Diagnosing Pulmonary Fibrosis Early Is Key

Author:
Albert Rizzo, MD 
Christiana Care Pulmonary Associates
American Lung Association 

Citation:
Rizzo A. Pulmonary fibrosis: confirming your clinical suspicion [published online December 19, 2018]. Pulmonology Consultant.

This commentary is based on a podcast with Dr Rizzo. Listen to the full audio file here.

Having a level of suspicion and knowing what questions to ask the patient—coupled with a physical examination—are key to diagnosing pulmonary fibrosis (PF).

Diagnosis
When initially diagnosing PF, a healthy level of suspicion that the disease may be existing in the patient is important, because PF can be very subtle in its presentations and can often be confused with more common pulmonary diseases, such as chronic bronchitis, chronic obstructive pulmonary disease (COPD), and asthma. 

In addition to using clinical skills, patient history, and physicals to make the diagnosis, a high-resolution computed tomography (HRCT) should be performed.

We know now that a high-resolution scan that is done specifically for interstitial lung disease, which idiopathic pulmonary fibrosis (IPF) is, can make the diagnosis in a fair number of cases and, therefore, save the patient from a more invasive procedure such as a lung biopsy. 

HRCT is something that radiologists are also becoming very well attuned to using to help diagnose the different PF that can present. 

Having the suspicion, having the right symptoms and physical examination, and getting an HRCT helps health care providers decide whether they have enough information to diagnose IPF or whether they need to do other studies or biopsies to confirm their suspicions. These steps are best done through a multidisciplinary approach.

Making the diagnosis early is imperative. This can be achieved by raising the level of suspicion not only in the pulmonary community, but also in the primary care community where patients may first present.

Treatment, Finding a Cure >>

Treatment
For many years, other than supportive care, there was very little we could do from a treatment standpoint for patients with PF, especially those with IPF. Patients would be given oxygen as their disease progressed and their functional status declined. 

Fortunately, several years ago, 2 new medications—nintedanib and pirfenidone—were approved to help treat and slow the progression of this disease, which is why it has become important to make the diagnosis early before there has been too much loss of lung function. Of course, the medications do not work in everybody, but unless a patient is given the opportunity of being on one of these two drugs, you will not know for sure. 

There is a lot of research now into other drugs that may impede the decline in lung function in different ways; we might find that using multiple drugs may be better than using one drug.

Without treatment, the average survival with an IPF diagnosis is between 3 to 5 years—a rate that is very similar to that of advanced lung cancer. Hopefully, with drugs that can slow the progression, we are going to see an improvement in that mortality rate. We are trying to make significant strides in improving outcomes in these patients.   

Finding a Cure
While a cure might not yet be on the horizon, there is a lot going on regarding research into one, so there is hope.

We know that IPF most likely has a genetic component. We also know there are associations with different environmental exposures, particularly smoking. That is not saying these environmental exposures cause IPF, but we do know it occurs in patients who have had certain exposures and certain genetics. 

Further, we know that the process that goes on is, unfortunately, very complex—there are a host of different mediators or molecules that take place in the process of healing the lung. And we think that the stiffening and scarring of the lung is an abnormal healing process that lays down collagen fibers or abnormal fibers in a way that makes the lung stiffer and makes those small air sacks—which normally have a very thin wall—thicker, making it harder for oxygen to get into the bloodstream. 

Through it all, it is very important to have a good dialogue with your patients so that they can live as successfully as possible with IPF.

Albert Rizzo, MD, is the chief medical officer at the American Lung Association and a pulmonologist at Christiana Care Pulmonary Associates in Delaware.