Idiopathic pulmonary fibrosis (IPF) is an inflammatory lung disorder of unknown origin characterized by abnormal formation of fibrous tissue between the tiny air sacs or ducts of the lungs. This is an extremely fatal disease, usually occurs in adult individuals of between 50 and 70 years of age, particularly those with a history of cigarette smoking, and affects more men than women.
People who are suspected to have the lung disease idiopathic pulmonary fibrosis may not have to undergo biopsies, as clinical and radiological results should provide a confident diagnosis, according to a new study published in The Lancet Respiratory Medicine journal. There is no cure for this and most people will only survive for 3 to 5 years following diagnosis. The disease causes scarring of the lungs, which worsens over time, until the lungs are unable to supply the required levels of oxygen to the rest of the body.
High resolution computed tomography is used to identify IPF, as the disease often leaves a characteristic "honeycomb" scarring pattern, known as usual interstitial pneumonia. Currently, when IPF is suspected in a patient but there is no sign of the UIP pattern; international guidelines recommend that a biopsy be taken to confirm the diagnosis.
But taking a biopsy from the lung is a risky procedure. Surgical lung biopsy is associated with substantial risks and many patients are too elderly, sick, and/or have co morbid conditions to tolerate the invasive procedure. A confident diagnosis of IPF is needed to ensure that patients are well informed of the poor prognosis associated with IPF, are treated with the most appropriate therapies, consider participation in clinical trials of new therapies, and to identify those most suitable for lung transplantation.
The researchers wanted to see if IPF could be accurately diagnosed in patients who do not exhibit UIP from HRCT scans, without the need for a biopsy. To test this, the team showed expert radiologists and pathologists HRCT scans of 315 patients who had previously been suspected of IPF - but who presented with little or no visible honeycombing. The experts were able to diagnose with 94% accuracy the patients who had IPF but whose scans exhibited little or no scarring. The researchers knew that the experts' diagnoses were accurate, as they also had access to results of the patients' biopsies.
These findings suggest that when a team of multidisciplinary experts in interstitial lung disease at a regional center (that includes a chest radiologist and a pulmonologist) work together to interpret possible UIP pattern on high-resolution CT in a patient suspected to have IPF, surgical lung biopsy might not be necessary to reach a diagnosis of IPF. However, because of the retrospective nature of the study and the way that cases were selected - results in day-to-day clinical settings may not be the same. But experts predict that the role of lung biopsy will diminish, with lung disease diagnoses more likely to come from non-invasive techniques such as multidisciplinary meetings in conjunction with HRCT.
People who are suspected to have the lung disease idiopathic pulmonary fibrosis may not have to undergo biopsies, as clinical and radiological results should provide a confident diagnosis, according to a new study published in The Lancet Respiratory Medicine journal. There is no cure for this and most people will only survive for 3 to 5 years following diagnosis. The disease causes scarring of the lungs, which worsens over time, until the lungs are unable to supply the required levels of oxygen to the rest of the body.
High resolution computed tomography is used to identify IPF, as the disease often leaves a characteristic "honeycomb" scarring pattern, known as usual interstitial pneumonia. Currently, when IPF is suspected in a patient but there is no sign of the UIP pattern; international guidelines recommend that a biopsy be taken to confirm the diagnosis.
But taking a biopsy from the lung is a risky procedure. Surgical lung biopsy is associated with substantial risks and many patients are too elderly, sick, and/or have co morbid conditions to tolerate the invasive procedure. A confident diagnosis of IPF is needed to ensure that patients are well informed of the poor prognosis associated with IPF, are treated with the most appropriate therapies, consider participation in clinical trials of new therapies, and to identify those most suitable for lung transplantation.
The researchers wanted to see if IPF could be accurately diagnosed in patients who do not exhibit UIP from HRCT scans, without the need for a biopsy. To test this, the team showed expert radiologists and pathologists HRCT scans of 315 patients who had previously been suspected of IPF - but who presented with little or no visible honeycombing. The experts were able to diagnose with 94% accuracy the patients who had IPF but whose scans exhibited little or no scarring. The researchers knew that the experts' diagnoses were accurate, as they also had access to results of the patients' biopsies.
These findings suggest that when a team of multidisciplinary experts in interstitial lung disease at a regional center (that includes a chest radiologist and a pulmonologist) work together to interpret possible UIP pattern on high-resolution CT in a patient suspected to have IPF, surgical lung biopsy might not be necessary to reach a diagnosis of IPF. However, because of the retrospective nature of the study and the way that cases were selected - results in day-to-day clinical settings may not be the same. But experts predict that the role of lung biopsy will diminish, with lung disease diagnoses more likely to come from non-invasive techniques such as multidisciplinary meetings in conjunction with HRCT.
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