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Tests and diagnosis
By Mayo Clinic staffDiagnosing pulmonary fibrosis can be extremely challenging. The difference between idiopathic and nonidiopathic forms of the disease isn't always clear, and the naming and classification systems for both have historically been confusing and controversial. In addition, many medical conditions, including chronic obstructive pulmonary disease (COPD), asthma and even heart failure, can mimic pulmonary fibrosis, so doctors must rule these out before making a definitive diagnosis.
A complete medical history, physical exam and even a chest X-ray aren't enough to diagnose pulmonary fibrosis, but they can help exclude other conditions. For that reason, you may have tests such as:
- Chest X-ray. This will usually show the scar tissue typical of pulmonary fibrosis and is useful for following the course of the illness and treatment. Occasionally, the chest X-ray is normal and further tests are required to explain the unexplained shortness of breath (dyspnea).
- High-resolution computerized tomography (HRCT) scan. This provides sharper and more-detailed images of your lungs than do conventional CT scans or regular chest X-rays.
- Pulmonary function tests. These tests determine how well your lungs work overall. They measure how much air your lungs can hold, and how quickly you can move air in and out of your lungs.
- Oximetry. This simple test uses a small device placed on one of your fingers to measure the oxygen saturation in your blood. Oximetry can serve as an easy way to monitor the course of the disease, sometimes more accurately than a chest X-ray can.
- Exercise stress test. An exercise test on a treadmill or stationary bike may be used to monitor your lung function when you're active.
Often, though, pulmonary fibrosis can be definitively diagnosed only by examining a small amount of lung tissue (biopsy) in a laboratory. The tissue sample may be obtained in one of these ways:
- Bronchoscopy (transbronchial biopsy). In this procedure, your doctor removes very small tissue samples — generally no larger than the head of a pin — using a small, flexible tube (bronchoscope) that's passed through your mouth or nose into your lungs. The risks of bronchoscopy are generally minor — most often a sore throat and temporary hoarseness from swallowing the bronchoscope — but the tissue samples are sometimes too small for an accurate diagnosis.
- Bronchoalveolar lavage. In this procedure, your doctor injects salt water (saline) through a bronchoscope into a section of your lung, and then immediately suctions it out. The solution that's withdrawn contains cells from your air sacs. Although bronchoalveolar lavage samples a larger area of the lung than other procedures do, it may not provide enough information to diagnose pulmonary fibrosis.
- Surgical biopsy (video-assisted thoracoscopic surgery). In some cases, your doctor may recommend a surgical biopsy. Although this is a more invasive procedure with potential complications, it's often the only way to obtain a large enough tissue sample to make an accurate diagnosis. During the procedure, surgical instruments and a small camera are inserted through two or three small incisions between your ribs. The camera allows your surgeon to view your lungs on a video monitor while removing tissue samples from your lungs. Because video-assisted thoracoscopic surgery doesn't require cutting through a rib, you're likely to have less pain and to heal more quickly than you are with traditional open-lung surgery.
- Garantziotis S, et al. Serum inter-alpha-trypsin inhibitor and matrix hyaluronan promote angiogenesis in fibrotic lung injury. American Journal of Respiratory and Critical Care Medicine. 2008;178:939.
- Interstitial lung disease and pulmonary fibrosis. American Lung Association. http://www.lungusa.org/site/apps/nlnet/content3.aspx?c=dvLUK9O0E&b=4294229&ct=3052333. Accessed Jan. 13, 2008.
- King TE. Clinical advances in the diagnosis and therapy of the interstitial lung diseases. American Journal of Respiratory and Critical Care Medicine. 2005;172:26.
- What are the signs and symptoms of idiopathic pulmonary fibrosis? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_signsandsymptoms.html. Accessed Jan. 13, 2009.
- How is idiopathic pulmonary fibrosis treated? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_treatments.html, Accessed Jan, 13, 2009.
- McKay S, et al. Patient information handbook. Chicago, Ill.: American Pulmonary Fibrosis Foundation; 2004. http://www.pulmonaryfibrosis.org/patient.pdf. Accessed Jan 13, 2009.
- What causes idiopathic pulmonary fibrosis? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_causes.html. Accessed Jan. 14, 2009.
- Idiopathic pulmonary fibrosis: Diagnosis and treatment: International consensus statement. American Journal of Respiratory and Critical Care Medicine. 2000;161:646.
