Fibrosis (PF) do not receive a diagnosis until they have had symptoms for some time. […] The diagnosis of Pulmonary Fibrosis can be challenging because it can be confused with other lung conditions such as asthma, Chronic Obstructive Pulmonary Disease (COPD) and heart disease. […] It is important for your healthcare team to identify the underlying type of PF, to help determine the most appropriate treatment options for your condition. […] It therefore requires careful evaluation by a specialist. […] Some of the tests they will perform include: […] Physical examination: Your specialist doctor will listen to your chest to see if they can hear fine crackles in your chest that can sound like velcro. These can be a valuable sign in early diagnosis. […] Blood tests: This is mainly to exclude known causes of lung scaring.

• #14 Idiopathic pulmonary fibrosis – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/446

  Other diagnostic factors include weight loss, fatigue, malaise, and clubbing. […] 1st investigations to order include CXR, high-resolution CT (HRCT) chest, and pulmonary function tests. […] Investigations to consider include surgical lung biopsy, bronchoalveolar lavage (BAL), trans-bronchial lung biopsy and cryobiopsy, CRP, erythrocyte sedimentation rate (ESR), anti-nuclear antibody immunofluorescence, rheumatoid factor, anti-cyclic citrullinated peptide, and myositis panel.

• #15

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

  If a GP thinks you could have a lung condition such as IPF, they can refer you to a hospital specialist for tests such as: […] breathing (lung function) tests […] blood tests […] a chest X-ray and CT scan […] a lung biopsy, where a small piece of lung tissue is removed during keyhole surgery so it can be analysed.

• #16 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Results from routine laboratory studies are nonspecific for the diagnosis of idiopathic pulmonary fibrosis; however, some laboratory studies may be helpful for ruling out other causes of interstitial lung disease. Reportedly, up to 30% of patients with idiopathic pulmonary fibrosis (IPF) have positive tests for antinuclear antibodies or rheumatoid factor; however, these titers are generally not high. […] The chest radiograph lacks diagnostic specificity for idiopathic pulmonary fibrosis. Virtually all patients with idiopathic pulmonary fibrosis (IPF) have an abnormal chest radiograph at the time of diagnosis. […] High-resolution computed tomography (HRCT) findings are significantly more sensitive and specific for the diagnosis of idiopathic pulmonary fibrosis and are an essential component of the diagnostic pathway of idiopathic pulmonary fibrosis.

• #17 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/pulms/evaluation-diagnosis-of-ILDs/diagnosis-of-ILDs

  HRCT is the gold standard diagnostic tool for ILDs. […] In the absence of other known causes, HRCT, in discussion with an MDT, is necessary to confirm an IPF diagnosis. […] MDTs reach an IPF diagnosis more frequently and with higher confidence than individual clinicians. […] Diagnosis of IPF is confirmed from either HRCT, BAL or surgical lung biopsy. […] If a specific diagnosis is not made or no potential cause for ILD is identified, then clinical findings and appropriate combination of HRCT and histopathological patterns are considered during multidisciplinary discussion to either ascertain or exclude the diagnosis of IPF. […] Patients clinically suspected of having IPF are those with unexplained symptomatic or asymptomatic patterns of bilateral pulmonary fibrosis on a chest radiograph or chest computed tomography, bibasilar inspiratory crackles, and age greater than 60 years.

• #18 Idiopathic Pulmonary Fibrosis Imaging: Practice Essentials, Radiography, Computed Tomography

  https://emedicine.medscape.com/article/363273-overview

  Idiopathic pulmonary fibrosis (IPF) is a form of idiopathic interstitial pneumonia that is associated with a median survival of 2-5 years from the time of diagnosis. […] Treatment can delay the progression of IPF, so early diagnosis is fundamental. […] Diagnosis can be made without biopsy with compatible imaging tests, appropriate clinical history, and exclusion of other conditions. […] If diagnosis is uncertain, IPF can be diagnosed by lung biopsy. […] Idiopathic pulmonary fibrosis is diagnosed on the basis of patient history, clinical findings, pulmonary physiology, and imaging results. The diagnosis is one of exclusion. […] High-resolution CT (HRCT) is currently the method of choice for diagnosis of IPF. […] Studies have shown that HRCT may obviate surgical lung biopsy in some patients.

• #19 How Is Pulmonary Fibrosis Diagnosed? | American Lung Association

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

  Pulmonary fibrosis (PF) may be difficult to diagnose as the symptoms of PF are similar to other lung diseases. […] If your doctor suspects you might have PF, it is important to see a specialist to confirm your diagnosis. This will help ensure you are treated for the exact disease you have. […] A high-resolution computed tomography scan, or HRCT scan, is an X-ray that provides sharper and more detailed pictures than a standard chest X-ray and is an important component of diagnosing PF. […] If more sampling is required to make a diagnosis, your doctor might perform a biopsy.

• #20 Idiopathic Pulmonary Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448162/

  The diagnosis of IPF is primarily clinical, including identifying potential risk factors such as exposure to inhalation pollutants and manifestations of connective tissue diseases such as rheumatoid arthritis, rashes, or skin manifestations. However, various evaluations can assist in confirming the diagnosis. […] Although chest imaging is essential for diagnosis, chest x-rays lack the details necessary to confirm IPF. HRCT of the chest should be performed with axial cuts of 1.5 mm or less and acquired at 10 mm intervals for the diagnosis of ILD and IPF. The characteristic feature of HRCT is a UIP pattern, which, on imaging, is characterized by bilateral subpleural basal predominant honeycombing or traction bronchiectasis or bronchiectasis. […] The American Thoracic Society guidelines recommend considering surgical lung biopsy if the diagnosis of IPF cannot be confirmed through noninvasive methods, especially when clinical and radiological findings are incongruent with UIP, unless the patient is at high risk for complications.

• #21 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment

  https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/

  Multidisciplinary meetings remain the gold standard for ILD diagnosis, including IPF. […] A diagnosis of IPF requires definite exclusion of ILDs with known causes, such as drug-related ILD, fibrotic hypersensitivity pneumonitis (fHP), or connective tissue disease-associated ILD. […] Once other causes of ILD have been ruled out and/or IPF is suspected based on clinical suspicion, a combination of clinical and radiological features should be employed to determine the probability of IPF. […] A morphological pattern of usual interstitial pneumonia (UIP), either a radiological or a histopathological one, is required to establish an IPF diagnosis. […] HRCT has become central to the diagnosis of IPF. […] In other patterns, such as probable or indeterminate UIP, BAL or lung biopsy could be performed in order to improve diagnostic accuracy, although a probable UIP pattern in an appropriate clinical context of high suspicion of IPF is accepted by most thoracic/respiratory societies as diagnostic for IPF without biopsy.

• #22 Idiopathic Pulmonary Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448162/

  The diagnosis of IPF is primarily clinical, including identifying potential risk factors such as exposure to inhalation pollutants and manifestations of connective tissue diseases such as rheumatoid arthritis, rashes, or skin manifestations. However, various evaluations can assist in confirming the diagnosis. […] Although chest imaging is essential for diagnosis, chest x-rays lack the details necessary to confirm IPF. HRCT of the chest should be performed with axial cuts of 1.5 mm or less and acquired at 10 mm intervals for the diagnosis of ILD and IPF. The characteristic feature of HRCT is a UIP pattern, which, on imaging, is characterized by bilateral subpleural basal predominant honeycombing or traction bronchiectasis or bronchiectasis. […] The American Thoracic Society guidelines recommend considering surgical lung biopsy if the diagnosis of IPF cannot be confirmed through noninvasive methods, especially when clinical and radiological findings are incongruent with UIP, unless the patient is at high risk for complications.

• #23 Idiopathic Pulmonary Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448162/

  Idiopathic pulmonary fibrosis (IPF) is a progressive lung disorder characterized by scarring of the lungs from an unknown cause. Early diagnosis is pivotal for effective management, given the disease’s tendency to progress rapidly in advanced stages. Diagnosis can often be established without biopsy through a comprehensive assessment of clinical history, imaging results, and exclusion of alternative conditions. However, a lung biopsy may be required in cases where diagnostic uncertainty persists. […] Imaging techniques, particularly computed tomography scans, frequently display a distinctive pattern of fibrosis, typically observed at the periphery of both lungs and more prominently concentrated at the bases, aiding in the diagnosis of IPF. In cases of diagnostic uncertainty, a lung biopsy can confirm the diagnosis.

• #24 Idiopathic pulmonary fibrosis – Wikipedia

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

  The presence of a typical radiological pattern of usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT). […] In the right clinical setting, it is possible to make the diagnosis of IPF by HRCT alone, obviating the need for surgical lung biopsy. […] Various technologies using artificial intelligence have been developed to help with diagnosis. […] Recent developments indicate a potential benefit of positron emission tomography (PET) for diagnosis of IPF if suitable radiotracers are applied. […] The likely most important activator of TGF- is v6-integrin, which releases TGF- from its latent form. […] Hence, v6-integrin has been recognized earlier as a potential prognostic biomarker for IPF. […] The tracer 68Ga-Trivehexin showed an uptake of SUVmax = 5.53 in fibrotic lung tissue and enabled a differentiation between fibrotic and non-fibrotic lung areas in PET/CT images. […] In the evaluation of patients with suspected IPF, the most important application of BAL is in the exclusion of other diagnoses. […] The diagnosis of IIPs requires exclusion of known causes of ILD.

• #25 A Diagnostic Algorithm for Idiopathic Pulmonary Fibrosis

  https://consultqd.clevelandclinic.org/a-diagnostic-algorithm-for-idiopathic-pulmonary-fibrosis

  Idiopathic pulmonary fibrosis (IPF) is a devastating and fatal lung disease that generally affects older adults. Accurate diagnosis of IPF is crucial. […] The diagnosis of IPF requires exclusion of other known causes of UIP. […] If the pattern on HRCT is considered definite, evaluation of pathology is not necessary. If the pattern is categorized as possible or is inconsistent, then surgical lung biopsy-confirmed UIP is necessary for the definitive diagnosis of IPF. […] Given the multitude of ILDs, their complexities and the lack of a gold standard definitive diagnostic test, the diagnosis of IPF can be difficult, requiring the integration of clinical, radiologic and, if necessary, pathologic findings. […] Multiple pathologic processes can appear as UIP on imaging or lung biopsy, and these pathologic processes must be ruled out before diagnosing IPF.

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

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

  To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. […] During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs. […] A high-resolution CT scan can be helpful in diagnosing pulmonary fibrosis and in finding out how much lung damage has occurred. […] In addition to showing whether you have pulmonary fibrosis, imaging and lung function tests can be used to check your condition over time and see how treatments are working. […] If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed. This is called a biopsy. The biopsy sample is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. […] A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life.

• #27

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

  Idiopathic pulmonary fibrosis (IPF) can be hard to diagnose because its symptoms are similar to other lung conditions, such as chronic obstructive pulmonary disease (COPD). […] A GP can refer you to hospital specialists for a number of tests to help rule out other conditions and confirm the diagnosis. […] Lung function tests (also called pulmonary function tests) assess how well your lungs work and can help indicate what the problem may be. […] A commonly used lung function test is spirometry. During the test, you breathe into a mouthpiece that’s attached to a monitor. […] If IPF is suspected, the chest X-ray will be followed by a CT scan. […] This can help your doctor spot signs of scarring in your lungs. […] If doctors are still not sure what the problem is after these tests, they may suggest having a bronchoscopy. […] If other tests are not conclusive, a lung biopsy may need to be carried out. […] This involves keyhole surgery to remove a small sample of lung tissue so it can be analysed for signs of scarring.

• #28 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  The typical findings on pulmonary function tests in patients with idiopathic pulmonary fibrosis are a restrictive ventilatory defect and a reduced diffusion capacity for carbon monoxide. […] The 6MWT is a marker of functional exercise capacity that is being increasingly used in the initial and longitudinal clinical assessment of patients with idiopathic pulmonary fibrosis. […] Bronchoalveolar lavage (BAL) has been an immensely useful research tool in idiopathic pulmonary fibrosis. However, the role of BAL in the clinical diagnosis of idiopathic pulmonary fibrosis remains limited. […] A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias. […] The diagnosis of idiopathic pulmonary fibrosis now requires the following: The exclusion of other known causes of interstitial lung disease (ILD), including domestic and occupational environmental exposures, connective tissue disease, and drug toxicity; The presence of a UIP pattern on HRCT in patients not subjected to a surgical lung biopsy; Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

• #29 Pulmonary Fibrosis: Causes, Symptoms & Treatment

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

  Pulmonary fibrosis is scarring in your lungs. It can make it hard to expand your lungs and get enough oxygen to your body. […] Pulmonary fibrosis usually gets worse over time, but how quickly it gets worse is different for everyone. […] Pulmonary fibrosis (PF) is a scarring and thickening in your lungs that makes it hard to breathe deeply. […] Pulmonary fibrosis is permanent and usually gets progressively worse over time (called progressive pulmonary fibrosis). […] In most cases, the specific cause is unknown. […] It might take a number of tests and imaging procedures to diagnose pulmonary fibrosis. […] Your provider will listen to your lungs and get pulmonary function tests to understand how your lungs are working. […] Specific tests and procedures providers might use to diagnose pulmonary fibrosis include: Chest X-ray, High-resolution CT (HRCT) scan, Pulmonary (lung) function tests, Blood tests, including an arterial blood gas, Bronchoscopy, Biopsy.

• #30 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  The typical findings on pulmonary function tests in patients with idiopathic pulmonary fibrosis are a restrictive ventilatory defect and a reduced diffusion capacity for carbon monoxide. […] The 6MWT is a marker of functional exercise capacity that is being increasingly used in the initial and longitudinal clinical assessment of patients with idiopathic pulmonary fibrosis. […] Bronchoalveolar lavage (BAL) has been an immensely useful research tool in idiopathic pulmonary fibrosis. However, the role of BAL in the clinical diagnosis of idiopathic pulmonary fibrosis remains limited. […] A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias. […] The diagnosis of idiopathic pulmonary fibrosis now requires the following: The exclusion of other known causes of interstitial lung disease (ILD), including domestic and occupational environmental exposures, connective tissue disease, and drug toxicity; The presence of a UIP pattern on HRCT in patients not subjected to a surgical lung biopsy; Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

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

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

  To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. […] During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs. […] A high-resolution CT scan can be helpful in diagnosing pulmonary fibrosis and in finding out how much lung damage has occurred. […] In addition to showing whether you have pulmonary fibrosis, imaging and lung function tests can be used to check your condition over time and see how treatments are working. […] If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed. This is called a biopsy. The biopsy sample is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. […] A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life.

• #32 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  The typical findings on pulmonary function tests in patients with idiopathic pulmonary fibrosis are a restrictive ventilatory defect and a reduced diffusion capacity for carbon monoxide. […] The 6MWT is a marker of functional exercise capacity that is being increasingly used in the initial and longitudinal clinical assessment of patients with idiopathic pulmonary fibrosis. […] Bronchoalveolar lavage (BAL) has been an immensely useful research tool in idiopathic pulmonary fibrosis. However, the role of BAL in the clinical diagnosis of idiopathic pulmonary fibrosis remains limited. […] A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias. […] The diagnosis of idiopathic pulmonary fibrosis now requires the following: The exclusion of other known causes of interstitial lung disease (ILD), including domestic and occupational environmental exposures, connective tissue disease, and drug toxicity; The presence of a UIP pattern on HRCT in patients not subjected to a surgical lung biopsy; Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

• #33 Idiopathic pulmonary fibrosis – Wikipedia

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

  The presence of a typical radiological pattern of usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT). […] In the right clinical setting, it is possible to make the diagnosis of IPF by HRCT alone, obviating the need for surgical lung biopsy. […] Various technologies using artificial intelligence have been developed to help with diagnosis. […] Recent developments indicate a potential benefit of positron emission tomography (PET) for diagnosis of IPF if suitable radiotracers are applied. […] The likely most important activator of TGF- is v6-integrin, which releases TGF- from its latent form. […] Hence, v6-integrin has been recognized earlier as a potential prognostic biomarker for IPF. […] The tracer 68Ga-Trivehexin showed an uptake of SUVmax = 5.53 in fibrotic lung tissue and enabled a differentiation between fibrotic and non-fibrotic lung areas in PET/CT images. […] In the evaluation of patients with suspected IPF, the most important application of BAL is in the exclusion of other diagnoses. […] The diagnosis of IIPs requires exclusion of known causes of ILD.

• #34 Idiopathic Pulmonary Fibrosis Diagnosis

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

  Traditionally the diagnosis of IPF required a lung biopsy done by a surgeon under general anesthesia. In the past 20 years, we have learned that many patients can be confidently diagnosed based on a special type of CT scan of their chest. If this HRCT (high resolution computed tomography chest) shows the characteristic findings of scarring or honeycomb change at the bases in the correct distribution (along the periphery of the lung) then a biopsy is not needed. Unfortunately, many patients do not have the required findings on their scans and a lung biopsy is required. […] Unfortunately at this time there is not a specific blood test that can confirm the diagnosis of idiopathic pulmonary fibrosis. […] This technique is not acceptable or useful in making the diagnosis of IPF. Other diseases such as sarcoid and hypersensitivity pneumonitis may be diagnosed in this way but not IPF. If your lung doctor is most worried about IPF then transbrochial biopsy of the lung is not the best approach.

• #35 Frontiers | Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00151/full

  High-resolution computed tomography plays a pivotal role in disease diagnosis and determines the need of SLB to establish a definite diagnosis. […] Surgical lung biopsy in patients with ILDs as it can trigger an acute exacerbation, regardless disease severity. […] By strictly adhering to current guidelines, a large number of patients (almost 50% with a suspicion of IPF) will need to be subjected to SLB. […] Bronchoscopic lung cryobiopsy is dynamically emerging during the past few years as an alternative diagnostic tool to SLB, claiming the same diagnostic efficacy and reduced mortality. […] The ILD community has made significant progress in understanding IPF. With the development of antifibrotic agents, accurate diagnosis is crucial. Guidance is needed to focus on practical implementation of current guidelines in a real-world clinical setting.

• #36 Diagnosing Idiopathic Pulmonary Fibrosis | NYU Langone Health

  https://nyulangone.org/conditions/idiopathic-pulmonary-fibrosis/diagnosis

  Pulmonary function testing assesses lung function by measuring how much air your lungs can hold, how quickly you are able to inhale and exhale, and how much oxygen your lungs are able to deliver to the blood. […] If the results of imaging and lung function tests are inconclusive, your doctor may recommend a lung biopsy, or tissue sample. A biopsy can confirm idiopathic pulmonary fibrosis and rule out other conditions. […] Either type of lung biopsy can be used to help to rule out sarcoidosis, an inflammatory condition that affects multiple organs, including the lungs, as well as lung cancer and infection. A biopsy can also reveal how far idiopathic pulmonary fibrosis has progressed, which can help doctors create a treatment plan.

• #37 Idiopathic Pulmonary Fibrosis Diagnosis

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

  In contrast to a transbrochial biopsy, a surgical lung biopsy (often called VATS for video assisted thoracoscopic surgery) is done under general anesthesia. A surgeon inserts two small telescope-like ports into the chest. Through these ports, small instruments can then be used to obtain much larger pieces of tissue. Ideally 3 pieces from 2 lobes of the lung should be submitted.

• #38 Frontiers | Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00151/full

  High-resolution computed tomography plays a pivotal role in disease diagnosis and determines the need of SLB to establish a definite diagnosis. […] Surgical lung biopsy in patients with ILDs as it can trigger an acute exacerbation, regardless disease severity. […] By strictly adhering to current guidelines, a large number of patients (almost 50% with a suspicion of IPF) will need to be subjected to SLB. […] Bronchoscopic lung cryobiopsy is dynamically emerging during the past few years as an alternative diagnostic tool to SLB, claiming the same diagnostic efficacy and reduced mortality. […] The ILD community has made significant progress in understanding IPF. With the development of antifibrotic agents, accurate diagnosis is crucial. Guidance is needed to focus on practical implementation of current guidelines in a real-world clinical setting.

• #39 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Results from routine laboratory studies are nonspecific for the diagnosis of idiopathic pulmonary fibrosis; however, some laboratory studies may be helpful for ruling out other causes of interstitial lung disease. Reportedly, up to 30% of patients with idiopathic pulmonary fibrosis (IPF) have positive tests for antinuclear antibodies or rheumatoid factor; however, these titers are generally not high. […] The chest radiograph lacks diagnostic specificity for idiopathic pulmonary fibrosis. Virtually all patients with idiopathic pulmonary fibrosis (IPF) have an abnormal chest radiograph at the time of diagnosis. […] High-resolution computed tomography (HRCT) findings are significantly more sensitive and specific for the diagnosis of idiopathic pulmonary fibrosis and are an essential component of the diagnostic pathway of idiopathic pulmonary fibrosis.

• #40 Idiopathic pulmonary fibrosis – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/446

  Other diagnostic factors include weight loss, fatigue, malaise, and clubbing. […] 1st investigations to order include CXR, high-resolution CT (HRCT) chest, and pulmonary function tests. […] Investigations to consider include surgical lung biopsy, bronchoalveolar lavage (BAL), trans-bronchial lung biopsy and cryobiopsy, CRP, erythrocyte sedimentation rate (ESR), anti-nuclear antibody immunofluorescence, rheumatoid factor, anti-cyclic citrullinated peptide, and myositis panel.

• #41 Idiopathic pulmonary fibrosis – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/446

  Other diagnostic factors include weight loss, fatigue, malaise, and clubbing. […] 1st tests to order include CXR, high-resolution CT (HRCT) chest, and pulmonary function tests. […] Tests to consider include surgical lung biopsy, bronchoalveolar lavage (BAL), transbronchial lung biopsy and cryobiopsy, CRP, erythrocyte sedimentation rate (ESR), antinuclear antibody immunofluorescence, rheumatoid factor, anticyclic citrullinated peptide, and myositis panel.

• #42 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Results from routine laboratory studies are nonspecific for the diagnosis of idiopathic pulmonary fibrosis; however, some laboratory studies may be helpful for ruling out other causes of interstitial lung disease. Reportedly, up to 30% of patients with idiopathic pulmonary fibrosis (IPF) have positive tests for antinuclear antibodies or rheumatoid factor; however, these titers are generally not high. […] The chest radiograph lacks diagnostic specificity for idiopathic pulmonary fibrosis. Virtually all patients with idiopathic pulmonary fibrosis (IPF) have an abnormal chest radiograph at the time of diagnosis. […] High-resolution computed tomography (HRCT) findings are significantly more sensitive and specific for the diagnosis of idiopathic pulmonary fibrosis and are an essential component of the diagnostic pathway of idiopathic pulmonary fibrosis.

• #43 Idiopathic pulmonary fibrosis – Wikipedia

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

  An earlier diagnosis of IPF is a prerequisite for earlier treatment and, potentially, improvement of the long-term clinical outcome of this progressive and ultimately fatal disease. […] If IPF is suspected, diagnosis can be challenging but a multidisciplinary approach involving a pulmonologist, radiologist and pathologist expert in interstitial lung disease has been shown to improve the accuracy of IPF diagnosis. […] A Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS) in 2000 proposed specific major and minor criteria for establishing the diagnosis of IPF. […] Currently, a diagnosis of IPF requires: Exclusion of known causes of interstitial lung disease (ILD), e.g., domestic and occupational environmental exposures, connective tissue disorders, or drug exposure/toxicity.

• #44 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/pulms/evaluation-diagnosis-of-ILDs/diagnosis-of-ILDs

  Differential diagnosis of ILDs can be challenging due to overlapping clinical, radiological and pathological presentations. […] A MULTIDISCIPLINARY APPROACH INVOLVING A CLINICAL, RADIOLOGICAL AND PATHOLOGICAL REVIEW IS RECOMMENDED FOR EARLY AND ACCURATE ILD DIAGNOSIS. […] A multidisciplinary team including a pulmonologist, radiologist and histopathologist should be involved in ILD diagnosis. […] It is recommended that all collected diagnostic information be evaluated by a multidisciplinary team experienced in ILD, which may either establish a diagnosis or discuss the indication for further diagnostic procedures such as thoracoscopic lung biopsy or transbronchial cryobiopsy. […] The pulmonary fibrosis diagnosis algorithm includes a multidisciplinary team. […] The participants consider all data available and propose a provisional first-choice diagnosis, assess the need for biopsy and confidence in the diagnosis, and consider possible alternative diagnoses.

• #45 Diagnosis of Idiopathic Pulmonary Fibrosis

  https://www.hcplive.com/view/diagnosis-of-idiopathic-pulmonary-fibrosis

  The guidelines for the diagnostic process for idiopathic pulmonary fibrosis [IPF] for all of the interstitial lung diseases in general have revised over the years, and in large part have been driven by the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and the Latin American Society (LAS)those groups that have identified experts to come up with recommendations. […] A very prominent role for chest imaging, particularly high-resolution CT [computerized tomography] where theres been a tremendous amount of advance in understanding the patterns of abnormality that would be most likely to be associated with a final diagnosis of idiopathic pulmonary fibrosis. […] The pattern is the pattern of usual interstitial pneumonia. […] The most recent significant change has been to really codify the importance of a multidisciplinary discussion, whats called an MDD, as a format for ensuring in each step that were doing the right thing by the patient and their caregivers as we go through the diagnostic process.

• #46 Frontiers | Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00151/full

  It is important to note that disease diagnosis does not represent anymore an academic exercise since it directly influences and guides therapeutic decisions. […] According to current diagnostic criteria, HRCT plays a pivotal role in the diagnostic procedure. There are three diagnostic categories based on HRCT appearance: UIP pattern, possible UIP pattern, and inconsistent with UIP. […] In the appropriate clinical setting in patients with a UIP pattern, IPF diagnosis can be established without the need for surgical lung biopsy (SLB). […] MDT approach is acknowledged as a major advance in IPF diagnosis. It refers to the constructive exchange of views between a respiratory physician, radiologist, and pathologist with expertise in the field of ILDs. […] The 2011 guidelines are a clear step forward considering that they provide clear guidance on an evidence-based approach.

• #47 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/pulms/evaluation-diagnosis-of-ILDs/diagnosis-of-ILDs

  Differential diagnosis of ILDs can be challenging due to overlapping clinical, radiological and pathological presentations. […] A MULTIDISCIPLINARY APPROACH INVOLVING A CLINICAL, RADIOLOGICAL AND PATHOLOGICAL REVIEW IS RECOMMENDED FOR EARLY AND ACCURATE ILD DIAGNOSIS. […] A multidisciplinary team including a pulmonologist, radiologist and histopathologist should be involved in ILD diagnosis. […] It is recommended that all collected diagnostic information be evaluated by a multidisciplinary team experienced in ILD, which may either establish a diagnosis or discuss the indication for further diagnostic procedures such as thoracoscopic lung biopsy or transbronchial cryobiopsy. […] The pulmonary fibrosis diagnosis algorithm includes a multidisciplinary team. […] The participants consider all data available and propose a provisional first-choice diagnosis, assess the need for biopsy and confidence in the diagnosis, and consider possible alternative diagnoses.

• #48 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/pulms/evaluation-diagnosis-of-ILDs/diagnosis-of-ILDs

  HRCT is the gold standard diagnostic tool for ILDs. […] In the absence of other known causes, HRCT, in discussion with an MDT, is necessary to confirm an IPF diagnosis. […] MDTs reach an IPF diagnosis more frequently and with higher confidence than individual clinicians. […] Diagnosis of IPF is confirmed from either HRCT, BAL or surgical lung biopsy. […] If a specific diagnosis is not made or no potential cause for ILD is identified, then clinical findings and appropriate combination of HRCT and histopathological patterns are considered during multidisciplinary discussion to either ascertain or exclude the diagnosis of IPF. […] Patients clinically suspected of having IPF are those with unexplained symptomatic or asymptomatic patterns of bilateral pulmonary fibrosis on a chest radiograph or chest computed tomography, bibasilar inspiratory crackles, and age greater than 60 years.

• #49 Idiopathic Pulmonary Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448162/

  Idiopathic pulmonary fibrosis (IPF) is a progressive lung disorder characterized by scarring of the lungs from an unknown cause. Early diagnosis is pivotal for effective management, given the disease’s tendency to progress rapidly in advanced stages. Diagnosis can often be established without biopsy through a comprehensive assessment of clinical history, imaging results, and exclusion of alternative conditions. However, a lung biopsy may be required in cases where diagnostic uncertainty persists. […] Imaging techniques, particularly computed tomography scans, frequently display a distinctive pattern of fibrosis, typically observed at the periphery of both lungs and more prominently concentrated at the bases, aiding in the diagnosis of IPF. In cases of diagnostic uncertainty, a lung biopsy can confirm the diagnosis.

• #50 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  The typical findings on pulmonary function tests in patients with idiopathic pulmonary fibrosis are a restrictive ventilatory defect and a reduced diffusion capacity for carbon monoxide. […] The 6MWT is a marker of functional exercise capacity that is being increasingly used in the initial and longitudinal clinical assessment of patients with idiopathic pulmonary fibrosis. […] Bronchoalveolar lavage (BAL) has been an immensely useful research tool in idiopathic pulmonary fibrosis. However, the role of BAL in the clinical diagnosis of idiopathic pulmonary fibrosis remains limited. […] A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias. […] The diagnosis of idiopathic pulmonary fibrosis now requires the following: The exclusion of other known causes of interstitial lung disease (ILD), including domestic and occupational environmental exposures, connective tissue disease, and drug toxicity; The presence of a UIP pattern on HRCT in patients not subjected to a surgical lung biopsy; Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

• #51 Idiopathic pulmonary fibrosis – Wikipedia

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

  An earlier diagnosis of IPF is a prerequisite for earlier treatment and, potentially, improvement of the long-term clinical outcome of this progressive and ultimately fatal disease. […] If IPF is suspected, diagnosis can be challenging but a multidisciplinary approach involving a pulmonologist, radiologist and pathologist expert in interstitial lung disease has been shown to improve the accuracy of IPF diagnosis. […] A Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS) in 2000 proposed specific major and minor criteria for establishing the diagnosis of IPF. […] Currently, a diagnosis of IPF requires: Exclusion of known causes of interstitial lung disease (ILD), e.g., domestic and occupational environmental exposures, connective tissue disorders, or drug exposure/toxicity.

• #52 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  The typical findings on pulmonary function tests in patients with idiopathic pulmonary fibrosis are a restrictive ventilatory defect and a reduced diffusion capacity for carbon monoxide. […] The 6MWT is a marker of functional exercise capacity that is being increasingly used in the initial and longitudinal clinical assessment of patients with idiopathic pulmonary fibrosis. […] Bronchoalveolar lavage (BAL) has been an immensely useful research tool in idiopathic pulmonary fibrosis. However, the role of BAL in the clinical diagnosis of idiopathic pulmonary fibrosis remains limited. […] A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias. […] The diagnosis of idiopathic pulmonary fibrosis now requires the following: The exclusion of other known causes of interstitial lung disease (ILD), including domestic and occupational environmental exposures, connective tissue disease, and drug toxicity; The presence of a UIP pattern on HRCT in patients not subjected to a surgical lung biopsy; Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

• #53 Idiopathic Pulmonary Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448162/

  The diagnosis of IPF is primarily clinical, including identifying potential risk factors such as exposure to inhalation pollutants and manifestations of connective tissue diseases such as rheumatoid arthritis, rashes, or skin manifestations. However, various evaluations can assist in confirming the diagnosis. […] Although chest imaging is essential for diagnosis, chest x-rays lack the details necessary to confirm IPF. HRCT of the chest should be performed with axial cuts of 1.5 mm or less and acquired at 10 mm intervals for the diagnosis of ILD and IPF. The characteristic feature of HRCT is a UIP pattern, which, on imaging, is characterized by bilateral subpleural basal predominant honeycombing or traction bronchiectasis or bronchiectasis. […] The American Thoracic Society guidelines recommend considering surgical lung biopsy if the diagnosis of IPF cannot be confirmed through noninvasive methods, especially when clinical and radiological findings are incongruent with UIP, unless the patient is at high risk for complications.

• #54 Frontiers | Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00151/full

  Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice” […] The past few years have signaled a major breakthrough on the management of idiopathic pulmonary fibrosis (IPF). Finally, we have drugs in our arsenal able to slow down the inexorable disease natural course. On the other hand, the latter evidence has increased the responsibility for a timely and accurate diagnosis. Establishment of IPF diagnosis directly affects the choice of appropriate treatment. […] The current diagnostic guidelines represent a major step forward providing an evidence-based road map; yet, clinicians are encountering major diagnostic dilemmas that inevitably affect therapeutic decisions. This review article aims to summarize the current state of knowledge on the diagnostic procedure of IPF based on the current guidelines and discuss pragmatic difficulties and challenges encountered by clinicians with regards to their applicability in the everyday clinical practice.

• #55 Frontiers | Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00151/full

  Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice” […] The past few years have signaled a major breakthrough on the management of idiopathic pulmonary fibrosis (IPF). Finally, we have drugs in our arsenal able to slow down the inexorable disease natural course. On the other hand, the latter evidence has increased the responsibility for a timely and accurate diagnosis. Establishment of IPF diagnosis directly affects the choice of appropriate treatment. […] The current diagnostic guidelines represent a major step forward providing an evidence-based road map; yet, clinicians are encountering major diagnostic dilemmas that inevitably affect therapeutic decisions. This review article aims to summarize the current state of knowledge on the diagnostic procedure of IPF based on the current guidelines and discuss pragmatic difficulties and challenges encountered by clinicians with regards to their applicability in the everyday clinical practice.

• #56 Frontiers | Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00151/full

  High-resolution computed tomography plays a pivotal role in disease diagnosis and determines the need of SLB to establish a definite diagnosis. […] Surgical lung biopsy in patients with ILDs as it can trigger an acute exacerbation, regardless disease severity. […] By strictly adhering to current guidelines, a large number of patients (almost 50% with a suspicion of IPF) will need to be subjected to SLB. […] Bronchoscopic lung cryobiopsy is dynamically emerging during the past few years as an alternative diagnostic tool to SLB, claiming the same diagnostic efficacy and reduced mortality. […] The ILD community has made significant progress in understanding IPF. With the development of antifibrotic agents, accurate diagnosis is crucial. Guidance is needed to focus on practical implementation of current guidelines in a real-world clinical setting.

• #57 Frontiers | Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00151/full

  High-resolution computed tomography plays a pivotal role in disease diagnosis and determines the need of SLB to establish a definite diagnosis. […] Surgical lung biopsy in patients with ILDs as it can trigger an acute exacerbation, regardless disease severity. […] By strictly adhering to current guidelines, a large number of patients (almost 50% with a suspicion of IPF) will need to be subjected to SLB. […] Bronchoscopic lung cryobiopsy is dynamically emerging during the past few years as an alternative diagnostic tool to SLB, claiming the same diagnostic efficacy and reduced mortality. […] The ILD community has made significant progress in understanding IPF. With the development of antifibrotic agents, accurate diagnosis is crucial. Guidance is needed to focus on practical implementation of current guidelines in a real-world clinical setting.

• #58

  https://link.springer.com/article/10.1007/s41030-023-00216-0

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. […] Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. […] Early diagnosis of IPF is crucial to ensure timely treatment selection and improve outcomes. […] Early diagnosis can be enhanced by improving awareness among primary care physicians, lung cancer screening programmes and the use of artificial intelligence (AI) systems to analyse computed tomography (CT) images and pulmonary function test results. […] In diagnosing and evaluating patients with IPF, HRCT plays a crucial role. […] The current standard of visually assessing HRCT scans to determine IPF disease extent is hindered by inter-observer variation with poor reproducibility.

• #59 Pulmonary Fibrosis: Diagnosis and Treatment | Consultant360

  https://www.consultant360.com/articles/pulmonary-fibrosis-diagnosis-and-treatment

  Several scoring systems and biomarkers are being developed to identify patients at high risk of progression. […] The 2011 guidelines identify baseline and longitudinal risk factors associated with increased risk of progression and higher mortality in patients with IPF. […] The majority of patients with IPF have some sort of exercise intolerance due to altered respiratory mechanics, impaired gas exchange, peripheral muscle dysfunction, and circulatory limitation. […] Once the diagnosis is made, the physician should spend adequate time with the patient to explain the prognosis and assess the patient’s preferences and values. […] The burden of the disease is emotionally overwhelming for the patients and the ramification of having a morbid disease will likely impact family members as well.

• #60 New guidelines for diagnosis of Idiopathic pulmonary fibrosis – ERS – European Respiratory Society

  https://www.ersnet.org/news-and-features/news/new-guidelines-for-diagnosis-of-idiopathic-pulmonary-fibrosis/

  In contrast, for patients with newly detected ILD who have a HRCT pattern of UIP, strong recommendations were made against performing SLB, TBBx and lung cryobiopsy; a conditional recommendation was made against performing BAL. […] Strong recommendation against measurement of serum biomarkers for the sole purpose of distinguishing IPF from other ILDs.

• #61 Idiopathic pulmonary fibrosis – Wikipedia

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

  The presence of a typical radiological pattern of usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT). […] In the right clinical setting, it is possible to make the diagnosis of IPF by HRCT alone, obviating the need for surgical lung biopsy. […] Various technologies using artificial intelligence have been developed to help with diagnosis. […] Recent developments indicate a potential benefit of positron emission tomography (PET) for diagnosis of IPF if suitable radiotracers are applied. […] The likely most important activator of TGF- is v6-integrin, which releases TGF- from its latent form. […] Hence, v6-integrin has been recognized earlier as a potential prognostic biomarker for IPF. […] The tracer 68Ga-Trivehexin showed an uptake of SUVmax = 5.53 in fibrotic lung tissue and enabled a differentiation between fibrotic and non-fibrotic lung areas in PET/CT images. […] In the evaluation of patients with suspected IPF, the most important application of BAL is in the exclusion of other diagnoses. […] The diagnosis of IIPs requires exclusion of known causes of ILD.

• #62

  https://link.springer.com/article/10.1007/s41030-023-00216-0

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. […] Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. […] Early diagnosis of IPF is crucial to ensure timely treatment selection and improve outcomes. […] Early diagnosis can be enhanced by improving awareness among primary care physicians, lung cancer screening programmes and the use of artificial intelligence (AI) systems to analyse computed tomography (CT) images and pulmonary function test results. […] In diagnosing and evaluating patients with IPF, HRCT plays a crucial role. […] The current standard of visually assessing HRCT scans to determine IPF disease extent is hindered by inter-observer variation with poor reproducibility.

• #63 Researchers develop screening tool to aid early diagnosis of idiopathic pulmonary fibrosis | Biological Sciences Division | The University of Chicago

  https://biologicalsciences.uchicago.edu/news/idiopathic-pulmonary-fibrosis-screening-algoritm

  The scanning tool had an 88 percent accuracy rate for scanning one year before conventional diagnosis and 85 percent for four years. […] It seems to be a very accurate screening test. […] Next, the team would like to see the screening tool implemented in primary care centers to test it in real-world settings. […] This approach is a paradigm shift in the screening for IPF. […] If we could screen for these diseases by using existing electronic health records without the need for any new tests, that could have a major impact on health care.

• #64 Automatic diagnostic support for diagnosis of pulmonary fibrosis | medRxiv

  https://www.medrxiv.org/content/10.1101/2024.08.14.24312012v1

  Patients with pulmonary fibrosis (PF) often experience long waits before getting a correct diagnosis, and this delay in reaching specialized care is associated with increased mortality, regardless of the severity of the disease. […] Early diagnosis and timely treatment of PF can potentially extend life expectancy and maintain a better quality of life. […] This paper describes an automated system for differentiating lung sounds related to PF from other pathological lung conditions using the average number of crackles per breath cycle (NOC/BC). […] The overall performance of the automatic classifier based on receiver operating curve-derived cut-off value for average NOC/BC of 18.65 (AUC=0.845, 95 % CI 0.739-0.952, p0.001; sensitivity=91.7 %; specificity=59.3 %) compares favorably with the averaged performance of the physicians (sensitivity=83.3 %; specificity=56.25 %).

• #65 Automatic diagnostic support for diagnosis of pulmonary fibrosis | medRxiv

  https://www.medrxiv.org/content/10.1101/2024.08.14.24312012v1

  Although radiological assessment should remain the gold standard for diagnosis of fibrotic interstitial lung disease, the automatic classification system has strong potential for diagnostic support, especially in assisting general practitioners in the auscultatory assessment of lung sounds to prompt further diagnostic work up of patients with suspect of interstitial lung disease.

• #66 CHEST Annual Meeting

  https://www.healio.com/news/pulmonology/20221025/time-lost-is-lung-lost-early-diagnosis-critical-in-pulmonary-fibrosis

  From the time of symptom onset to a pulmonologist evaluation or evaluation at a specialized ILD center, several tests are required, including pulmonary function tests, radiological assessment with chest X-ray and high-resolution CT scans and, based on these results, sometimes surgical lung biopsies and histopathological review for a more definitive diagnosis. […] Diagnosis and management of pulmonary fibrosis is complex, but a comprehensive management of these patients is of utmost importance because only a few therapies exist in the current landscape for the various ILDs, especially IPF. […] Although there are efforts being made to develop treatments that not only slow down disease progression but perhaps even cure or aid in the reversal of fibrosis as well as improve quality of life among patients with pulmonary fibrosis, ultimately, early diagnosis will remain the key to improving outcomes among patients with pulmonary fibrosis.

• #67 Delayed Diagnosis | ILD Collaborative

  https://www.ildcollaborative.org/resources/ipf-patient-journey/delayed-diagnosis

  Many people with idiopathic pulmonary fibrosis (IPF) experience a long journey before they are correctly diagnosed. Right now, the average delay in the United States between the start of a persons earliest symptoms of breathlessness and accurate diagnosis of IPF is a little over two years. However, an accurate diagnosis can take as long as four or five years. […] A survey study of 600 people with IPF found that over half (55%) had at least one wrong diagnosis before they were correctly diagnosed. […] The longer the delay in correct diagnosis, the more risk that a person will die from complications of IPF in the five years after diagnosis. Early treatment is better for survival. […] IPF is difficult for primary care doctors to recognize in the early stages for two reasons. First, IPF is very rare. Because IPF is a rare disease, some primary care doctors have never seen a patient with IPF.

• #68

  https://link.springer.com/article/10.1007/s41030-023-00216-0

  The 2018 ATS/ERS/JRS/ALAT evidence-based guidelines for IPF diagnosis suggested a multidisciplinary discussion for the diagnosis of IPF. […] The critical role that primary care physicians play in the early diagnosis of IPF highlights the need for educational intervention to raise awareness of ILD in this setting. […] An early diagnosis of IPF may lead to earlier treatment with anti-fibrotic medications and even though individual clinical trials were not sufficiently powered to demonstrate significant effects on acute exacerbations and mortality, evidence is growing supporting the effects of pirfenidone and nintedanib in decreasing the risk of acute decline in lung function and improving life expectancy by slowing the progression rate of IPF. […] A delay of this magnitude would have a significant impact on the number of patients receiving anti-fibrotic medications. […] These studies highlight the importance of early diagnosis to allow proper management of patients with idiopathic pulmonary fibrosis.

• #69 Researchers develop screening tool to aid early diagnosis of idiopathic pulmonary fibrosis | Biological Sciences Division | The University of Chicago

  https://biologicalsciences.uchicago.edu/news/idiopathic-pulmonary-fibrosis-screening-algoritm

  The team took these factors into consideration, as well as the time constraint faced by primary care physicians, when developing the screening tool. […] This tool takes literally zero additional minutes and can recognize features of the disease before symptoms manifest. […] Early detection plays a pivotal role in the effective treatment of IPF. […] Currently, the diagnosis of IPF is delayed approximately three years from the onset of symptoms. […] This new screening tool will help identify patients at an earlier stage of the disease so they can receive FDA approved treatments as well as be eligible for clinical trial therapies for this progressive disease. […] The algorithm behind the screening tool works by tabulating various data points in a patients existing medical records, such as any diagnoses from past medical encounters and medications prescribed and uses a probabilistic framework to determine the likelihood that the individual could develop the disease.

• #70 Diagnosis of Idiopathic Pulmonary Fibrosis

  https://www.hcplive.com/view/diagnosis-of-idiopathic-pulmonary-fibrosis

  IPF can be diagnosed early, or it can be diagnosed late. […] Now, sometimes patients can go misdiagnosed. […] Some patients can present for the first time with an acute exacerbation of their underlying IPF, and some patients will only come to attention once they have an IPF acute exacerbation, which would then automatically put them late in their disease course because the prognosis of acute exacerbations tends to be rather poor. […] This does impact how we manage them. […] Some of the patients who present late might still be transplant candidates, but its always better to work them up for transplant earlier in their disease course rather than trying to work them up later in their disease course where they might have limited rehab potential or might have comorbidities that take a longer period of time to address, such as obesity.

• #71 Pulmonary Fibrosis: Diagnosis and Treatment | Consultant360

  https://www.consultant360.com/articles/pulmonary-fibrosis-diagnosis-and-treatment

  Several scoring systems and biomarkers are being developed to identify patients at high risk of progression. […] The 2011 guidelines identify baseline and longitudinal risk factors associated with increased risk of progression and higher mortality in patients with IPF. […] The majority of patients with IPF have some sort of exercise intolerance due to altered respiratory mechanics, impaired gas exchange, peripheral muscle dysfunction, and circulatory limitation. […] Once the diagnosis is made, the physician should spend adequate time with the patient to explain the prognosis and assess the patient’s preferences and values. […] The burden of the disease is emotionally overwhelming for the patients and the ramification of having a morbid disease will likely impact family members as well.

• #72 Idiopathic Pulmonary Fibrosis | AAFP

  https://www.aafp.org/family-physician/patient-care/care-resources/respiratory-health/idiopathic-pulmonary-fibrosis.html

  MDD enhances the accuracy of diagnosis, with family physicians playing a vital role through early detection and timely referral. […] Diagnosing IPF requires not only knowledge of the signs and symptoms of IPF, but also the ability to distinguish it from other diseases with similar clinical presentations. […] Guidelines are available to help primary care physicians identify patients with IPF and make earlier referrals. Early referral means earlier treatment, which can help improve patient outcomes.

• #73 Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  The typical findings on pulmonary function tests in patients with idiopathic pulmonary fibrosis are a restrictive ventilatory defect and a reduced diffusion capacity for carbon monoxide. […] The 6MWT is a marker of functional exercise capacity that is being increasingly used in the initial and longitudinal clinical assessment of patients with idiopathic pulmonary fibrosis. […] Bronchoalveolar lavage (BAL) has been an immensely useful research tool in idiopathic pulmonary fibrosis. However, the role of BAL in the clinical diagnosis of idiopathic pulmonary fibrosis remains limited. […] A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias. […] The diagnosis of idiopathic pulmonary fibrosis now requires the following: The exclusion of other known causes of interstitial lung disease (ILD), including domestic and occupational environmental exposures, connective tissue disease, and drug toxicity; The presence of a UIP pattern on HRCT in patients not subjected to a surgical lung biopsy; Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

• #74 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment

  https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/

  Therefore, a provisional diagnosis with higher or lower confidence is acceptable in many practical clinical scenarios; however, the pursuit of alternative diagnosis should be restless. […] In summary, the diagnostic criteria include the exclusion of alternative diagnosis of ILD (extensively investigated) and a UIP pattern on HRCT and/or lung biopsy or a combination of HRCT and/or histological patterns.

• #75 CHEST Annual Meeting

  https://www.healio.com/news/pulmonology/20221025/time-lost-is-lung-lost-early-diagnosis-critical-in-pulmonary-fibrosis

  From the time of symptom onset to a pulmonologist evaluation or evaluation at a specialized ILD center, several tests are required, including pulmonary function tests, radiological assessment with chest X-ray and high-resolution CT scans and, based on these results, sometimes surgical lung biopsies and histopathological review for a more definitive diagnosis. […] Diagnosis and management of pulmonary fibrosis is complex, but a comprehensive management of these patients is of utmost importance because only a few therapies exist in the current landscape for the various ILDs, especially IPF. […] Although there are efforts being made to develop treatments that not only slow down disease progression but perhaps even cure or aid in the reversal of fibrosis as well as improve quality of life among patients with pulmonary fibrosis, ultimately, early diagnosis will remain the key to improving outcomes among patients with pulmonary fibrosis.

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