Interstitial Lung Disease

Idiopathic Pulmonary Fibrosis

Signs and Symptoms
Diagnosis
Treatment

Diagnosis

Diagnosing idiopathic pulmonary fibrosis, or IPF, requires input from pulmonologists, radiologists and in many cases, pathologists experienced in evaluating patients with interstitial lung disease. A face-to-face discussion among these various specialists is often necessary to make an accurate diagnosis.

To determine if you have IPF, your doctor will start by conducting a thorough medical history and physical examination. The medical history will include discussing other medical problems you have that could be related to lung scarring, such as connective tissue diseases, and reviewing any medications you're taking. The physical exam will include listening to your chest with a stethoscope to check for a crackling sound and carefully examining your skin and joints.

In addition to a thorough medical history and physical exam, your doctor may conduct the following tests:

• Pulmonary Function Test (PFT) — This test involves a series of breathing exercises that measure the airflows, volume of air in your lungs and ability of your lungs to extract oxygen from the air. This allows your doctor to assess the function of your lungs.
  
  
• High Resolution Computed Tomography (HRCT) — This is a special type of CT scan that provides your doctor with high-resolution images of your lungs. Images are taken in several different ways, including lying on your back (supine), lying on your chest (prone) and while breathing air out of your chest (dynamic expiration). These images are extremely valuable in determining whether or not you have IPF. Having an HRCT is very similar to having a regular CT scan; they both take only a few minutes.
  
  
• Blood Tests — Also called serologies, blood tests may be ordered to look for evidence of connective tissue diseases such as rheumatoid arthritis or scleroderma. Some patients with these connective tissue diseases develop lung problems before the more typical signs and symptoms appear.
  
  
• Six-Minute Walk Test — This test evaluates the distance you can walk within six minutes and the oxygen saturations, measured by finger or ear probe, you achieve while walking.
  
  
• Bronchoscopy — In this test, the doctor passes a flexible fiberoptic scope, about the diameter of a pencil, into the lungs to take fluid and tissue samples. This test doesn't require an overnight stay in the hospital. It's unclear if bronchoscopy provides any benefit in diagnosing IPF, but it may be performed in certain circumstances.
  
  
• Surgical Lung Biopsy — Some patients need this test to definitively diagnose IPF. A cardiothoracic surgeon performs the surgical lung biopsy, usually with small tools and cameras through one-inch-long incisions. Patients will need to stay in the hospital for a few days.

Reviewed by health care specialists at UCSF Medical Center.
Last updated June 11, 2009