Idiopathic Pulmonary Fibrosis – Diagnosis

Transcript

“The diagnosis of IPF can be difficult to make and requires a detailed clinical history and exam blood work to rule out other diseases that could cause pulmonary fibrosis. CT, chest imaging, and pulmonary function testing to assess the disease severity. The first step is to rule out other types of interstitial lung disease with a detailed review of the patient’s medical history. Remember, idiopathic pulmonary fibrosis is a disease of unknown origin. So it’s important to rule out other causes. Other forms of ILD include occupational diseases, hypersensitivity pneumonitis, drug induced lung disease, and autoimmune disease. Interestingly, interstitial lung disease can be the first manifestation of autoimmune disease and consult with a rheumatologist is sometimes needed to definitively rule out autoimmune diseases. After a detailed history and physical is obtained, we then proceed with the high resolution CT chest. The CT chest will show the extent of lung fibrosis and give clues to the type of interstitial lung disease that is present.

If the CT scan and medical history are both suggestive of IPF, then the diagnosis of IPF can be made. The high resolution CT scan will show the extent of lung fibrosis and give clues to the type of interstitial lung disease that is present. If the CT scan and medical history of both suggestive of IPF, then the diagnosis of IPF can be made. If the CT scan is not conclusive for IPF, a surgical lung biopsy or bronchoscopic cryo lung biopsy will then be needed to be performed. The combination of a patient’s medical history, CT scan, plus / minus the biopsy will usually provide the necessary information to confirm a final diagnosis of IPF. In addition to those diagnostic tests, the six-minute walk test and pulmonary function testing are performed serially over time to monitor for disease progression. A decline in the six-minute walk test and pulmonary function studies can suggest that the lung is getting worse and can prompt starting anti-fibrotic medications.”