Materiały źródłowe

• #1 Diagnosis and management of interstitial lung disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4215823/

  The complex tasks of making a confident diagnosis of a specific form of interstitial lung disease (ILD) and formulating a patient-centered, personalized management plan in an attempt to achieve remission or stabilization of the disease process can pose formidable challenges to clinicians. […] A well-performed patient history and physical examination provides invaluable information that can be combined with appropriate laboratory testing, imaging, and, if needed, tissue biopsy to reach a confident ILD diagnosis, and high-resolution computed tomography (HRCT) of the thorax is usually a key component of the diagnostic evaluation. […] Successful management of patients with ILD is dependent upon establishing an accurate and specific diagnosis. […] Because various forms of ILD such as IPF, non-IPF forms of idiopathic interstitial pneumonia (IIP), CTD-ILD, and hypersensitivity pneumonitis (HP) can have similar clinical presentations, patients with suspected ILD must undergo an evaluation that adequately establishes a confident diagnosis of a specific ILD, as treatment and various management decisions are diagnosis-specific and may vary considerably according to the specific form of ILD that is diagnosed.

• #2 Approach to the adult with interstitial lung disease: Diagnostic testing – UpToDate

  https://www.uptodate.com/contents/approach-to-the-adult-with-interstitial-lung-disease-diagnostic-testing

  Approach to the adult with interstitial lung disease: Diagnostic testing […] The initial evaluation of patients with ILD is aimed at identifying the etiology of the ILD and its severity. The results of laboratory, radiographic, and pulmonary function tests guide the decisions about whether to pursue bronchoalveolar lavage and/or transbronchoscopic, thoracoscopic, or open lung biopsy. […] An overview of the diagnostic testing that is helpful in the diagnosis of ILD will be presented here (algorithm 2). The clinical findings that aid in the diagnosis of ILD and the individual causes of ILD are discussed separately. […] The list of causes of ILD, also known as diffuse parenchymal lung disease, is broad and includes those ILDs that are associated with a broad range of diseases, exposures, and drugs. ILD may also occur as an idiopathic condition. The treatment choices and prognosis vary among the different causes and types of ILD, so ascertaining the correct diagnosis is important. […] A variety of infectious processes cause interstitial opacities on chest radiograph, including fungal pneumonias, atypical bacterial pneumonias, and viral pneumonias. These infections often occur in immunocompromised hosts and are discussed separately.

• #3 Diagnosis & Monitoring – Lung Foundation Australia

  https://lungfoundation.com.au/health-professionals/conditions/interstitial-lung-disease/diagnosis-monitoring/

  The diagnosis and classification of ILD is complex. […] Early and accurate diagnosis is important to optimise patient outcomes. […] Detailed clinician history, examination findings, blood testing, lung function and imaging findings are critical to a timely and accurate diagnosis of ILD. […] In certain instances, histopathological sampling via bronchoscopy or surgical or endobronchial lung biopsy is also required to diagnose specific ILDs. […] The gold standard for ILD diagnosis is ultimately via an ILD specific multi-disciplinary meeting (MDM). […] If ILD is suspected or confirmed, early referral to a respiratory physician is recommended for further investigation and management. […] These detailed examinations and investigations to diagnose ILD are typically performed or arranged by respiratory physicians.

• #4 Diagnosing Interstitial Lung Disease – ARRS InPractice

  https://arrsinpractice.org/diagnosing-interstitial-lung-disease/

  The diagnosis of interstitial lung disease (ILD) involves multidisciplinary collaboration among radiology, pulmonary medicine, rheumatology, and anatomic pathology disciplines. Imaging findings play a major role in the diagnosis of a variety of diffuse lung diseases, and the radiologists input into the ultimate diagnosis is often substantial. Imaging is of critical importance in the diagnosis of ILD, although the accurate interpretation of characteristic high-resolution CT (HRCT) findings can be challenging. […] The goals of the radiologist in the evaluation of a patient with suspected pulmonary fibrosis are to determine whether a diffuse lung disease is present, determine the pattern of fibrosis, and provide an appropriate differential diagnosis. Usual interstitial pneumonia (UIP) pattern of pulmonary fibrosis is the most common ILD.

• #5 How to diagnose RA-ILD | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/rheums/ra-ild/diagnosis-of-ra-ild

  High-resolution computed tomography (HRCT) is considered the gold standard for diagnosis of interstitial lung disease (ILD) in rheumatoid arthritis (RA). […] Chest HRCT is the most sensitive and specific method for diagnosing ILD in RA, and allows for ILD extent and radiological pattern to be assessed. […] A multidisciplinary approach, including at least a rheumatologist, pulmonologist and radiologist, enhances diagnosis and management of RA-ILD. […] In addition to HRCT, diagnosis of RA-ILD is primarily based on clinical presentation, physical examination and PFTs. […] Multidisciplinary teams can play a central role in ILD diagnosis and management.

• #6 Diagnosis & Monitoring – Lung Foundation Australia

  https://lungfoundation.com.au/health-professionals/conditions/interstitial-lung-disease/diagnosis-monitoring/

  An ILD multi-disciplinary meeting (ILD-MDM) is considered best-practice standard of care in international guidelines. […] An ILD-MDM is a collaborative meeting of specialists with the purpose of accurately determining a consensus diagnosis, prognosis and management plan. […] The decision to attain tissue for histological analysis should be ideally determined via multidisciplinary discussion. […] The diagnosis of ILD is heavily reliant on good quality CT imaging. […] Baseline screening blood work is important at the time of initial investigation and referral. […] Common clinical features and investigation findings present in idiopathic pulmonary fibrosis (IPF) are summarised in the table below. […] Early symptom identification and diagnosis are important in optimising outcomes by ensuring the right therapy is commenced as early as possible to alter disease trajectory.

• #7 Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data | Nature Communications

  https://www.nature.com/articles/s41467-023-37720-5

  According to the American Thoracic Society (ATS) guidelines, accurate diagnosis of ILD subtypes requires a multidisciplinary assessment reviewing clinical history, HRCT, and pathology. […] In addition, longitudinal monitoring with CT can assess the progression of Serial CT that can reveal changes in the extent of parenchymal architectural distortion, reticulation, bronchiectasis and honeycombing, allowing for the identification of progressive fibrotic disease which correlates with poorer survival. […] The purpose of our study is to develop an AI system that (1) can classify 5 different types of ILD based on initial chest CT scans and relevant clinical history as well as (2) monitor a patients disease progression. […] The joint CNN model showed superior performance in the classification of ILD subtypes.

• #8 Diagnosis and management of interstitial lung disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4215823/

  A thorough and comprehensive history may provide invaluable information that can suggest certain entities and provide suspicion that a patient may have a specific diagnosis such as hypersensitivity pneumonitis (HP) or CTD-ILD. […] The routine postero-anterior chest X-ray (CXR) can be highly suggestive of specific ILD entities, and previous CXRs, if available, should be sought to determine whether the disease is acute versus chronic. […] Although the combination of history, physical examination, CXR, and other appropriate laboratory testing may provide a likely diagnosis, additional testing is usually needed to reach a confident diagnosis of a specific ILD. […] HRCT of the thorax can provide invaluable information that strongly supports a specific diagnosis and may be diagnostic (e.g. typical changes of UIP) such that further testing with bronchoscopy or surgical lung biopsy is not required.

• #9 Approach to the adult with interstitial lung disease: Clinical evaluation – UpToDate

  https://www.uptodate.com/contents/approach-to-the-adult-with-interstitial-lung-disease-clinical-evaluation

  Approach to the adult with interstitial lung disease: Clinical evaluation […] The diffuse parenchymal lung diseases, often collectively referred to as the interstitial lung diseases (ILDs), are a heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic, or pathologic manifestations (algorithm 1) [1-7]. The descriptive term „interstitial” reflects the pathologic appearance that the abnormality begins in the interstitium, but the term is somewhat misleading, as most of these disorders are also associated with extensive alteration of alveolar and airway architecture. […] An overview of the clinical findings that can aid in the diagnosis of ILD will be presented here (algorithm 2). The individual causes of ILD and the diagnostic testing that is helpful in evaluating patients with ILD are discussed separately. […] […] […] (See „Approach to the adult with interstitial lung disease: Diagnostic testing”.) […] […] […] (See „Role of bronchoalveolar lavage in diagnosis of interstitial lung disease”.)

• #10 Diffuse Interstitial Lung Disease – Diagnosis and Treatment

  https://www.radiologyinfo.org/en/info/diffuselung

  Blood tests, pulmonary function tests (spirometry), pulse oximetry, chest x-ray, chest CT, bronchoscopy with biopsy, surgical biopsy, or a combination of the above may be performed to help diagnose your condition. […] To determine the cause of interstitial lung disease, a physician may perform a physical examination and order diagnostic tests, including: […] Chest x-ray: The patterns of lung damage associated with various interstitial lung diseases are often identifiable on chest x-rays. Chest x-rays may also be used to track the progression of interstitial lung disease. […] CT imaging of the chest: Computed tomography (CT), including a specific technique known as high resolution CT, is used to see fine detail of the interstitium that may not be visible on a chest x-ray. In some cases, a specific diagnosis (such as idiopathic pulmonary fibrosis) can be confirmed based on the CT appearance, potentially avoiding the need for lung biopsy.

• #11 Interstitial Lung Disease (ILD) Diagnosis & Treatment | Mount Sinai – New York

  https://www.mountsinai.org/care/pulmonology/services/pulmonary-fibrosis-ild/treatment

  Interstitial lung disease (ILD) is a category of lung disorders characterized by inflammation and/or scarring of lung tissue. Such damage can affect your ability to breathe and to get adequate oxygen into the blood stream. […] To diagnose ILD, your doctor will take your thorough medical history and perform a physical exam, looking for the signs and symptoms of interstitial lung disease such as rales, (sounds heard with the stethoscope) and clubbing of the fingers. […] Additional diagnostic tests may include: Pulmonary function tests (including 6 minute walk test), Blood tests (to look for connective tissue diseases and other disorders), CT scan of the chest, Bronchoscopy and/or lung lavage, Echocardiogram, And possibly surgical lung biopsy.

• #12 Diagnosing Interstitial Lung Disease (ILD) | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/healthcare-professionals/ild/diagnosing-ild

  This section will help you to understand: […] The diagnosis of ILDs usually takes place in secondary care and is often made by a multidisciplinary team. However, primary care is often where patients will first present with symptoms so its important that HCPs can spot the signs and symptoms of ILDs and know when to refer a patient. […] Up to 55% of ILD patients are initially misdiagnosed, leading to significant delays in treatment. […] This overlap makes it challenging for healthcare professionals to recognise ILD early on, particularly in older adults, who may already have multiple comorbidities. […] Ensure that red flag symptoms such as chest pain, leg swelling, palpitations, haemoptysis and weight loss and recorded and investigated appropriately. […] ILDs will often be unresponsive to treatments for more common respiratory conditions like asthma or COPD, such as inhaled or oral corticosteroids, or bronchodilators, despite good adherence and technique.

• #13 Interstitial lung disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/diagnosis-treatment/drc-20353113

  Finding the cause of interstitial lung disease can be challenging, and sometimes the cause can’t be found. […] Healthcare professionals must rule out these conditions before making a diagnosis. […] Some of the following tests may be necessary. […] This imaging test is key to the diagnosis of interstitial lung disease. […] A high-resolution CT scan can be especially helpful in figuring out how much lung damage there is. […] Sometimes interstitial lung diseases can be diagnosed only by looking at a small amount of lung tissue in a lab what’s known as a biopsy. […] Your doctor or other healthcare professional may take a tissue sample using one of these methods: […] Although this is a more invasive procedure with potential complications, it’s often the only way to get a large enough tissue sample to make the correct diagnosis. […] Let your pulmonologist know if you’ve had any chest X-rays, CT scans or other tests so the healthcare professional can request the results before your visit. […] The pulmonologist can make a diagnosis by comparing an old imaging test with those of a current test.

• #14 Interstitial lung disease – Wikipedia

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

  Diagnosis of ILD involves assessing the signs and symptoms as well as a detailed history investigating occupational exposures. ILD usually presents with dyspnea, worsening exercise intolerance and 30-50% of those with ILD have a chronic cough. On examination, velcro crackles, in which the crackles compare to the sound of velcro being unfastened, are common in ILD. […] Pulmonary function tests usually show a restrictive defect with decreased diffusion capacity of carbon monoxide (DLCO) indicating reduced alveolar to blood capillary transport. Pulmonary function testing is indicated for all people with ILD and the FVC loss and DLCO is prognostic, with an FVC loss of greater than 5% per year associated with a poor prognosis in fibrosis subtypes of ILD. […] A chest x-ray is 63% sensitive and 93% specific for ILD. With advances in computed tomography, CT scans of the chest have supplanted lung biopsy as the preferred diagnostic test for ILD. A thoracic CT scan is 91% sensitive and 71% specific for ILD.

• #15 Interstitial Lung Disease (ILD) | Symptoms, Treatments & Stages | ELF

  https://europeanlung.org/en/information-hub/lung-conditions/interstitial-lung-disease/

  Essential investigations include imaging of the lungs by chest radiography and computed tomography (CT) scanning. Typical appearances of interstitial lung disease include widespread fine nodules, a net-like pattern or a general haziness. High resolution CT scans can be used to help define the specific type of interstitial lung diseases by looking at the type and spread of the lung shadowing on the image. […] Lung function testing and oxygen levels in the blood are also important for diagnosis and monitoring progress of interstitial lung disease. […] A surgical lung biopsy, where samples of lung tissue are removed and examined, is sometimes performed for accurate diagnosis of interstitial lung disease but its use has declined as the quality of CT scans has improved.

• #16 Interstitial lung disease – Wikipedia

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

  Diagnosis of ILD involves assessing the signs and symptoms as well as a detailed history investigating occupational exposures. ILD usually presents with dyspnea, worsening exercise intolerance and 30-50% of those with ILD have a chronic cough. On examination, velcro crackles, in which the crackles compare to the sound of velcro being unfastened, are common in ILD. […] Pulmonary function tests usually show a restrictive defect with decreased diffusion capacity of carbon monoxide (DLCO) indicating reduced alveolar to blood capillary transport. Pulmonary function testing is indicated for all people with ILD and the FVC loss and DLCO is prognostic, with an FVC loss of greater than 5% per year associated with a poor prognosis in fibrosis subtypes of ILD. […] A chest x-ray is 63% sensitive and 93% specific for ILD. With advances in computed tomography, CT scans of the chest have supplanted lung biopsy as the preferred diagnostic test for ILD. A thoracic CT scan is 91% sensitive and 71% specific for ILD.

• #17 Imaging Innovations in the Screening, Diagnosis, and Monitoring of Systemic Autoimmune Disease-Related Interstitial Lung Disease – European Medical Journal

  https://www.emjreviews.com/radiology/article/imaging-innovations-in-the-screening-diagnosis-and-monitoring-of-systemic-autoimmune-disease-related-interstitial-lung-disease-j140124/

  Interstitial lung disease (ILD) is a common and serious complication of systemic autoimmune rheumatic diseases (SARD). […] High-resolution CT of the chest is the current gold standard test for diagnosis of ILD, yet, it is not uniformly performed as an initial diagnostic test. […] When suspected, high resolution CT (HRCT) of the chest remains the centre piece to confirm the diagnosis, besides obtaining pulmonary function tests (PFT), and testing the exercise capacity with hall walk testing. […] The gold standard for screening ILD is HRCT. […] According to the proposed 2023 guidelines by the American College of Rheumatology (ACR), for people with SARDs at increased risk of developing ILD, it is recommended against screening with surgical lung biopsy or transbronchial biopsy. […] In patients with either IIM-ILD or SSc-ILD, PFTs are recommended every 3-6 months for the first year, then less frequently once stable. […] HRCT has been a pivotal imaging development in the past two decades, and has become the mainstay in the diagnosis of ILD in patients with SARDs.

• #18 Interstitial Lung Disease | Cleveland Clinic

  https://my.clevelandclinic.org/departments/respiratory/depts/interstitial-lung-disease

  A biopsy (taking a tissue sample) performed in the operating room by a surgeon, either with an incision or using a videoscope that is inserted through small (approximately 1 cm each) incisions in the chest wall. A surgical lung biopsy carries higher risks than other diagnostic procedures, and may not be necessary when the chest CT or other testing is sufficiently definitive. Some ILDs (UIP, NSIP, chronic hypersensitivity pneumonitis, COP) are more likely to require surgical lung because of inconclusive CT scans and the fact that they cannot typically be diagnosed definitively with bronchoscopy. […] A CT scan of the lungs using specific protocols. This type of CT scan is best used at the time of diagnosis and later only when an unusual aspect of the disease occurs which requires the physician to look for complications or additional diseases. […] For ILD, this type of chest CT should be obtained without IV contrast (dye), and with thin sections to allow for detailed examination of the lung tissue. With an experience CT reader, many ILDs can be diagnosed with a high degree of confidence using CT alone, without the need for a biopsy.

• #19 Diffuse Interstitial Lung Disease – Diagnosis and Treatment

  https://www.radiologyinfo.org/en/info/diffuselung

  Blood tests, pulmonary function tests (spirometry), pulse oximetry, chest x-ray, chest CT, bronchoscopy with biopsy, surgical biopsy, or a combination of the above may be performed to help diagnose your condition. […] To determine the cause of interstitial lung disease, a physician may perform a physical examination and order diagnostic tests, including: […] Chest x-ray: The patterns of lung damage associated with various interstitial lung diseases are often identifiable on chest x-rays. Chest x-rays may also be used to track the progression of interstitial lung disease. […] CT imaging of the chest: Computed tomography (CT), including a specific technique known as high resolution CT, is used to see fine detail of the interstitium that may not be visible on a chest x-ray. In some cases, a specific diagnosis (such as idiopathic pulmonary fibrosis) can be confirmed based on the CT appearance, potentially avoiding the need for lung biopsy.

• #20 Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data | Nature Communications

  https://www.nature.com/articles/s41467-023-37720-5

  For accurate diagnosis of interstitial lung disease (ILD), a consensus of radiologic, pathological, and clinical findings is vital. […] Management of ILD also requires thorough follow-up with computed tomography (CT) studies and lung function tests to assess disease progression, severity, and response to treatment. […] Here, we utilize RadImageNet pretrained models to diagnose five types of ILD with multimodal data and a transformer model to determine a patients 3-year survival rate. […] When clinical history and associated CT scans are available, the proposed deep learning system can help clinicians diagnose and classify ILD patients and, importantly, dynamically predict disease progression and prognosis. […] High-resolution computed tomography (HRCT) plays a significant role in accurately classifying the various subtypes of ILD.

• #21 Interstitial Lung Diseases – Diagnosis | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/interstitial-lung-diseases/diagnosis

  Your healthcare provider will diagnose your ILD based on your symptoms, your medical and family histories, your risk factors, and the results from tests and procedures. […] Your healthcare provider may order one of these common tests to help diagnose an ILD. […] Blood tests are used to look for signs of an infection or an autoimmune disease that can cause an ILD. […] Lung tests include imaging tests like chest X-rays and CT scans that take pictures of your lungs. You may also do lung function tests that measure how much air you breathe out or how much air your lungs can hold. These tests help your doctor see how well your lungs are working. […] Sometimes your doctor may order other tests if they need more information to help diagnose an ILD. […] During this test, your healthcare provider will collect fluid from your lungs. This fluid will be tested for high levels of white blood cells and other immune cells or for substances in your environment that can cause lung scarring. […] Your healthcare provider will take a very small sample of your lung tissue. Your healthcare provider will check this sample to see if your lung tissue shows signs of inflammation, scarring, or other changes that happen when you have an ILD.

• #22 Interstitial Lung Disease | The University of Kansas Health System

  https://www.kansashealthsystem.com/care/conditions/interstitial-lung-disease

  The first step in diagnosis is an examination of your medical history, followed by a physical examination. […] Pulmonary function tests are commonly performed with the aid of specialized machines that measure the lungs’ ability to properly function. These tests can include chest X-rays, CT scans and blood tests. […] Spirometry is a common test that measures the lungs ability to function. This test uses a device called a spirometer, which can determine the lungs ability to take in, hold and expel air. Spirometry helps doctors determine the severity and type of problems experienced within the lungs, and is also often used throughout treatment to determine its effectiveness. […] Your interdisciplinary care team is comprised of pulmonologists with advanced training in interstitial lung disease, as well as dedicated respiratory therapists, thoracic radiologists, lung pathologists and rheumatologists. Together, these specialists offer unparalleled expertise in both diagnosis and treatment of IDL. They collaborate to ensure that you benefit from accurate diagnosis, comprehensive care, support services and the opportunity to participate in groundbreaking clinical trials.

• #23 Interstitial lung disease – Wikipedia

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

  Diagnosis of ILD involves assessing the signs and symptoms as well as a detailed history investigating occupational exposures. ILD usually presents with dyspnea, worsening exercise intolerance and 30-50% of those with ILD have a chronic cough. On examination, velcro crackles, in which the crackles compare to the sound of velcro being unfastened, are common in ILD. […] Pulmonary function tests usually show a restrictive defect with decreased diffusion capacity of carbon monoxide (DLCO) indicating reduced alveolar to blood capillary transport. Pulmonary function testing is indicated for all people with ILD and the FVC loss and DLCO is prognostic, with an FVC loss of greater than 5% per year associated with a poor prognosis in fibrosis subtypes of ILD. […] A chest x-ray is 63% sensitive and 93% specific for ILD. With advances in computed tomography, CT scans of the chest have supplanted lung biopsy as the preferred diagnostic test for ILD. A thoracic CT scan is 91% sensitive and 71% specific for ILD.

• #24 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.

• #25 Diagnosis of ILD | Interstitial Lung Disease | NewYork-Presbyterian

  https://www.nyp.org/columbiachestcare/ild_program_diagnosis

  Identifying the type of ILD that a patient has can be a challenging process. The goal of the ILD team at NewYork-Presbyterian Hospital/Columbia University Medical Center is to complete each patient’s diagnostic evaluation in a thorough, efficient, and expedient manner. […] The evaluation begins with a physical exam, blood tests, a chest x-ray, a chest CT scan, and pulmonary function testing. […] If a diagnosis cannot be made using these tests, a lung biopsy is often necessary. […] Some types of ILD are more amenable to diagnosis by bronchoscopy than others. […] Lastly, exercise testing (such as a cardiopulmonary exercise test and a six-minute walking test) will be performed to help gauge the severity of disease. […] Once diagnostic testing is complete, each case is presented and discussed at a multidisciplinary ILD conference, where an expert group of clinicians and researchers achieve a consensus on the diagnosis and treatment plan for each patient.

• #26 Interstitial Lung Disease | Condition | UT Southwestern Medical Center

  https://utswmed.org/conditions-treatments/interstitial-lung-disease/

  Echocardiogram to produce video images of the heart using ultrasound (sound waves) in a noninvasive procedure […] Endobronchial ultrasound (EBUS) to evaluate and diagnose lung disorders using a bronchoscope with an ultrasound probe to create images of the inside of the lungs […] Exercise stress test typically a six-minute walk to measure how well the lungs and heart function during exercise […] Pulmonary function tests to evaluate lung function, such as the volume of air flow in and out of the lungs, how well the lungs move oxygen into the bloodstream, and lung size […] Surgical biopsy to obtain a larger tissue sample, which may be needed to confirm a diagnosis and is performed by our thoracic surgeons.

• #27 Interstitial Lung Diseases – Diagnosis | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/interstitial-lung-diseases/diagnosis

  Your healthcare provider will diagnose your ILD based on your symptoms, your medical and family histories, your risk factors, and the results from tests and procedures. […] Your healthcare provider may order one of these common tests to help diagnose an ILD. […] Blood tests are used to look for signs of an infection or an autoimmune disease that can cause an ILD. […] Lung tests include imaging tests like chest X-rays and CT scans that take pictures of your lungs. You may also do lung function tests that measure how much air you breathe out or how much air your lungs can hold. These tests help your doctor see how well your lungs are working. […] Sometimes your doctor may order other tests if they need more information to help diagnose an ILD. […] During this test, your healthcare provider will collect fluid from your lungs. This fluid will be tested for high levels of white blood cells and other immune cells or for substances in your environment that can cause lung scarring. […] Your healthcare provider will take a very small sample of your lung tissue. Your healthcare provider will check this sample to see if your lung tissue shows signs of inflammation, scarring, or other changes that happen when you have an ILD.

• #28 Interstitial Lung Disease | ARUP Laboratories

  https://www.aruplab.com/testing/ild

  Interstitial lung disease (ILD) describes a large group of conditions that cause inflammation and scar tissue in the lungs. […] ILD may be characterized by four main manifestations: respiratory symptoms (e.g., shortness of breath and cough), specific chest radiographic abnormalities, decreased lung volume on pulmonary function test, and characteristic microscopic patterns of inflammation and fibrosis. […] Recent evidence suggests that use of biomarkers for lung injury and inflammation may be useful in the evaluation and management of ILD. […] If CTD-ILD is suspected, recent guidelines recommend testing for anti-nuclear antibodies, rheumatoid factor, and anti-citrullinated cyclic peptide antibody with additional autoantibody evaluation for selected cases guided by patients clinical history and presentation. […] For additional information refer to our Laboratory Test Directory regarding Interstitial Lung Disease (ILD) Biomarkers Panel.

• #29 Overview of Interstitial Lung Diseases – Lung and Airway Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/lung-and-airway-disorders/interstitial-lung-diseases/overview-of-interstitial-lung-diseases

• #30 Interstitial Lung Diseases | Choose the Right Test

  https://arupconsult.com/content/interstitial-lung-diseases

  Interstitial lung diseases (ILDs) are a varied group of disorders characterized by impairment in lung function and gas exchange due to the accumulation of extracellular matrix proteins in the parenchyma of the lungs. […] Diagnosis depends on clinical examination, a thorough patient history, laboratory testing, imaging, and in some cases, bronchoalveolar lavage analysis and/or tissue biopsy. […] A full serologic workup to detect autoimmune antibodies associated with connective tissue diseases is recommended. […] In some patients, interstitial lung disease (ILD) may be the first manifestation of a connective tissue disease. Workup of the ILD should include a thorough autoimmune evaluation (ie, serologic testing) to identify an associated connective tissue disease. […] In combination with other clinical findings, serologic testing can help narrow the diagnosis to a particular type of interstitial lung disease (ILD).

• #31 Interstitial Lung Disease (ILD) Panel | Test Summary | Quest Diagnostics Interstitial Lung Disease (ILD) PanelInterstitial Lung Disease (ILD) Panel

  https://testdirectory.questdiagnostics.com/test/test-guides/TS_ILD/interstitial-lung-disease-ild-panel

  This test is used to identify connective tissue disease (CTD) as a possible cause of interstitial lung disease (ILD). […] Interstitial Lung Disease (ILD) Panel […] Identify connective tissue disease (CTD) as a possible cause of interstitial lung disease (ILD). […] Determining the underlying cause of ILD can augment treatment decisions. […] The clinical and laboratory findings that inform a diagnosis of ILD may not help identify CTD-ILD, especially when ILD is the first manifestation of the autoimmune disease. […] Fortunately, testing for specific antibodies that are associated with different CTDs can help identify an underlying CTD. […] A positive result for 1 or more of the specific biomarkers in this panel may indicate the presence of the corresponding CTD; thus, a positive result may help diagnose CTD-ILD. […] Negative results may help exclude CTDs as a potential cause of ILD but do not always rule out a CTD; some individual markers are not diagnostic and have variable sensitivity and specificity for individual CTDs.

• #32 Interstitial Lung Disease Panel | Test Detail | Quest Diagnostics

  https://testdirectory.questdiagnostics.com/test/test-detail/39149/interstitial-lung-disease-panel?p=r&cc=MASTER

  Interstitial Lung Disease Panel – A subset of interstitial lung disease (ILD) patients have underlying connective tissue diseases (CTDs). This panel serves as an aid for the diagnosis of CTDs, including rheumatoid arthritis, inflammatory myositis, systemic sclerosis, and Sjogren’s syndrome, that are associated with the development of ILD. If left untreated, these CTD patients may develop lung fibrosis and inflammation. ILD is a direct consequence of these pathological changes. When severe, ILD leads to restrictive lung disease, pulmonary arterial hypertension, and cardiac disease (cor pulmonale). […] Interstitial Lung Disease (ILD) Panel […] Methodology: Enzyme Linked Immunosorbent Assay (ELISA) • Immunoturbidimetry • Multiplex Flow Immunoassay • Line Blot. […] Reference ranges are provided as general guidance only. To interpret test results use the reference range in the laboratory report.

• #33 Diagnostic Value of Serum KL-6 in Interstitial Lung Diseases | IJGM

  https://www.dovepress.com/diagnostic-value-of-serum-kl-6-in-interstitial-lung-diseases-peer-reviewed-fulltext-article-IJGM

  Objective: To explore serum KL-6 level and investigate its diagnostic value in interstitial lung diseases (ILDs). […] Conclusion: Serum KL-6 level was confirmed to be highly sensitive, specific, and accurate in the diagnosis of ILD. […] An easy-to-perform, minimally invasive, and reproducible biomarker should be taken as an ideal diagnostic method. […] KL-6, as an easy-to-perform, inexpensive, reproducible, and minimally invasive biomarker, is of great significance to the diagnosis of ILDs. Serum KL-6 level can be used to diagnose ILDs, with high sensitivity, specificity, and accuracy.

• #34 Tests and Diagnosis of Interstitial Lung Disease | PatientsEngage

  https://www.patientsengage.com/conditions/interstitial-lung-disease/diagnosis-tests

  Interstitial lung disease (ILD) is an umbrella term used for a large group of diseases that cause scarring (fibrosis) of the lungs. […] The diagnosis for Interstitial Lung Disease includes the following tests and examinations: […] Your doctor may ask about the onset, severity, rate of worsening of symptoms, the family history, smoking and drug history, and the occupational and environmental exposure history. Smoking can cause and worsen ILDs. […] The doctor may perform a 6-minute walk test to assess the aerobic exercise capacity and thus the lung and heart function. […] ILDs classically produce the 3Cs: cough, clubbing of the nails, and coarse crackles on auscultation. […] Inflammatory markers like ESR, CRP, IL6, KL-6, Surfactant Protein-D help to assess the extent of lung inflammation, scarring, and related prognosis. […] This is used to demarcate whether the lung condition is restrictive or obstructive lung disease. […] HRCT (High resolution computed tomography) […] Bronchoscopy or Lung Biopsy to view the tissue under microscope.

• #35 Interstitial lung disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/diagnosis-treatment/drc-20353113

  Finding the cause of interstitial lung disease can be challenging, and sometimes the cause can’t be found. […] Healthcare professionals must rule out these conditions before making a diagnosis. […] Some of the following tests may be necessary. […] This imaging test is key to the diagnosis of interstitial lung disease. […] A high-resolution CT scan can be especially helpful in figuring out how much lung damage there is. […] Sometimes interstitial lung diseases can be diagnosed only by looking at a small amount of lung tissue in a lab what’s known as a biopsy. […] Your doctor or other healthcare professional may take a tissue sample using one of these methods: […] Although this is a more invasive procedure with potential complications, it’s often the only way to get a large enough tissue sample to make the correct diagnosis. […] Let your pulmonologist know if you’ve had any chest X-rays, CT scans or other tests so the healthcare professional can request the results before your visit. […] The pulmonologist can make a diagnosis by comparing an old imaging test with those of a current test.

• #36 Diagnosis and management of interstitial lung disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4215823/

  Bronchoscopy and/or surgical lung biopsy may be required to make a confident diagnosis of a specific ILD. […] Patients with suspected ILD should have information from the history, physical examination, thoracic imaging, and other testing carefully and thoughtfully reviewed to determine whether or not additional procedures are needed and whether such procedures are likely to be helpful in reaching a confident diagnosis. […] Some specific combinations of clinical data with imaging results and other findings can strongly support specific ILD diagnoses. […] Once a confident diagnosis has been reached, a plan to treat and monitor disease activity can be put in place. […] A treatment plan should not only consist of pharmacologic agents that are prescribed to prevent progression and/or induce remission but should also include supportive therapies.

• #37 Diffuse Interstitial Lung Disease – Diagnosis and Treatment

  https://www.radiologyinfo.org/en/info/diffuselung

  Bronchoscopy and bronchoscopic biopsy: In this procedure, a very small sample of tissue is removed from the lung using a small, flexible tube called a bronchoscope that is passed through the mouth or nose, down the trachea (windpipe) and into the lungs. […] Surgical biopsy: A surgical biopsy may be needed to obtain a larger piece of lung than is possible with bronchoscopy. During this procedure, surgical instruments and a small camera are inserted through two or three small incisions between the ribs, allowing a surgeon to obtain a small piece of lung tissue.

• #38

  https://www.thoracic.org/professionals/career-development/residents-medical-students/ats-reading-list/adult/interstitial-lung-disease.php

  An Official American Thoracic Society/European Respiratory Society Statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188:744-748. Written to standardize and update the diagnostic criteria and terminology for idiopathic interstitial pneumonias, this article nicely summarizes the clinical, radiologic, and histologic features of the ILD alphabet soup. Discusses more recently described rare entities. PMID: 24032382 Free Full Text […] Meyer KC, Raghu G, Baughman RP, et al. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med 2012;185:1004-14. This article offers some help in selecting patients most suitable for bronchoalveolar lavage. Arguably the greatest strength of the guideline is how it nicely pulls together clinical presentation and typical BAL findings for a wide spectrum of interstitial lung diseases, which is difficult to find assembled in one place. PMID: 22550210

• #39 Interstitial (Nonidiopathic) Pulmonary Fibrosis Workup: Approach Considerations, Laboratory Studies, Imaging Studies

  https://emedicine.medscape.com/article/301337-workup

  The role of lung biopsy remains controversial, with expert opinion weighing in on both sides. Consensus appears to be building on the side of forgoing biopsy when the typical clinical and high-resolution CT scan features of a specific disease process are present. […] A multidisciplinary discussion is an essential part of diagnosis of ILD and deciding whether biopsy is necessary. This discussion often includes pulmonologists, radiologists, pathologists, thoracic surgeons, and rheumatologists. […] Transbronchial and endobronchial lung biopsies may be diagnostic, particularly for sarcoidosis, HP, or lymphangitic spread of carcinoma but frequently are not useful for other diagnoses. […] Surgical lung biopsy is regarded as the gold standard diagnostic test, however the most recent guidelines suggest that transbronchial cryobiopsy may be an acceptable alternative, in centers with sufficient experience performing the procedure. […] The histopathology observed in diffuse interstitial diseases of the lung is varied. The histopathologic classification of idiopathic interstitial pneumonias were updated by Katzenstein and Myers in 1998 to include the following 4 subgroups: UIP, AIP (diffuse alveolar damage), DIP/RBILD, and NSIP.

• #40 Interstitial Lung Disease Treatment at University Hospitals | Expertise in Diagnosis and Care for ILD near Cleveland, OH | University Hospitals

  https://www.uhhospitals.org/services/pulmonary-and-sleep-services/conditions-and-treatments/interstitial-lung-disease

  Many ILDs have similar symptoms or look the same on a computed tomography (CT) scan, but its important to pinpoint the specific cause of the disease to determine the best course of treatment. […] University Hospitals offers a comprehensive approach to the diagnosis of ILD, with complete pulmonary function testing, walking evaluations, exercise testing, high-resolution chest computed tomography (HRCT), and in some cases, lung biopsies. […] Biopsies can be done via bronchoscopy, using an innovative technology called cryobiopsy or by a video-assisted thoracoscopic technique. Cryobiopsy gives specialists a much larger tissue sample to test than a traditional bronchoscopy and is less invasive than a surgical biopsy. Our teams discussion allows us to carefully select which patients are suited for which type of diagnostic procedure.

• #41

  https://www.thoracic.org/professionals/career-development/residents-medical-students/ats-reading-list/adult/interstitial-lung-disease.php

  Raj R, Raparia K, Lynch DA, et al. Surgical lung biopsy for interstitial lung diseases. Chest. 2017; 151:1131-40. Reviews the role of surgical lung biopsy in the diagnosis and treatment of interstitial lung disease with specific focus on when a biopsy can be diagnostic as well as when it should be avoided. PMID: 27471113 […] Troy LK, Grainge C, Corte TJ, et al. Diagnostic accuracy of transbronchial lung cryobiopsy for interstitial lung disease diagnosis (COLDICE): a prospective, comparative study. Lancet Respir Med. 2020; 8:171-181. Direct comparisons of cryobiopsy and surgical biopsy results in the same patient are scarce. This study compared diagnostic accuracy by doing sequential procedures in 65 patients with mean FVC 84% predicted. Masked pathologists examined the slides with histopathologic agreement achieved in 70.8% of patients. With incorporation of pathology into multi-disciplinary discussions, diagnostic agreement was 76.4%. Among the 40% (26 patients) with a non-diagnostic cryobiopsy, surgical biopsy provided a confident diagnosis in 26% (6 patients). Of note, the study was not designed to assess the safety of the individual procedures. PMID 31578168

• #42 Diagnosis and management of interstitial lung disease | Translational Respiratory Medicine | Full Text

  https://transrespmed.springeropen.com/articles/10.1186/2213-0802-2-4

  If one needs to obtain invasive testing (bronchoscopy with BAL and/or TBLBx, VATS biopsy), all findings should be reviewed (preferably in a multi-disciplinary fashion) to identify the ultimate diagnosis that best fits with the combination of clinical information, imaging, and invasive testing results. […] A surgical lung biopsy (SLB) obtained via video-assisted thoracic surgery (VATS) or open biopsy is likely to provide an excellent specimen (if properly performed) that shows a histopathologic pattern that can usually be considered to be definitively diagnostic of a specific disease entity. […] However, confirming the diagnosis and differentiating among specific forms of IIP may not be possible without performing SLB. […] The diagnosis and treatment of the various types of ILD present a considerable challenge to clinicians. Nonetheless, a comprehensive clinical evaluation combined with appropriate imaging and diagnostic procedures can achieve a confident diagnosis of a specific type of ILD, and invasive testing with bronchoscopy or SLB may not be required.

• #43 Interstitial lung disease – Wikipedia

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

  A lung biopsy may be required if the clinical history and imaging are not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled out. Surgical lung biopsy or via a video-assisted thoracoscopic surgery (VATS) biopsy is associated with a mortality rate up to 1-2%. A bronchoscopic transbronchial cryobiopsy, in which a camera is introduced into the airways followed by rapid freezing of an area of lung tissue prior to biopsy is associated a lower complication rate and a much lower mortality rate compared to VATS or surgical biopsy with near comparable diagnostic accuracy. […] There is significant overlap of the histopathological and radiologic features of each ILD type making diagnosis challenging; even with lung biopsy, 15% of cases of ILD cannot be classified.

• #44 Interstitial lung disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/diagnosis-treatment/drc-20353113

  Finding the cause of interstitial lung disease can be challenging, and sometimes the cause can’t be found. […] Healthcare professionals must rule out these conditions before making a diagnosis. […] Some of the following tests may be necessary. […] This imaging test is key to the diagnosis of interstitial lung disease. […] A high-resolution CT scan can be especially helpful in figuring out how much lung damage there is. […] Sometimes interstitial lung diseases can be diagnosed only by looking at a small amount of lung tissue in a lab what’s known as a biopsy. […] Your doctor or other healthcare professional may take a tissue sample using one of these methods: […] Although this is a more invasive procedure with potential complications, it’s often the only way to get a large enough tissue sample to make the correct diagnosis. […] Let your pulmonologist know if you’ve had any chest X-rays, CT scans or other tests so the healthcare professional can request the results before your visit. […] The pulmonologist can make a diagnosis by comparing an old imaging test with those of a current test.

• #45 Approach to the adult with interstitial lung disease: Diagnostic testing – UpToDate

  https://www.uptodate.com/contents/approach-to-the-adult-with-interstitial-lung-disease-diagnostic-testing

  Approach to the adult with interstitial lung disease: Diagnostic testing […] The initial evaluation of patients with ILD is aimed at identifying the etiology of the ILD and its severity. The results of laboratory, radiographic, and pulmonary function tests guide the decisions about whether to pursue bronchoalveolar lavage and/or transbronchoscopic, thoracoscopic, or open lung biopsy. […] An overview of the diagnostic testing that is helpful in the diagnosis of ILD will be presented here (algorithm 2). The clinical findings that aid in the diagnosis of ILD and the individual causes of ILD are discussed separately. […] The list of causes of ILD, also known as diffuse parenchymal lung disease, is broad and includes those ILDs that are associated with a broad range of diseases, exposures, and drugs. ILD may also occur as an idiopathic condition. The treatment choices and prognosis vary among the different causes and types of ILD, so ascertaining the correct diagnosis is important. […] A variety of infectious processes cause interstitial opacities on chest radiograph, including fungal pneumonias, atypical bacterial pneumonias, and viral pneumonias. These infections often occur in immunocompromised hosts and are discussed separately.

• #46

  https://www.chestnet.org/learning-and-events/bridging-specialties/timely-diagnosis-for-ild

  Clinicians are focused on one missionbetter outcomes for their patients. […] Bridging Specialties: Timely Diagnosis for ILD brings together pulmonologists and primary care physicians to define a clearer clinician-guided approach to diagnosis for ILDs like pulmonary fibrosis (PF). […] ILDs like PF present with ubiquitous symptoms like dyspnea, cough, and fatigue, which are also common in asthma, COPD, and cardiac diseases. […] In a survey of 50 patients living with PF, the majority of respondents said their diagnosis took 2 or more years. […] There is no cure for ILDs that cause lung inflammation and/or permanent scars, but treatments can slow progression and lessen the severity of symptoms, allowing patients to live longer with a higher quality of life. […] Closing the gap in diagnosing ILDs like pulmonary fibrosis is critical so therapies can be initiated to manage the symptoms and progression of the disease.

• #47 Diagnosis and management of interstitial lung disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4215823/

  The diagnosis and treatment of the various types of ILD present a considerable challenge to clinicians. […] Nonetheless, a comprehensive clinical evaluation combined with appropriate imaging and diagnostic procedures can achieve a confident diagnosis of a specific type of ILD, and invasive testing with bronchoscopy or SLB may not be required.

• #48

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

  There are more than 200 types of interstitial lung diseases (ILD), which are characterized by varied amounts of inflammation, scarring, or both, that damage the ability of the lung to absorb oxygen from the air. […] When a person is diagnosed with PF, sometimes a doctor is able to find the cause of the disease. In many cases a doctor cant find a reason why a person has developed pulmonary fibrosis. […] Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial lung disease. […] Your doctor will use detailed X-rays of your lungs called high-resolution computed tomography (HRCT) and sometimes a lung biopsy to look for a specific pattern of scarring on your lungs, called usual interstitial pneumonia (UIP). A diagnosis of IPF requires that your doctor cannot find a cause and the presence of a pattern of UIP on either HRCT or a surgical lung biopsy sample.

• #49 Diagnosis of interstitial lung diseases: from Averill A. Liebow to artificial intelligence

  https://www.jpatholtm.org/journal/view.php?number=17093

  Histopathologic criteria of usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) were defined over the years and endorsed by leading organizations decades after Dr. Averill A. Liebow first coined the term UIP in the 1960s as a distinct pathologic pattern of fibrotic interstitial lung disease. […] Pathologic examination has been the gold standard to classify ILDs until high-resolution computed tomography (HRCT) became an acceptable method after the second ATS/ERS statement in 2013, which led to a significant decrease in the frequency of lung biopsies for diagnosing ILDs. […] The role of surgical lung biopsy in the diagnosis of IPF has changed over time. […] The 2002 ATS/ERS IIP guidelines stated that biopsy was required for a confident diagnosis of UIP/IPF, but also noted that in more than 50% of cases of suspected IPF, the presence of typical clinical and HRCT features of UIP was sufficiently characteristic to allow a confident diagnosis without the need for surgical lung biopsy.

• #50 Diagnosing Interstitial Lung Disease – ARRS InPractice

  https://arrsinpractice.org/diagnosing-interstitial-lung-disease/

  Given the importance of correctly making a diagnosis of UIP and avoiding overdiagnosis of this entity, radiologists interpreting HRCT should be mindful of the potential pitfalls described in the following sections. […] Numerous CT findings are of a diagnosis other than UIP pattern including the presence of significant ground-glass opacity, marked mosaic attenuation, nodules, and consolidation. Each of these findings points the radiologist toward a diagnosis other than UIP. […] Awareness of common pitfalls in the diagnosis of ILD including the UIP pattern of fibrosis, cystic lung disease, airways disease, diffuse nodular disease, and lung diseases with subtle HRCT findings will better equip the radiologist to contribute to the multidisciplinary diagnosis of patients with ILD.

• #51 Interstitial Lung Diseases | Choose the Right Test

  https://arupconsult.com/content/interstitial-lung-diseases

  A full serologic workup to detect antibodies associated with specific connective tissue diseases is recommended in all patients with ILD. […] Genetic testing should be considered in all patients who have ILD and a family history of ILD. […] Monitoring of ILDs primarily involves lung function testing, evaluation of dyspnea, and imaging.

• #52 Diagnosis, screening, and follow-up of patients with familial interstitial lung disease: Results from an international survey | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-025-03532-0

  A family history of ILD was asked for by 91% of respondents while fewer asked for symptoms related to telomere disorders. […] Genetic testing for familial ILD was available to 59% of the respondents. […] A standardized screening program was present at 16% of the respondents hospitals for first-degree relatives with a pathogenic gene variant. […] Most pulmonologists ask for a family history of ILD and recommend genetic testing for ILD and screening in relatives but have limited knowledge of specific tests and access to genetic MDT. […] Evidence-based guidelines to inform patients, relatives, and physicians are still warranted. […] Most respondents stated that the lack of evidence-based specific treatment options for familial ILD and the lack of guidelines for screening for familial ILD were the current major challenges regarding genetics and ILD. […] The majority considered genetic testing in ILD useful.

• #53

  https://esmed.org/MRA/mra/article/view/2428

  Interstitial lung diseases (ILD) are a complex and diverse group of disorders. ILD are more frequently diagnosed and prevalent now. In this article, diagnosis approach, including new bronchoscopy and genetic tools, and some recently added concepts are revisited, as progressive fibrosing interstitial lung diseases and interstitial lung abnormalities. […] Precise diagnosis of interstitial lung disease is a challenging and probably the most difficult step in the study of these diseases. […] High resolution computed tomography (HRCT) is the fundamental tool for diagnosis. […] In the scenery of diagnostic uncertainty after a exhaustive study, lung biopsy usually is proposed. […] Recent evidence confirms than multidisciplinary committee improve the diagnosis accuracy of ILD. […] New genetic testing (Veracyte, MUC5B promoter risk allele, Telomerase components) to classify ILD are being reported. […] The diagnosis is hard; HP must be considered in every new patient with ILD. […] The approach to diagnosis of HP will depend on the clinical presentation.

• #54 Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data | Nature Communications

  https://www.nature.com/articles/s41467-023-37720-5

  For accurate diagnosis of interstitial lung disease (ILD), a consensus of radiologic, pathological, and clinical findings is vital. […] Management of ILD also requires thorough follow-up with computed tomography (CT) studies and lung function tests to assess disease progression, severity, and response to treatment. […] Here, we utilize RadImageNet pretrained models to diagnose five types of ILD with multimodal data and a transformer model to determine a patients 3-year survival rate. […] When clinical history and associated CT scans are available, the proposed deep learning system can help clinicians diagnose and classify ILD patients and, importantly, dynamically predict disease progression and prognosis. […] High-resolution computed tomography (HRCT) plays a significant role in accurately classifying the various subtypes of ILD.

• #55 Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data | Nature Communications

  https://www.nature.com/articles/s41467-023-37720-5

  Our present study created a joint CNN model by integrating CT images with clinical information. […] Our diagnostic joint model, for example, showed superior sensitivity in identifying UIP and significant improvement in specificity for the diagnosis of CHP, NSIP, and other ILD as compared to human readers. […] The Transformer models using longitudinal radiomics and CT scan features and clinical information were used to predict a 3-year survival rate. […] Our deep learning system has the potential to be integrated into the daily workflow of pulmonologists, rheumatologists, pathologists, and radiologists, where it could serve as a second opinion for a diagnosis of ILD subtypes and dynamically provide personalized insights regarding current and future treatment efficacy using its 3-year survival prediction feature. […] In conclusion, the proposed deep learning system demonstrates high potential in accurately diagnosing five subtypes of ILD.

• #56

  https://journals.lww.com/investigativeradiology/fulltext/2023/08000/artificial_intelligence_and_interstitial_lung.8.aspx

  Interstitial lung disease (ILD) is now diagnosed by an ILD-board consisting of radiologists, pulmonologists, and pathologists. They discuss the combination of computed tomography (CT) images, pulmonary function tests, demographic information, and histology and then agree on one of the 200 ILD diagnoses. […] Radiologists use a combination of CT images, clinical data, and pulmonary function tests to diagnose ILDs. AI-based methods have recently gained significant attention in the field of computational medicine. These advances can be seen in the creation of computer-aided tools for diagnosis, disease monitoring, and accurate prognostication. […] The concept of computer-aided diagnosis (CAD) systems was first introduced in the 1950s. These systems can be defined as software capable of replicating the diagnostic abilities of human experts.

• #57 Diagnosis of interstitial lung diseases: from Averill A. Liebow to artificial intelligence

  https://www.jpatholtm.org/journal/view.php?number=17093

  Recent advances in whole slide imaging (WSI) and artificial intelligence (AI) technology, such as deep learning (DL) based image processing, have opened the door to quantitatively evaluate histopathologic findings. […] AI-assisted diagnosis may offer a crucial contribution by detecting some features that are not discernable by traditional diagnostic methods. […] Once AI-assisted diagnostic tool becomes widely available, it may mitigate the challenges encountered by many hospitals without direct access to experienced pathologists who are well versed in ILD diagnoses. […] The ATS guideline on PPF explicitly stated that this new concept of PPF should not discourage clinicians from their rigorous effort to identify the underlying type of ILD before antifibrotic therapy.

• #58 Improving the Diagnosis of Interstitial Lung Disease – Pulmonology Advisor

  https://www.pulmonologyadvisor.com/cch/improving-the-diagnosis-of-interstitial-lung-disease/

  Given the heterogeneity of interstitial lung diseases (ILD), a correct diagnosis often eludes clinicians. Guidelines have attempted to clarify and streamline the diagnostic process, but it is still common for more than half of patients who present with symptoms to be misdiagnosed. On average, it takes up to 2 years to diagnose idiopathic pulmonary fibrosis (IPF), one of the most common forms of ILD. An accurate diagnosis, which can improve quality of life by directing appropriate therapy, takes time because multidisciplinary assessments clinical, radiologic, and pathologic are essential. Despite advances in imaging, including the advent of high-resolution computed tomography (HRCT), no single test can pinpoint an ILD diagnosis. Even after a patient has undergone a combination of detailed medical and family history analysis, physical examination, serologic testing, imaging, and pulmonary function tests, the diagnosis may still not be obvious. If diagnosis remains elusive, lung biopsy may be considered to establish a specific diagnosis that will aid in assessing prognosis and guiding treatment. Lung biopsy can be performed via bronchoscopy or surgical lung biopsy.

• #59 Improving the Diagnosis of Interstitial Lung Disease – Pulmonology Advisor

  https://www.pulmonologyadvisor.com/cch/improving-the-diagnosis-of-interstitial-lung-disease/

  Though 75% of respondents had visited at least 3 physicians to get a diagnosis, more than half of the patients (55%) surveyed said that they had been misdiagnosed at least once, with 38% receiving at least 2 misdiagnoses prior to receiving their current diagnosis of ILD. Furthermore, 43% of patients had to wait 1 year from the onset of their first symptoms, and 19% waited 3 years for a correct diagnosis. […] Diagnosing ILD is challenging now more than ever during the COVID-19 pandemic yet it is critical for clinicians to ensure their patients receive the most appropriate treatment. Guidelines and registries may help to identify certain characteristics of the heterogeneous disease to enable clinicians to arrive at the correct diagnosis.

• #60 Diagnosing Interstitial Lung Disease (ILD) | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/healthcare-professionals/ild/diagnosing-ild

  This section will help you to understand: […] The diagnosis of ILDs usually takes place in secondary care and is often made by a multidisciplinary team. However, primary care is often where patients will first present with symptoms so its important that HCPs can spot the signs and symptoms of ILDs and know when to refer a patient. […] Up to 55% of ILD patients are initially misdiagnosed, leading to significant delays in treatment. […] This overlap makes it challenging for healthcare professionals to recognise ILD early on, particularly in older adults, who may already have multiple comorbidities. […] Ensure that red flag symptoms such as chest pain, leg swelling, palpitations, haemoptysis and weight loss and recorded and investigated appropriately. […] ILDs will often be unresponsive to treatments for more common respiratory conditions like asthma or COPD, such as inhaled or oral corticosteroids, or bronchodilators, despite good adherence and technique.

• #61 The diagnostic pathway for patients with interstitial lung disease: a mixed-methods study of patients and physicians | BMJ Open Respiratory Research

  https://bmjopenrespres.bmj.com/content/11/1/e002333

  Objectives The diagnostic process for patients with interstitial lung diseases (ILD) remains complex. The aim of this study was to characterise the diagnostic care pathway and identify barriers and potential solutions to access a timely and accurate ILD diagnosis. […] This mixed-methods study identified patient-reported and physician-reported barriers leading to the diagnostic delay of patients with ILD including delayed presentation to primary care, initial misdiagnoses, delayed chest imaging and long wait-times for specialists. […] This mixed-methods study identified patient and system-related factors that contribute to diagnostic delays for patients with ILD, with most delays occurring prior to respirology referral. ILD awareness and education, earlier presentation to primary care, expedited access to chest imaging and earlier referral to respirology may expedite diagnosis.

• #62 Interstitial lung disease – Wikipedia

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

  A lung biopsy may be required if the clinical history and imaging are not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled out. Surgical lung biopsy or via a video-assisted thoracoscopic surgery (VATS) biopsy is associated with a mortality rate up to 1-2%. A bronchoscopic transbronchial cryobiopsy, in which a camera is introduced into the airways followed by rapid freezing of an area of lung tissue prior to biopsy is associated a lower complication rate and a much lower mortality rate compared to VATS or surgical biopsy with near comparable diagnostic accuracy. […] There is significant overlap of the histopathological and radiologic features of each ILD type making diagnosis challenging; even with lung biopsy, 15% of cases of ILD cannot be classified.

• #63 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. […] Regular monitoring for progression of ILDs is vital to inform appropriate care. […] Patients should understand it is important to assess if their ILD is worsening to ensure they receive appropriate care. […] Unclassifiable ILD and idiopathic nonspecific interstitial pneumonia (iNSIP) are not unique diseases, and so when a patient is provisionally diagnosed, further investigation is required to better understand and characterize the type of ILD.

• #64 Interstitial lung disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/interstitial-lung-disease?lang=us

  Interstitial lung disease (ILD) is an umbrella term that encompasses a large number of disorders that are characterized by diffuse cellular infiltrates in a periacinar location. […] This is the provenance of multi-disciplinary teams; clinical, functional laboratory and CT findings are integrated to reach a consensus diagnosis, if possible. Biopsy is only helpful if it might influence treatment decisions and outcome. Surgical lung biopsy carries significant morbidity and mortality; cryobiopsy is available in some centers. […] Follow-up and monitoring will depend on a multitude of factors such as symptoms and other co-morbidities. While no strict consensus recommendations are available in relation to imaging follow-up for patients with progressive fibrosing forms, many consider an HRCT at the patients initial presentation and then every 12-18 months to assess for progression dependent on symptoms etc.

• #65 Interstitial Lung Disease Clinic – Overview – Mayo Clinic

  https://www.mayoclinic.org/departments-centers/interstitial-lung-disease-clinic/overview/ovc-20398498

  Interstitial lung disease describes a large group of lung diseases. Many of them cause a harmful buildup of cells or scarring in the tissue between the air sacs of the lungs. This process results in thickening and stiffness of lung tissue. […] It is important to get an early and accurate diagnosis to limit damage to the lungs. If your doctor suspects you may have interstitial lung disease, you may be referred to a doctor with specialized training in pulmonary medicine and the diagnosis and treatment of interstitial lung disease. […] Together they can develop an individualized treatment plan for you that’s based on a comprehensive evaluation and accurate diagnosis.

• #66 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.

• #67 Diagnosis & Monitoring – Lung Foundation Australia

  https://lungfoundation.com.au/health-professionals/conditions/interstitial-lung-disease/diagnosis-monitoring/

  The diagnosis and classification of ILD is complex. […] Early and accurate diagnosis is important to optimise patient outcomes. […] Detailed clinician history, examination findings, blood testing, lung function and imaging findings are critical to a timely and accurate diagnosis of ILD. […] In certain instances, histopathological sampling via bronchoscopy or surgical or endobronchial lung biopsy is also required to diagnose specific ILDs. […] The gold standard for ILD diagnosis is ultimately via an ILD specific multi-disciplinary meeting (MDM). […] If ILD is suspected or confirmed, early referral to a respiratory physician is recommended for further investigation and management. […] These detailed examinations and investigations to diagnose ILD are typically performed or arranged by respiratory physicians.

• #68 Interstitial Lung Disease (ILD) | University of Michigan Health

  https://www.uofmhealth.org/conditions-treatments/pulmonary/interstitial-lung-disease

  The University of Michigan Interstitial Lung Disease Program leads the field in accurate diagnostic methods, active scientific research and extensive clinical trials. […] Interstitial lung disease is often difficult to diagnose, requiring a range of tests and approaches. There are more than 100 diseases characterized as ILD, so an accurate diagnosis is vital to offering further information and treatment options. […] The University of Michigan has tracked and tested their diagnostic methods, publishing results that set a standard of multidisciplinary approach for interstitial lung disease diagnosis nationwide. […] Physicians from the program are also members of an international task force that are rewriting guidelines for the diagnosis and treatment of interstitial pulmonary fibrosis.

• #69 Interstitial Lung Disease Program | Patient Care

  https://weillcornell.org/interstitial-lung-disease-program

  The Weill Cornell Medicine Interstitial Lung Disease (ILD) Program is dedicated to providing comprehensive and personalized care to our patients living with ILD and pulmonary fibrosis. […] We work with an expert multidisciplinary team of providers to ensure that we provide an accurate diagnosis, offer patients the most up-to-date information about their disease and treatment options, and develop a patient-centered care plan that aligns with our patients values and goals. […] Diagnose and treat patients with all forms of ILD Provide patients with the most up-to-date information about diagnosis and treatment options based on cutting edge research in the field Work closely with our expert colleagues in other departments across Weill Cornell Medicine in order to provide comprehensive multidisciplinary care Develop care plans in partnership with our patients and their families that focus on improving the lives of patients with ILD.

• #70 Interstitial Lung Disease Program | Patient Care

  https://weillcornell.org/interstitial-lung-disease-program

  Interstitial lung disease can take on a variety of forms. Often, understanding if a patient has interstitial lung disease and what caused it can be a challenge. It requires taking a detailed history, performing a thorough physical exam, ordering the appropriate testing, and reviewing imaging and biopsy results (if performed) with a team of specialists with expertise in ILD. […] Our team of ILD providers work closely as part of a multidisciplinary group of specialists in order to provide our patients with an accurate diagnosis.

• #71 Diagnosis and management of interstitial lung disease | Translational Respiratory Medicine | Full Text

  https://transrespmed.springeropen.com/articles/10.1186/2213-0802-2-4

  The complex tasks of making a confident diagnosis of a specific form of interstitial lung disease (ILD) and formulating a patient-centered, personalized management plan in an attempt to achieve remission or stabilization of the disease process can pose formidable challenges to clinicians. […] A well-performed patient history and physical examination provides invaluable information that can be combined with appropriate laboratory testing, imaging, and, if needed, tissue biopsy to reach a confident ILD diagnosis, and high-resolution computed tomography (HRCT) of the thorax is usually a key component of the diagnostic evaluation. […] Successful management of patients with ILD is dependent upon establishing an accurate and specific diagnosis. […] Patients with suspected ILD should have information from the history, physical examination, thoracic imaging, and other testing (e.g. peripheral blood tests, pulmonary function testing) carefully and thoughtfully reviewed to determine whether or not additional procedures are needed and whether such procedures are likely to be helpful in reaching a confident diagnosis.

• #72 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. […] Multidisciplinary discussion amongst pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease is of utmost importance to an accurate diagnosis.

• #73 Diagnostics | Special Issue : Advances in the Diagnosis of Interstitial Lung Diseases

  https://www.mdpi.com/journal/diagnostics/special_issues/Interstitial_Lung_Disease_Diagnosis

  Advances in the Diagnosis of Interstitial Lung Diseases […] The term “interstitial lung diseases” (ILDs) includes a wide spectrum of heterogenous entities with different prognoses and treatment options. Due to the recent progress in the diagnostic and therapeutic landscape of ILD management, an accurate and early diagnosis of a progressive fibrosing ILD is crucial to improve the survival and quality of life of these patients. A diagnostic work-up is, however, still a challenging process, as, according to the ATS/ERS guidelines, it requires an integrated multidisciplinary approach involving pulmonologists, radiologists, and, when classic criteria are not met, also pathologists. Medical history, physical examination, lung function tests, high-resolution computed tomography, bronchoalveolar lavage, and, in case of still inconclusive results, a lung tissue sample are key steps for diagnosis. The huge progress in the field of ILD over the recent decades have coupled with outstanding advancements in technology in interventional pulmonology and radiology, leading to the development of innovative, more accurate and less invasive diagnostic tools. For example, new sophisticated guidance systems have been developed and adopted in interventional pulmonology practice to allow limited and early parenchymal alterations, and these include navigation technology, confocal microscopy, cone-beam computed tomography and other tools. Moreover, in the field of radiology, micro-TC and sophisticated software are now available to identify specific patterns and the relative extent, and they are likely to improve diagnostic accuracy. Finally, molecular analyses to detect usual interstitial pneumonia (UIP) signatures in lung samples have been developed and studies assessing their accuracy are currently ongoing. The main goals of this Special Issue are to update and summarize the innovations in diagnostic approach of ILDs and leaders in the different fields will be invited to provide comprehensive reviews on these topics.

• #74

  https://journals.lww.com/investigativeradiology/fulltext/2023/08000/artificial_intelligence_and_interstitial_lung.8.aspx

  The last point on proportional hazard modelling is that it is prone to overfitting and does not perform well if there is missing data. In future work, it would be useful to see different AI approaches to predict progression visually over time. […] Given the promising results and the even more exciting outlook, it is clear that AI will play a vital role in the future of chest radiology.

• #75 The diagnostic pathway for patients with interstitial lung disease: a mixed-methods study of patients and physicians | BMJ Open Respiratory Research

  https://bmjopenrespres.bmj.com/content/11/1/e002333

  The objective of this mixed-methods study was to characterise the diagnostic pathway of patients with ILD and identify sources of diagnostic delay, using data from chart reviews, surveys and focus groups. […] Patients often recalled a clear diagnosis date, after a lengthy process of tests and appointments. Once given a diagnosis, patients described experiencing confusion, uncertainty and wished for more information. […] This study identified several sources that led to the diagnostic delay of patients with ILD. Advocating for increased awareness, improved access and report standardisation of CT scans, and creation of ILD centres may improve time-to-diagnosis.

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