Question: What is the role, if any, for pulmonary rehabilitation in patients with IPF?

Answer: We are learning more and more about the benefits of exercise in patients with many forms of lung and heart disease. Most of the data on the benefits of pulmonary rehabilitation (PR) have come from the COPD literature where it is well established. Indeed, it is mandated in this population prior to lung volume reduction surgery and transplantation. PR is generally required for lung transplant candidates. There is also emerging data in patients with interstitial lung disease and IPF about the benefits of PR. Interestingly, a recent study of patients with pulmonary arterial hypertension showed that PR improved patients' six minute walk distance more than most commonly prescribed medications. A comprehensive approach, including ongoing education, dietary consultation, and adaptive breathing strategies enables a global improvement in quality of life, in addition to gains in functional status. With a lack of effective medical therapies for IPF, PR is one strategy that physicians can prescribe that could be of significant benefit.

Question: I have a patient who for unrelated reasons underwent a CAT scan of the chest. The radiologist's report mentions some "subpleural scarring which is probably chronic." My question is, how do I know this is not a condition like IPF and what do I do with this information?

Answer: This is not an uncommon scenario. As more CAT scans of the chest are obtained, unsuspected findings crop up. Radiologists are very attentive to nodules that might herald an underlying carcinoma, and usually recommend appropriate follow-up in their reports. Unfortunately, similar vigilance is often not applied to changes consistent with scarring which are often assumed to be benign. Some of these cases may be early IPF, which has a prognosis similar to that of lung cancer. In this particular case, without seeing the CAT scan, there is no way to know if the changes are consistent with early IPF or not. Indeed, data on what "early IPF" looks like on CAT scan are lacking. Most of our studies and hence definition of a "typical" CAT scan for IPF are based on populations that have included patients with some degree of honeycombing in the lungs. However, radiographic honeycombing usually correlates pathologically with advanced fibrosis and may not be observed earlier in the disease course. It is also important to consider the whole clinical picture, including age, functional status and the presence or absence of any comorbidities.

It might be worthwhile repeating the CAT scan with supine and prone views to verify that the changes persist and are not due to dependent atelectasis. It would also be worthwhile to obtain some baseline lung function data. One could also take a "watch and wait" approach and plan on a follow-up CAT scan in 3-6 months. If the patient is young and robust, I would be inclined to recommend a surgical lung biopsy sooner rather than later. One could question the value of a lung biopsy in an asymptomatic patient, since we don't have any effective therapies for IPF. However, if the patient is proven to have IPF, they might be a candidate for one of the available treatment trials. Also, counseling about the disease and its prognosis as well as an early referral for a lung transplant evaluation would be appropriate.

Question: I have a 63-year-old male patient with IPF who is on oxygen therapy. He has been turned down by our regional transplant center for a lung transplant because he had lung cancer 3 years ago. His left lung was removed and he has been disease free since then. What is the rationale for excluding him from the transplant list?

Answer: All transplant programs exclude patients with potential contraindications from the transplantation list. Cancer is a contraindication to lung transplantation for 2 reasons. First, not enough time has passed to be confident of no recurrence. This window is generally 2 years, but longer in some instances such as lung cancer, where the recommended waiting time is 5 years. The second reason is that transplantation requires immunosuppression to prevent rejection of the lung. Immunosuppression increases the likelihood that residual cancer cells will proliferate and spread. Therefore, the transplant and immune suppression might do more harm than good by enabling the uncontrolled spread of the malignancy.