Diagnosing Idiopathic Pulmonary Fibrosis: What to Expect

Common Diagnostic Tests and Why Diagnosis Is Difficult

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Since the symptoms of idiopathic pulmonary fibrosis (IPF) mimic those of several heart and lung conditions, it often takes an extended period of time to diagnose the disease. During this period of time, frequently a year or two, patients often see several doctors in an attempt to find the reason for their symptoms.

Common IPF Symptoms

Like some other lung diseases, such as COPD, there is often significant damage to the lungs before symptoms appear.

 These symptoms may include:

  • Shortness of breath, especially with activity, is usually the first symptom.  With time this breathing difficulty worsens so that shortness of breath occurs even at rest.
  • A persistent cough which is usually dry (non-productive)
  • Shallow, rapid breathing
  • Fatigue
  • Losing weight without trying (unexplained weight loss)
  • Clubbing - Clubbing is a condition in which the fingers and toes become enlarged at the end, resembling an upside-down spoon.
  • Muscle and joint aches

Screening for IPF

Currently, there is not a screening test for IPF, and diagnosis is based on people presenting with symptoms. The technique for screening for lung cancer, however, may pick up IPF in people for who screening is approved and recommended.

Diagnosis and Staging

The diagnosis of IPF often takes some time, with many people going a year or two and seeing multiple physicians before the diagnosis is made.

The reason for this is that the early symptoms of IPF closely mimic those for many other conditions, including heart disease. Many people undergo an extensive cardiac workup before being diagnosed based on common initial symptoms.

Some tests your doctor may order include:

  • High-resolution CT Scan: IPF is usually diagnosed based on typical findings seen on a high-resolution CT scan of the chest. On a CT scan, the fibrosis appears in what radiologist refer to as a honeycombed pattern.
  • Lung biopsy: There are times when a CT scan alone can confirm the diagnosis of IPF, but if the diagnosis is in question a lung biopsy may be recommended. This may be done as part of a bronchoscopy, through a small incision in the chest (thoracoscopy) or by a thoracotomy. The biopsy is done primarily to rule out other potential causes of lung disease such as lung cancer or sarcoidosis.
  • Other tests that aid in diagnosis and staging: A combination of an exam and other tests are usually done both to aid in diagnosis, determine how well the lungs are functioning, and to figure out how far the disease has advanced. Some of these include:
    • History and physical exam.
    • Spirometry - Spirometry is a test done looking at how much air is forcefully exhaled over a period of one second, and is an indication of lung function.
    • Other pulmonary function tests
    • Oximetry
    • Arterial blood gases - Both oximetry and ABG's are done to evaluate the oxygen content of the blood.
    • Testing for tuberculosis
    • Blood tests - Research is looking into biomarkers which will help to diagnosed the disease in the future.
  • Genetic Predisposition: Idiopathic pulmonary fibrosis may run in families and certain mutations (on chromosome 11) have been found to be common in familial IPF.

    A Word From Verywell

    Whether or not your case of IPF takes some time to diagnose, once you are diagnosed know that you have treatment options available, and more are being researched and developed. Along with taking control of certain lifestyle factors, you can continue to live well with idiopathic pulmonary fibrosis.

    Sources:

    Antoniou, K., Tomassetti, S., Tsitoura, E., and C. Vancheri. Idiopathic pulmonary fibrosis and lung cancer: a clinical and pathogenesis update. Current Opinions in Pulmonary Medicine. 2015 Sep 18. (Epub ahead of print).

    de Boer, K., and J. Lee. Under-recognised comorbidities in idiopathic pulmonary fibrosis: a review. Respirology. 2015 Sep 13. (Epub ahead of print).

    Oldham, J. et al. TOLLIP, MUC5B and the Response to N-acetylcystein Among Individuals with Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 2015 Sep 2. (Epub ahead of print).

    O’Riordan, T., Smith, V., and G. Raghu. Development of Novel Agents for Idiopathic Pulmonary Fibrosis: progress in target selection and clinical trial design. Chest. 2015 May 8. (Epub ahead of print).

    Raghu, G. et al. Diagnosis of idiopathic pulmonary fibrosis with high-resolution CT in patients with little or no radiological evidence of honeycombing: secondary analysis of a randomized, controlled trial. Lancet Respiratory Medicine. 2014. 2(4):277-84.

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