Clinical Pearls for the Management of IPF

Steven D. Nathan, MD, FCCP: Some practical tips to share with community physicians. IPF [idiopathic pulmonary fibrosis] can be a difficult diagnosis. Sometimes it’s black and white, clearly IPF or clearly not. But most of the patients fall in that gray zone of uncertainty. What I would say is if you’re uncertain, and this might be self-serving, contact your local ILD [interstitial lung disease] center. Get a second opinion and see what they have to say. I look to my colleagues all the time in our clinic, and I say, what do you think of this CT, or what do you make of this or that?

Talking among ourselves and putting these patients before multidisciplinary discussion, which typically happens at ILD centers, can be very important for validation of the diagnosis. Because probably the most difficult aspect of IPF is to make an accurate diagnosis first. The value of sending folks to ILD centers is that they can be looked at for clinical trials. Many ILD centers, such as ours, are transplant centers as well.

It becomes 1-stop shopping. You can get validation of the diagnosis concurrently. If you don’t want to start the therapy yourselves and leave it to the ILD docs, you can do it. They get looked at for clinical trials, and they get evaluated even in a cursory fashion as a potential lung transplant candidate.

To the community docs out there, don’t feel like you’re flying alone. I think I speak for all the ILD centers out there. We’re only too happy to partner with you in taking care of your patients and making sure they have the best possible outcomes.

Fernando J. Martinez, MD, MS: I believe strongly that the primary care clinician should be the principal player in guiding patients and their caregivers through any disease process. I’ve had strong bias all along. I trained as a primary care physician years ago. My sense is that the primary care clinician needs to understand these disorders because of their severity and the implication for patient and their family members. That includes having a high index of suspicion of when this disease may be present.
So there have been a series of studies that are lovely and demonstrate that an older person presenting with a chronic cough is a tip-off to the primary care clinician that they should consider these disorders, and IPF in particular, as a potential diagnosis. Because it’s our suspicion that earlier diagnosis, particularly with these effective drugs, is likely going to be better in the long term. It makes sense, and the data would support that. Trying to work with our primary care clinicians to have a higher index of suspicion of when these disorders are present is crucial. The same thing for our radiologists, because we have a sense that early changes on a CT scan can be very instructive with regard to the early diagnostic process.

That interaction has improved. If you think about how we consider optimizing the patient’s quality of life and survival, the importance of being able to address the potential tolerability issues of the drugs and how to manage them. The comorbid conditions and their interaction. In the middle of all that is a primary care clinician.

When we develop therapeutic plans with our patients and their caregivers, when we have a disease-management plan, it’s immediately transmitted to their primary care clinician, ideally by a phone call or a video chat, so the primary care clinician is fully aware of where we are at each stage and can aid the patient in making decisions at each stage.

The primary care clinician is a crucial player from the early diagnosis to the management at the end stage of disease. They are the quarterback in most of what happens with these patients.

Transcript Edited for Clarity