Q&A: Dr. Andy Limper, MD

Dr. Limper is the Head of the Thoracic Research Unit at the Mayo Clinic in Rochester, Minnesota.

The following questions were submitted from Three Lakes 12-20 subscribers and followers as a part of our Ask the Expert series, where we provide a forum to industry professionals to widen access and education from those who are actively working towards the treatment and future of pulmonary fibrosis.

How does sleep apnea affect PF?  

Patients with idiopathic pulmonary fibrosis have a variety of other illnesses that may also occur including sleep apnea. Effective treatment of these other conditions is important for the best outcomes of patients with IPF. We recently demonstrated that treatment of sleep apnea in patients with IPF is associated with better outcomes including improved mortality. This is likely due to better oxygenation for the heart, lungs, and brain at night in patients with effective treatment for their sleep apnea.  Patients should be encouraged to be tested for sleep apnea and treated.

What are the most promising drugs to treat PF now in a study or soon to be in a study?

A variety of drugs are in trial or will soon be in trial.  This includes novel agents such as Setanaxib and Admilparant.  Other trials are addressing new uses for established lung medications such as Treprostinil which was originally approved for pulmonary hypertension, but is now in trials for patients with pulmonary fibrosis. The use of supplements that are of benefit to telomere biology and fibrosis such as zinc and nicotinamide riboside are in other studies. Finally, work is also underway to test repurposedmedications such as metformin, a long-standing treatment for diabetes that has potential benefit for patients with IPF.  All these agents showed promise in preclinical and early clinical studies.  However, each treatment will require carefully controlled clinical trials to prove whether they provide true benefit for patients with pulmonary fibrosis.

From your perspective at Mayo Clinic, what does truly excellent, comprehensive care for a pulmonary fibrosis patient look like today?

Compassionate and comprehensive care for pulmonary fibrosis requires several things. Of course, effectivediagnosis and treatment of pulmonary fibrosis needs to be based on a strong collaboration of experts, includingpulmonologists, radiologists and pathologists, to render an accurate diagnosis. In addition, comprehensive care requires access to pulmonary rehabilitation, transplantation services, spiritual and emotional care, and palliative care ifthat becomes necessary. Furthermore, excellent comprehensive care requires attention to the whole patient beyond the lung disease. This includes managing other medical illnesses such as heart disease, high blood pressure, and diabetes. The goal is to treat the entire patient not just a single disease.

What are the biggest unanswered scientific or clinical questions that still need to be solved to change outcomes for patients?

There are many important questions that remain. One very important question is what causes idiopathic pulmonaryfibrosis. We now know that this is a disease associated with aging, including damaged chromosomes, and exposure to a variety of factors in the environment. A better understanding of how IPF starts will lead to new approaches to treat this condition. A second important question is how lung fibrosis can be reversed once initiated. It is certainly important to prevent fibrosis from advancingbut finding ways to reverse fibrosis and improve lung function in patients would be even more beneficial.

Many patients want to know what they can do right now: what actions or decisions have the greatest impact on quality of life and disease progression?

First and foremost, the patient needs to become actively engaged in their disease management. This means adopting a healthy lifestyle including excellent balanced diet, adequate rest, and routine exercise. If oxygen is required, it is important to continue to exercise and use oxygen rather than forgoing exercise. In addition, patients need to work with their medical team to decide the best course of action with anti-fibrotic therapy and other treatments and to consider transplantation if that becomes necessary. Early aggressive management is believed to provide the best outcomes for patients with pulmonary fibrosis.

How important is early diagnosis, and what should patients and community physicians watch for to avoid delays in getting to specialty care?

 

Early diagnosis is essential to provide the greatest impactfor patients with idiopathic pulmonary fibrosis and other fibrotic lung diseases. The earlier that a diagnosis can be confirmed, the earlier the treatment can be initiated, including anti-fibrotic medication, and consideration oflung transplantation if necessary. We believe that the earlier the disease is treated, the greater the impact on slowingprogression and improving outcomes.

Clinical trials are critical for progress. How should patients think about participating in research, and how does it move the field forward?

 

Clinical trials are essential to find new medications for patients with pulmonary fibrosis. Without clinical trials the beneficial effects and safety for the three approved pulmonary fibrosis medications would not have been confirmed. Fortunately, most clinical trials now allow standard anti-fibrotic therapy as baseline treatment and the newer agents are tested in addition.  When considering clinical trials, besides the potential benefits and risks for yourself, participation contributes to the identification of future treatments for others with pulmonary fibrosis.

I've been taken Ofev since 2015 now that there is a new drug Jascayd available should I consider adding to the regimen?

There is some information that treatment with Jascayd in addition to Ofev provides benefit. We call that synergy. However, at this time, it’s not yet certain whether the insurance companies will pay for both Ofev and Jascayd at the same time. It would be worth looking into your policy to see whether the use of both medications is permitted. However, be aware that taking both medications could result in increased side effects.

My eGFR has dropped from 97 in 2022 to 66 in 2025 wouldn't adding another med just add strain?  

 

I am sorry to hear about your reduction in kidney function. Some medications reduce kidney function, but not all medications have this effect. When considering the additional of other medications, the potential for kidney toxicity should be discussed with your doctor.  In addition, you may need the expert opinion of a kidney specialist such as a nephrologist.

Looking ahead 5–10 years, what do you believe will most dramatically change the future for people living with pulmonary fibrosis and what gives you the most hope?

I believe within the foreseeable future of five years, we should be able to totally stop the progression of fibrosis with a combination of anti-fibrotic drugs. It is also my hope that over the next 10 years we will find means to reverse pulmonary fibrosis and restore lung function without lung transplantation. We are learning a great deal about lung regeneration and the cells responsible for lung repair during pulmonary fibrosis. This research should lead to new ways to reverse the fibrotic process and improve lung function.