Hiking was the first activity that became hard for Jim Hartmann, who had led an active lifestyle traveling, hiking and canoeing. Next, it was climbing stairs at work. He continued struggling for years, thinking his breathlessness was a sign of aging. Four years ago, Jim noticed that even light activities, such as gardening, were leaving him exhausted. He also had endless bouts of allergy-like symptoms and was finally diagnosed with pneumonia. Jim had three rounds of antibiotics which provided little or no relief after which a chest X-ray was taken showing some minor abnormalities and ultimately a CT scan was done. When the results came back, his doctor read him a summary of the report and finished by saying:   "I don’t know what that is."

Jim was referred to a pulmonologist and, eventually, diagnosed with idiopathic pulmonary fibrosis (IPF).

"My general practitioner is a really good doctor," Jim said. "When I talked with him again after months of diagnosis with the specialists, he admitted that in his 30 years of seeing thousands of patients, he’d never had one with IPF."

IPF, Jim learned, was a lung disease occurring spontaneously and insidiously, generally after the age of 50 and resulting in widespread, progressive scar tissue in the lower parts of both lungs. These scars distort the lungs normal architecture, making them stiffer and thereby requiring more effort to breathe in and out, creating the sensation of shortness of breath. The scar tissue also inserts itself between the lungs' air sacs (alveoli) and tiny blood vessels (capillaries), which surround the alveoli, and it becomes progressively harder to get oxygen into the bloodstream and from there to the rest of the body.

There are a number of situations for which we can determine a specific cause of pulmonary fibrosis. It can be caused by several rheumatic conditions such as rheumatoid arthritis, reactions to certain medications or radiation treatments and occupational dusts such as asbestos. In IPF, the word 'idiopathic' indicates that we don't yet know the cause(s) or the actual mechanisms by which the disease progresses.

Jim's story is typical. Estimates vary but approximately 140,000 Americans live with IPF and 50,000 new cases are diagnosed each year. These numbers meet the criteria of the National Institutes of Health definition for a rare disease (as it affects less than 200,000). IPF is found primarily in adults over the age of 50 and more often in men. The incidence of new cases is rising in part because of a growing elderly population in the U.S. and more awareness and more frequent detection.

Because IPF is rare, most primary care physicians are unfamiliar with it and therefore it is usually best diagnosed and managed by pulmonologists and centers specializing in their management.

IPF symptoms are similar to those of other respiratory diseases, such as chronic obstructive pulmonary disease (COPD), which is much more common. Patients with these symptoms—shortness of breath, fatigue and a dry and cough—often assume they are getting older and are out of shape, thereby delaying diagnosis. The scarring may spread relatively rapidly and more lung function is lost before any treatment can be initiated. Eventually, the worsening of symptoms and a CT scan will lead to a diagnosis, but by then, for many patients, the disease is in the advanced stage.

At present, there is no cure for IPF but there is symptomatic relief which include: pulmonary rehabilitation, oxygen therapy, education and prevention of exacerbations with appropriate vaccinations (e.g., influenza and pneumococcus) and use of antibiotics and corticosteroids (during exacerbations but not chronically). Currently, two drugs, pirfenidone and nintedanib, which slow the progression of scar tissue formation have been approved for use. Early intervention with these agents is more effective than late treatment, making early diagnosis all the more important. While the medications can slow progression, they do not undo the damage that has already occurred and they have side effects. Dr. Ivan Rosas, associate professor at Brigham and Women's Hospital, emphasizes that more research is needed to develop more and better treatment options aimed at the progression of the disease.

Until Food and Drug Administration approved of pirfenidone and nintedanib in 2014, the only treatment option for progressive IPF was a lung transplant. It remains an important solution for advanced disease and at this time, pulmonary fibrosis is one of the most frequent indications for transplantation.

IPF research has led to the development of these medications, and a greater understanding of the disease. Dr. Rosas said research in the last few years has uncovered that genetic and environmental factors can and do influence IPF.

As investigators begin to understand the mechanisms and triggers that initiate this disease, they'll be able to develop strategies for treatment and prevention. In the last three years, the American Lung Association has awarded 11 investigators who are studying some of the fundamental questions surrounding IPF. These studies include methods of how to fix lung damage, disease progression and protect patients at risk from developing the disease.

"Over the last two decades, there has been a growing interest from the scientific community, the clinical community, government agencies and patient advocacy groups to focus on the study of IPF," Dr. Rosas said. "Like you see with complex disorders, such as lung cancer and COPD, it takes a huge village. There is a growing interest and commitment to understanding the disease and treating patients with the disorder, but more is needed." 

To address the need for more pulmonary fibrosis support, the Lung Association revamped its pulmonary fibrosis section to be a comprehensive resource with content for patients, caregivers, healthcare professionals and anyone wanting to learn more about the disease. Start your research today at Lung.org/pf.

A version of this article originally appeared in the American Lung Association's Advancing Research magazine.

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