Pulmonary fibrosis (PF) is a disease that affects the respiratory system by the thickening and stiffening of lung tissue, which ends up as scar tissue. In cases when physicians are unable to identify the root cause of the disease, it is called idiopathic pulmonary fibrosis (IPF).
The symptoms of pulmonary fibrosis include shortness of breath (dyspnea), dry cough, fatigue, unexplained weight loss, and aching muscles and joints. Given the similarity of pulmonary fibrosis symptoms and symptoms of other lung disease, diagnosis can be difficult. However, techniques and exams exist to help confirm a pulmonary fibrosis diagnosis.
First steps for PF diagnosis
According to the National Institutes of Health (NIH): “Seeking medical help as soon as you have symptoms is important. If possible, seek care from a pulmonologist. This is a doctor who specializes in diagnosing and treating lung problems. Your doctor will diagnose IPF based on your medical history, a physical exam, and test results. Tests can help rule out other causes of your symptoms and show how badly your lungs are damaged.”
When a patient experiences symptoms that can indicate pulmonary fibrosis, the diagnosis process starts by analyzing the patient’s medical history. Questions include: age, history of smoking, environmental elements in the air at the job or elsewhere that might irritate the lungs, hobbies, history of legal and illegal drug use, other potential medical conditions, family medical history, and how long the symptoms have been experienced.
Additionally, the physician will order a series of tests and examinations.
Tests to diagnose pulmonary fibrosis
There is no single test able to correctly diagnose pulmonary fibrosis, which is why a group of diagnostic tests are often required to make a full diagnosis.
Some tests include:
- A chest X-ray, which generates a picture of the chest structures such as the heart and lungs and allows physicians to search for shadows that indicate scar tissue.
- A high-resolution computed tomography (HRCT) scan is a type of X-ray with higher precision and more detail.
- Lung function tests are also used to measure the amount of air that can be blown out of the lungs after a deep breath as well as the speed of breath, which help understand the damage in lungs.
- A pulse oximetry test consists of attaching a small sensor to the finger or ear, which uses light to estimate the amount of oxygen in the blood.
- An arterial blood gas test is also used to measure the levels of oxygen and carbon dioxide by collecting and analyzing a sample of blood from an artery, usually in the wrist. The arterial blood gas test is considered to be more accurate than the pulse oximetry test.
- During an exercise test, patients walk or pedal for a few minutes, which is used to reveal how the lungs move oxygen and carbon dioxide in and out of the bloodstream.
- A skin test for tuberculosis may be requested to exclude the possibility of tuberculosis.
Diagnosis of PF through biopsy
According to the NIH: “For a lung biopsy, your doctor will take samples of lung tissue from several places in your lungs. The samples are examined under a microscope. A lung biopsy is the best way for your doctor to diagnose IPF. This procedure can help your doctor rule out other conditions, such as sarcoidosis, cancer, or infection. Lung biopsy also can show your doctor how far your disease has advanced. Doctors use several procedures to get lung tissue samples.”
Biopsy options include:
- A video-assisted thoracoscopy, which is the most common procedure, consists of the insertion of a small tube, with a camera at the end, through the ribs and into the chest.
- With a bronchoscopy, a flexible tube is inserted through the nose or mouth, down the throat and into the airways.
- The bronchoalveolar lavage is a procedure that starts with a bronchoscopy and continues with the injection of a small amount of saline into the lungs to wash the fluid and bring up cells from the area around the air sacs.
- A thoracotomy consists of the removal of a few small pieces of lung tissue through a cut in the chest wall between the ribs.
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