Materiały źródłowe

• #1 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 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.

• #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.

• #1 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. […] 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.

• #1 Idiopathic pulmonary fibrosis: What primary care physicians need to know | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/85/5/377

  Idiopathic pulmonary fibrosis (IPF) is a specific type of fibrosing interstitial pneumonia of unknown cause. It is usually chronic and progressive, tends to affect mainly adults over age 60, has a predilection for men, and is often fatal. […] Accurate diagnosis of IPF is crucial. We recommend early referral to a center specializing in interstitial lung disease to confirm the diagnosis, start appropriate therapy, advise the patient on prognosis and enrollment in disease registries and clinical trials, and determine candidacy for lung transplant. […] The diagnosis of IPF can be difficult, requiring the integration of clinical, radiologic, and, if necessary, pathologic findings. […] The diagnosis of IPF requires exclusion of other known causes of UIP. […] A final diagnosis of IPF can be made if the histologic pattern is definite, probable, or possible UIP, and if the multidisciplinary team (MDD) concurs that IPF is the most likely diagnosis.

• #1 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. […] Idiopathic means that your doctors cannot determine a cause of your disease at the time of diagnosis. […] 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. […] Your doctor may look for signs of IPF during a physical exam, such as: […] 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.

• #1 IPF Diagnosis | ILD Collaborative

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

  The journey to a correct diagnosis of idiopathic pulmonary fibrosis nearly always begins at the primary care doctors office. Although idiopathic pulmonary fibrosis is difficult to diagnose, there is a key sign that a primary care doctor can recognize during a physical examination: Velcro crackles. When a person with IPF breathes in, very often a doctor can hear an unusual crackling sound through the stethoscope. This sound is similar to the sound heard when gently separating the joined strip of Velcro on the blood pressure cuff. These are usually heard near the bottom part of the chest. The Velcro crackles can be heard the whole time a person with IPF breathes in. […] If Velcro crackles are heard at two examinations that are several weeks apart, this is a strong clue that the patient should be referred to a lung specialist (pulmonologist) for more tests. This is especially true if the patient is 50 years old or more and has a history of smoking.

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

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

  Key diagnostic factors include the presence of risk factors, dyspnoea, cough, and crackles. […] 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.

• #1 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 diagnosis of idiopathic pulmonary fibrosis relies on the clinician to integrate and correlate the clinical, laboratory, radiologic, and/or pathologic data. […] The diagnosis of IPF requires the following: Exclusion of known causes of interstitial lung disease, Presence of the high-resolution computed tomography (HRCT) pattern of Typical or Probable UIP, Specific combinations of HRCT patterns and histopathology patterns in patients subjected to lung tissue sampling, Multi-Displinary discussion between Pulmonary, Radiology and Pathology specialists. […] The diagnosis of idiopathic pulmonary fibrosis relies on the multi-discplinary integration of the clinical, laboratory, radiologic and/or pathologic data to make a clinical-radiologic-pathologic correlation that supports the diagnosis of idiopathic pulmonary fibrosis.

• #1 A Diagnostic Algorithm for Idiopathic Pulmonary Fibrosis

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

  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. […] The guidelines include an algorithm as an aid to the systematic evaluation. […] A thorough and accurate medical history should include assessment of the severity of dyspnea and cough, signs and symptoms of connective tissue disease […] The physical examination must include careful auscultation for rales. […] Laboratory testing should include a serologic autoantibody panel to evaluate for connective tissue diseases that can manifest as ILD.

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

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

  A morphological pattern of usual interstitial pneumonia (UIP), either a radiological or a histopathological one, is required to establish an IPF diagnosis. On the other hand, a UIP pattern has been associated with other conditions, such as asbestosis, fHP, and rheumatoid arthritis. Therefore, the exclusion of alternative diagnoses remains central, even with a typical UIP pattern on HRCT. […] HRCT has become central to the diagnosis of IPF. The radiological appearance of UIP (or typical UIP) has a strong correlation with histological UIP, precluding the need for invasive procedures. 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, and some patients might be unsuitable for invasive procedures. […] 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.

• #1 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.

• #1 Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-guidelines-for-diagnosis-treatment-idiopathic-articulo-S1579212913001067

  HRCT represents possibly the greatest diagnostic advance of the last two decades in the study of diffuse lung diseases. […] The official 2011 ATS/ERS/JRS/ALAT consensus established that in HRCT, the definite diagnosis of UIP is based on the identification of four typical findings: (1) lung involvement should have subpleural and basal predominance, (2) presence of obvious reticulation, (3) existence of honeycombing with/without traction bronchiectasis/bronchiolectasis and (4) demonstrate the absence of findings considered to exclude a UIP pattern. […] If the HRCT does not show a definitive pattern typical of UIP, the definitive diagnosis should be made by surgical lung biopsy. […] The integration of the HRCT findings with the histological pattern is used to establish the diagnosis of IPF, exclude it or, if the data are inconclusive, maintain it as probable or possible.

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

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

  Idiopathic pulmonary fibrosis (IPF) is a clinical syndrome and considered the most common and the most lethal form of pulmonary fibrosis corresponding to the histologic and imaging pattern of usual interstitial pneumonia. It is more common in elderly men and diagnosed by: […] A multidisciplinary approach in a tertiary setting is strongly advised. Contributions from pulmonologists, chest radiologists, and chest pathologists are crucial in reaching the correct diagnosis of IPF. […] In 2000, the American Thoracic Society (ATS) and European Respiratory Society (ERS) jointly agreed major and minor criteria for the diagnosis of IPF in the absence of a surgical lung biopsy. […] In 2018, these criteria were revised in a collaborative statement by the ATS, ERS, Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT). The major and minor criteria were eliminated and only the following diagnostic criteria remain: […] The presence of a UIP pattern on HRCT in patients not subjected to surgical lung biopsy (diagnostic categories of UIP pattern based on HRCT chest – Fleischner Society guideline 2018). […] Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy.

• #1 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 no definite cause of the interstitial lung abnormality is found, the level of certainty of the histopathologic pattern of UIP helps formulate the clinical diagnosis and management plan. […] 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. […] However, evidence is emerging that in the correct clinical scenario, possible UIP behaves similarly to definite UIP and may be sufficient to make the clinical diagnosis of IPF even without surgical biopsy confirmation.

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

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

  Diagnosis is based on history, physical examination, high-resolution CT, and/or lung biopsy, if necessary. […] Diagnosis of idiopathic pulmonary fibrosis is suspected in patients with subacute dyspnea, nonproductive cough, and Velcro crackles on chest examination. However, IPF is commonly overlooked initially because of clinical similarities to other more common diseases, such as bronchitis, asthma, and heart failure. […] Diagnosis requires HRCT and in some cases lung biopsy. […] Chest x-ray typically shows diffuse reticular opacities in the lower and peripheral lung zones. Small cystic lesions (honeycombing) and dilated airways due to traction bronchiectasis are additional findings. […] HRCT shows diffuse, patchy, subpleural, reticular opacities with irregularly thickened interlobular septa and intralobular lines; subpleural honeycombing; and traction bronchiectasis. This is referred to as the usual interstitial pneumonia (UIP) pattern. Ground-glass opacities affecting 30% of the lung suggest an alternative diagnosis. […] Laboratory testing plays little role in diagnosis, except to exclude potential systemic rheumatic disorders.

• #1 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. […] You may have one or more of these tests. […] 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. […] Nintedanib also is approved for other types of pulmonary fibrosis that get worse quickly. These medicines may help slow the worsening of pulmonary fibrosis and may prevent bouts when symptoms suddenly get worse.

• #1 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 specimen can be obtained through either an open lung biopsy or video-assisted thoracoscopic surgery (VATS). A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias.

• #1 Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-guidelines-for-diagnosis-treatment-idiopathic-articulo-S1579212913001067

  Multidisciplinary assessment in which pulmonologists, radiologists and pathologists experienced in the diagnosis and management of DILD can increase the diagnostic accuracy, which at present is a widely accepted recommendation for establishing the diagnosis. […] The clinical presentation of IPF has an insidious onset, and is usually characterized by progressive dyspnea on exertion, often accompanied by non-productive cough. […] There are no specific laboratory abnormalities for this disease. However, even in the absence of specific signs or symptoms of connective tissue diseases, serological autoimmune tests should be performed in all patients. […] The last international consensus indicated that BAL cellular analysis should not be performed systematically in all patients in the diagnostic procedure, although it may be appropriate for a minority.

• #1 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.

• #1 Diagnostic and prognostic implications of 2018 guideline for the diagnosis of idiopathic pulmonary fibrosis in clinical practice | Scientific Reports

  https://www.nature.com/articles/s41598-021-95728-7

  The purpose of this study was to evaluate the implications of the 2018 updated guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) in clinical practice compared to 2011 guideline. […] The 2018 criteria provide better prognostic stratification than the 2011 in patients with possible UIP. […] BAL fluid analysis can improve the diagnostic certainty for IPF diagnosis in patients with probable UIP CT pattern. […] Diagnostic guidelines for IPF were updated in 2018 by the American Thoracic Society (ATS), the European Respiratory Society (ERS), the Japanese Respiratory Society (JRS), and the Latin American Thoracic Society (ALAT). […] The latest ARS/ERS/JRS/ALAT guideline recommend cellular analysis of BAL fluid for patients with newly detected ILD of unknown cause who are clinically suspected of having IPF and have an HRCT pattern of probable UIP, indeterminate for UIP or an alternative diagnosis. […] Adding BAL fluid results can improve the diagnostic certainty for IPF diagnosis in patients with probable UIP CT patterns.

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

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

  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. […] The histopathological lesion associated with idiopathic pulmonary fibrosis is usual interstitial pneumonia. Usual interstitial pneumonia is characterized by a heterogeneous, variegated appearance with alternating areas of healthy lung, interstitial inflammation, fibrosis, and honeycomb change, which results in a patchwork appearance at low magnification. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

• #1 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

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

  A MULTIDISCIPLINARY APPROACH INVOLVING A CLINICAL, RADIOLOGICAL AND PATHOLOGICAL REVIEW IS RECOMMENDED FOR EARLY AND ACCURATE 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. […] 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.

• #1 Diagnosis of Idiopathic Pulmonary Fibrosis: Differential Diagnosis

  https://www.mdpi.com/2076-3271/6/3/73

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrotic interstitial lung disease of unknown origin with a characteristic imaging and histologic pattern called usual interstitial pneumonia (UIP). The diagnosis of IPF is a complex procedure that requires the support of various specialists, who must integrate clinical, radiological, and histological data. […] The multidisciplinary team (MDT) has become the new gold standard to diagnose and manage the disease, increasing the accuracy and agreement of the diagnosis between different centers. It is mandatory to exclude nonspecific interstitial pneumonia or other diseases that can cause the UIP pattern, particularly drugs or exposure diseases, including chronic hypersensitivity pneumonitis or systemic autoimmune disease. […] The diagnosis of IPF is a complex procedure that requires the support of various specialists, who must integrate clinical, radiological, and histological data, sometimes with uncertain or conflicting information. Therefore, such multidisciplinary teams (MDTs) have become the new gold standard to diagnose and manage the disease.

• #1 Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis | Pulmonology

  https://www.journalpulmonology.org/en-consensus-document-for-diagnosis-treatment-articulo-S2173511516000130

  Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. […] The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an internationally accepted recommendation. […] The diagnosis of IPF requires a multidisciplinary approach, including the awareness of general practice physicians, and experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of diffuse interstitial lung disease (DILD). This approach helps increase diagnostic accuracy and is an internationally accepted recommendation.

• #1 A Diagnostic Algorithm for Idiopathic Pulmonary Fibrosis

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

  Assessing the patients pulmonary physiology should include spirometry, DLCO and body plethysmography (lung volumes). […] The decision whether to order HRCT in the workup for a patient who has dyspnea and a normal chest radiograph is challenging. […] The favored biopsy approach is surgical, using either an open or a video-assisted thoracoscopic technique. […] Frequently, neither HRCT nor surgical lung biopsy demonstrates UIP, making the definitive diagnosis of IPF difficult. […] When surgical lung biopsy is not possible, current practice at leading centers uses a multidisciplinary approach to allow for a confident diagnosis. […] If the discussion leads to a consensus diagnosis of IPF, then the patients clinician can move forward with treatment options.

• #1 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

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

  ATS/ERS/JRS/ALAT guidelines recommend that IPF can be confidently diagnosed if the clinical evaluation of the patient points to IPF and the HRCT shows a definite or probable pattern of UIP (conditional biopsy recommended). […] 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. […] IPF is the likely diagnosis when any of the following features are present: Moderate-to-severe traction bronchiectasis/bronchiolectasis in a man over age 50 years or in a woman over age 60 years; Extensive (30%) reticulation on HRCT and an age 70 years; Increased neutrophils and/or absence of lymphocytosis in BAL fluid; Multidisciplinary discussion reaches a confident diagnosis of IPF.

• #1 Idiopathic pulmonary fibrosis: MedlinePlus GeneticsLock

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

  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. […] 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. […] Most cases of idiopathic pulmonary fibrosis are sporadic; they occur in people with no history of the disorder in their family. […] Familial pulmonary fibrosis appears to have an autosomal dominant pattern of inheritance. Autosomal dominant inheritance means one copy of an altered gene in each cell is sufficient to cause the disorder.

• #1

  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. Patients typically present with symptoms of chronic dyspnoea and cough over a period of months to years. IPF has a poor prognosis, with an average life expectancy of 35 years from diagnosis if left untreated. […] 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.

• #1 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

  A total of 44,891 with IPF were identified. The average time to a diagnosis of IPF was 2.7 years after initial respiratory diagnosis. […] Our retrospective cohort demonstrates that IPF is often diagnosed late, usually preceded by other respiratory diagnoses and hospitalizations. […] The diagnosis of IPF can be challenging, as it requires the exclusion of other causes of fibrosing interstitial lung disease (ILD) such as hypersensitivity pneumonitis, exposures to various medications, radiation, and environmental exposures to inorganic dusts and organic agents associated with hypersensitivity reaction, as well as rheumatologic diseases. […] It has been proposed that late initiation of treatment may limit the overall benefit of these agents. […] Our data demonstrated that many patients received other respiratory diagnoses prior to their coded clinical diagnosis of IPF.

• #1 Diagnosis of PF | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/nurses/understanding-PF/diagnosis-of-pf

  Nurses play a key role in helping patients and their caregivers navigate the diagnostic process. […] Early and accurate detection of pulmonary fibrosis is essential in ILDs as pulmonary fibrosis takes away lung function and once this is lost, it is lost forever. Identifying pulmonary fibrosis in patients as early as possible may help to improve their burden of disease, slow decline in daily functioning and quality of life, and reduce the risk of early mortality. […] Patients should be informed that a diagnosis of ILD requires lung function tests, lung imaging and specialist doctors interpretation of their medical history and test results. A multidisciplinary team will determine an ILD diagnosis by considering the patients medical history and results from PFTs and HRCTs. […] A multidisciplinary team, including specialist doctors and radiologists, will discuss all available information, which increases the accuracy of ILD diagnosis and prognosis prediction.

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

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

  Idiopathic pulmonary fibrosis (IPF) is a rare fibrosing interstitial pneumonia of unknown cause. The disease is chronic, progressive, and ultimately fatal. […] Diagnosis is suspected clinically in patients with bi-basilar inspiratory crackles, older age (e.g., 60 years), and unexplained symptomatic or asymptomatic bilateral pulmonary fibrosis on a chest x-ray or high-resolution computed tomography. […] In the absence of findings suggesting an alternative disease process, IPF is usually confirmed by multi-disciplinary review based on the presence of a radiological (high-resolution chest CT) pattern of usual interstitial pneumonia (UIP). Lung biopsy histopathology may be considered when radiological and clinical data result in an uncertain diagnosis. […] Diagnosis requires the exclusion of other forms of interstitial pneumonia, including other idiopathic interstitial pneumonias and interstitial lung diseases associated with environmental exposures, medications, or systemic diseases (e.g., connective tissue disease).

• #2 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. […] 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.

• #2 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

  A total of 44,891 with IPF were identified. The average time to a diagnosis of IPF was 2.7 years after initial respiratory diagnosis. […] Our retrospective cohort demonstrates that IPF is often diagnosed late, usually preceded by other respiratory diagnoses and hospitalizations. […] The diagnosis of IPF can be challenging, as it requires the exclusion of other causes of fibrosing interstitial lung disease (ILD) such as hypersensitivity pneumonitis, exposures to various medications, radiation, and environmental exposures to inorganic dusts and organic agents associated with hypersensitivity reaction, as well as rheumatologic diseases. […] It has been proposed that late initiation of treatment may limit the overall benefit of these agents. […] Our data demonstrated that many patients received other respiratory diagnoses prior to their coded clinical diagnosis of IPF.

• #2 Idiopathic pulmonary fibrosis: MedlinePlus GeneticsLock

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

  Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively. […] The most common signs and symptoms of idiopathic pulmonary fibrosis are shortness of breath and a persistent dry, hacking cough. Many affected individuals also experience a loss of appetite and gradual weight loss. Some people with idiopathic pulmonary fibrosis develop widened and rounded tips of the fingers and toes (clubbing) resulting from a shortage of oxygen. […] In people with idiopathic pulmonary fibrosis, scarring of the lungs increases over time until the lungs can no longer provide enough oxygen to the body’s organs and tissues. […] Most affected individuals survive 3 to 5 years after their diagnosis. However, the course of the disease is highly variable; some affected people become seriously ill within a few months, while others may live with the disease for a decade or longer.

• #2 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.

• #2 Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis | Pulmonology

  https://www.journalpulmonology.org/en-consensus-document-for-diagnosis-treatment-articulo-S2173511516000130

  The general practice physician must be aware of IPF symptoms in order to consider this disease when establishing the initial diagnosis, and to refer IPF suspicious cases to a pulmonologist. […] The diagnosis of IPF requires the exclusion of other known causes of interstitial lung disease (domestic or occupational environmental exposures, connective tissue disease, drug toxicity) and the presence of a typical histological UIP pattern. […] According to the ATS/ERS recommendations and validated by international societies, in the absence of biopsy, IPF can be diagnosed only based on radiological criteria from the high resolution computed tomography (HRCT). […] The 2011 official ATS/ERS/JRS/ALAT consensus established the use of definitive and possible IPF; these definitions simplify the definitive diagnosis except for the other patients. According with to this multidisciplinary consensus, the diagnosis of UIP/IPF must be based on the consensus between the clinician, the radiologist and the pathologist.

• #2 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. […] Idiopathic pulmonary fibrosis should be considered for all patients with unexplained chronic exertional dyspnea, and those who present with a cough, bibasilar inspiratory crackles, and finger clubbing. […] An HRCT should be ordered for any patient who has abnormal findings on chest radiographs and clinical findings that are consistent with an ILD. […] A complete report of the family physicians findings is an important aspect of a referral to confirm IPF. […] Diagnosing IPF is challenging, but 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.

• #2 The role of imaging in the diagnosis and management of idiopathic pulmonary fibrosis in: Imaging Volume 14 Issue 1 (2021)

  https://akjournals.com/view/journals/1647/14/1/article-p16.xml

  The guidelines and imaging classification schemes for the diagnosis of IPF were created in 2011 based on a collaborative accordance of the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JPS) and Latin American Thoracic Association (ALAT). […] For patients presenting with basilar crackles and unexplained symptomatic or asymptomatic basilar fibrosis on chest x-ray, a high resolution computed tomography (HRCT) is recommended. […] Diagnosis of IPF must be carefully assessed to avoid any confounding secondary causes for pulmonary fibrosis. The ascertainment of IPF is a challenge, as individuals may also present with atypical HRCT patterns. But it is clinically relevant to make a precise diagnosis to direct the pharmacological therapy needed.

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

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

  Diagnosis is based on history, physical examination, high-resolution CT, and/or lung biopsy, if necessary. […] Diagnosis of idiopathic pulmonary fibrosis is suspected in patients with subacute dyspnea, nonproductive cough, and Velcro crackles on chest examination. However, IPF is commonly overlooked initially because of clinical similarities to other more common diseases, such as bronchitis, asthma, and heart failure. […] Diagnosis requires HRCT and in some cases lung biopsy. […] Chest x-ray typically shows diffuse reticular opacities in the lower and peripheral lung zones. Small cystic lesions (honeycombing) and dilated airways due to traction bronchiectasis are additional findings. […] HRCT shows diffuse, patchy, subpleural, reticular opacities with irregularly thickened interlobular septa and intralobular lines; subpleural honeycombing; and traction bronchiectasis. This is referred to as the usual interstitial pneumonia (UIP) pattern. Ground-glass opacities affecting 30% of the lung suggest an alternative diagnosis. […] Laboratory testing plays little role in diagnosis, except to exclude potential systemic rheumatic disorders.

• #2 Diagnosis of PF | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/nurses/understanding-PF/diagnosis-of-pf

  Pulmonary function tests (PFTs), including spirometry and lung diffusion test (DLco), are used to examine lung function. […] HRCT is the gold standard for early and accurate diagnosis of fibrotic ILDs. […] HRCT combined with multidisciplinary discussion can enable a confident ILD diagnosis. […] Regular re-evaluation and the search for new information that might increase the confidence in a specific ILD diagnosis is critical, as a specific ILD diagnosis may have implications regarding prognosis and treatment. […] A lung biopsy may be needed in some patients. This is a procedure where the doctor takes a small sample of lung tissue to help diagnose ILD. […] Pulmonary fibrosis is unpredictable and regular doctor visits can enable detection of progressive disease; patients should expect further testing including lung imaging, lung function tests, as well as monitoring of their symptoms.

• #2 The role of imaging in the diagnosis and management of idiopathic pulmonary fibrosis in: Imaging Volume 14 Issue 1 (2021)

  https://akjournals.com/view/journals/1647/14/1/article-p16.xml

  The diagnostic criteria of previously defined HRCT UIP patterns (ATS/ERS/JRS/ALAT classification) have been updated to definite UIP, probable UIP, indeterminate UIP and alternate diagnosis. […] Histological UIP is seen as an interstitial fibrosis with spatial heterogeneity and patchy involvement of the lung parenchyma. […] For the definite diagnosis of IPF, UIP must be identified with HRCT. HRCT has specific and broad agreements in the definition of IPF patterns, illustrating fibrotic patterns of reticulation, traction bronchiectasis, honeycombing and ground-glass opacity (GGO). […] The presence or absence of traction bronchiectasis is very important. On its own it does not mean definite UIP. […] The hallmark for assessing and monitoring restrictive lung diseases is the pulmonary function test (PFT) parameter TLC. However, FVC has been commonly used as an indicator of disease severity.

• #2

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

  In diagnosing and evaluating patients with IPF, HRCT plays a crucial role. […] To confirm a diagnosis of IPF, a multidisciplinary team discussion is recommended between respiratory consultants, radiologists and pathologists based on clinical data, HRCT and lung biopsy if acquired. […] IPF can be difficult to diagnose in its early stages due to overlap of symptoms with other more common conditions. […] 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. […] The planned change to the guidelines reflects campaigning by clinicians and patient groups who have been in favour of this development for many years.

• #2 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

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

  ATS/ERS/JRS/ALAT guidelines recommend that IPF can be confidently diagnosed if the clinical evaluation of the patient points to IPF and the HRCT shows a definite or probable pattern of UIP (conditional biopsy recommended). […] 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. […] IPF is the likely diagnosis when any of the following features are present: Moderate-to-severe traction bronchiectasis/bronchiolectasis in a man over age 50 years or in a woman over age 60 years; Extensive (30%) reticulation on HRCT and an age 70 years; Increased neutrophils and/or absence of lymphocytosis in BAL fluid; Multidisciplinary discussion reaches a confident diagnosis of IPF.

• #2 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 specimen can be obtained through either an open lung biopsy or video-assisted thoracoscopic surgery (VATS). A surgical lung biopsy provides the best sample for which to distinguish usual interstitial pneumonia from other idiopathic interstitial pneumonias.

• #2 Imaging in idiopathic pulmonary fibrosis: diagnosis and mimics | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-imaging-in-idiopathic-pulmonary-fibrosis-S1807593222006123

  The aim of this article was to review the clinical, radiological, and pathological features of idiopathic pulmonary fibrosis and of diseases that might mimic idiopathic pulmonary fibrosis presentation. […] A detailed clinical assessment is essential for the diagnosis of patients with interstitial lung diseases (ILDs) and for the diagnostic confirmation of IPF. […] The main symptoms of IPF, including progressive dyspnea and dry cough, are often nonspecific. […] Pulmonary function tests (PFTs) in IPF are characterized by a restrictive pattern combined with a decreased diffusing capacity. […] In approximately 66.6% of cases, a diagnosis of IPF can be performed based only on clinical and radiological features. […] A confident diagnosis of UIP based on HRCT is accurate in 80% to 95% of cases using pathology as a reference. […] The diagnosis of IPF is challenging, and a multidisciplinary discussion with ILD experts, including physicians, radiologists, and pathologists, improves the accuracy of the diagnosis, which is essential to establishing the appropriate management strategy.

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

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

  Key diagnostic factors include dyspnea, cough, and crackles. […] 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.

• #2 Diagnostic and prognostic implications of 2018 guideline for the diagnosis of idiopathic pulmonary fibrosis in clinical practice | Scientific Reports

  https://www.nature.com/articles/s41598-021-95728-7

  The purpose of this study was to evaluate the implications of the 2018 updated guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) in clinical practice compared to 2011 guideline. […] The 2018 criteria provide better prognostic stratification than the 2011 in patients with possible UIP. […] BAL fluid analysis can improve the diagnostic certainty for IPF diagnosis in patients with probable UIP CT pattern. […] Diagnostic guidelines for IPF were updated in 2018 by the American Thoracic Society (ATS), the European Respiratory Society (ERS), the Japanese Respiratory Society (JRS), and the Latin American Thoracic Society (ALAT). […] The latest ARS/ERS/JRS/ALAT guideline recommend cellular analysis of BAL fluid for patients with newly detected ILD of unknown cause who are clinically suspected of having IPF and have an HRCT pattern of probable UIP, indeterminate for UIP or an alternative diagnosis. […] Adding BAL fluid results can improve the diagnostic certainty for IPF diagnosis in patients with probable UIP CT patterns.

• #2 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Idiopathic-Pulmonary-Fibrosis-Diagnosis.aspx

  A chest X-ray is useful to detect abnormalities in the structure of the lungs and differentiate IPF from other similar conditions of the respiratory system. […] Computerized tomography (CT) scans can be used when a clearer image with greater detail is required. […] In general, the presence of a honeycomb effect in the lung tissue often reflects scarring in the area and is indicative of fibrosis. […] In some cases, further testing is required to investigate the symptoms and cause of the condition, which may include a bronchoscopy. […] Video-assisted thoracoscopic surgery (VATS) is a technique used to obtain a biopsy of the lung tissue, which may be required to eliminate other conditions in the diagnostic process.

• #2 Idiopathic pulmonary fibrosis – Wikipedia

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

  In the absence of a typical UIP pattern on HRCT, a surgical lung biopsy is required for confident diagnosis. […] Histologic specimens for the diagnosis of IPF must be taken at least in three different places and be large enough that the pathologist can comment on the underlying lung architecture. […] Lung tissue from people with IPF usually show a characteristic histopathologic UIP pattern and is therefore the pathologic counterpart of IPF. […] According to the updated 2011 guidelines, in the absence of a typical UIP pattern on HRCT, a surgical lung biopsy is required for confident diagnosis.

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

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

  A morphological pattern of usual interstitial pneumonia (UIP), either a radiological or a histopathological one, is required to establish an IPF diagnosis. On the other hand, a UIP pattern has been associated with other conditions, such as asbestosis, fHP, and rheumatoid arthritis. Therefore, the exclusion of alternative diagnoses remains central, even with a typical UIP pattern on HRCT. […] HRCT has become central to the diagnosis of IPF. The radiological appearance of UIP (or typical UIP) has a strong correlation with histological UIP, precluding the need for invasive procedures. 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, and some patients might be unsuitable for invasive procedures. […] 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.

• #2 Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis | Pulmonology

  https://www.journalpulmonology.org/en-consensus-document-for-diagnosis-treatment-articulo-S2173511516000130

  Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. […] The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an internationally accepted recommendation. […] The diagnosis of IPF requires a multidisciplinary approach, including the awareness of general practice physicians, and experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of diffuse interstitial lung disease (DILD). This approach helps increase diagnostic accuracy and is an internationally accepted recommendation.

• #2 Multidisciplinary diagnosis | PulmonaryFibrosis360.com

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

  A MULTIDISCIPLINARY APPROACH INVOLVING A CLINICAL, RADIOLOGICAL AND PATHOLOGICAL REVIEW IS RECOMMENDED FOR EARLY AND ACCURATE 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. […] 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.

• #2 A Diagnostic Algorithm for Idiopathic Pulmonary Fibrosis

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

  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. […] The guidelines include an algorithm as an aid to the systematic evaluation. […] A thorough and accurate medical history should include assessment of the severity of dyspnea and cough, signs and symptoms of connective tissue disease […] The physical examination must include careful auscultation for rales. […] Laboratory testing should include a serologic autoantibody panel to evaluate for connective tissue diseases that can manifest as ILD.

• #2 Idiopathic pulmonary fibrosis: MedlinePlus GeneticsLock

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

  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. […] 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. […] Most cases of idiopathic pulmonary fibrosis are sporadic; they occur in people with no history of the disorder in their family. […] Familial pulmonary fibrosis appears to have an autosomal dominant pattern of inheritance. Autosomal dominant inheritance means one copy of an altered gene in each cell is sufficient to cause the disorder.

• #2 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 term idiopathic means that we do not currently understand what causes the disease, a major obstacle to identifying it at its beginning stages. […] This tool takes literally zero additional minutes and can recognize features of the disease before symptoms manifest. […] This work is novel in that information already captured in the medical record is used to identify patients in the system who may be at a higher risk. […] 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.

• #2 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

  It is concerning that only a small percentage of patients ultimately received anti-fibrotic treatment. […] 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.

• #2 Idiopathic pulmonary fibrosis: new approaches to diagnosis and treatment | Respiratory Medicine Today

  https://respiratory.medicinetoday.com.au/rmt/2020/may/feature-article/idiopathic-pulmonary-fibrosis-new-approaches-diagnosis-and-treatment

  The comprehensive management of IPF involves disease-specific therapies and management of comorbidities, disease-related symptoms, pulmonary rehabilitation as well as palliative care and/or lung transplantation. […] Early referral is critical for both the diagnosis and management of IPF. Multiple studies have shown that using an ILD MDT improves accuracy and confidence of IPF diagnosis compared with doctors working individually. […] In order to confirm a diagnosis of IPF and access medical treatment via PBS, all IPF patients need to be discussed at an ILD MDT meeting. […] High-resolution CT (HRCT) plays a central role in the diagnosis of IPF. The presence of a definite usual interstitial pneumonia (UIP) pattern on HRCT is adequate to diagnose IPF in the correct clinical context, without the need for tissue biopsy. […] Lung biopsy is sometimes required when the above testing is insufficient for a diagnosis. This decision requires discussion at an ILD multidisciplinary team (MDT) meeting.

• #2 CHEST Annual Meeting

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

  When we look at the pulmonologists survey results, one notable finding is that they have strong knowledge about the guidelines for IPF diagnosis, but it is the process of diagnosis that really is limited. […] The main factors contributing to delayed diagnosis relate to awareness awareness about pulmonary fibrosis, awareness about the disease process and bringing that up within the differential diagnosis of a patient who presents with shortness of breath or cough. […] 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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