Pulmonary Fibrosis

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General Information About IPF

pulmonary fibrosis week

Advances in IPF Research

Clinicians who care for IPF are often either optimists or pessimists. Optimists are cheerleaders for their patients. The educational effort to improve lifestyle with the right amount of oxygen and exercise is independent of the search for a cure.  Optimists help with the emotional rollercoaster that is universal in this disease. They motivate the family to get involved and understand the disease. Optimists want to find earlier onset disease to improve our understanding of the biology, not so that disease burdens can last longer.

Optimists explain that the word "pulmonary fibrosis" means lung scarring; but then go on to explain that there are many places in the body in which scars get better over time. Collagen turnover in the fibrotic lung continues. Our job is to understand the process of progressive fibrosis and why the reparative signaling pathways do not work well in these diseases. We need to understand the processes that cause some individuals to progress rapidly and inexorably and others to follow a stuttering course. We help our patients measure their progress to help with life decisions.

Some have suggested that the word "Idiopathic", a word that means of unknown cause, stifles innovative and thoughtful searches for the cause of this disease. IPF therefore means that pulmonary fibrosis "just happens." The association of IPF with past smoking, use of some drugs, and particulate inhalations would argue that there are environmental and genetic causes that we need to better understand.

One of the important movements in the IPF community is to enlist the aid of as many brilliant scientists as possible to study the disease. Although IPF is defined as a disease of pulmonary fibrosis limited to the lung, there are signals that fibrosis sometimes happens in other sites of the body. In our focus on pulmonary physiology, might there be occult hepatic fibrosis (cryptogenic cirrhosis), or fibrosis of other organs that have been overlooked as co-morbidities in some patients with this disease?   Should we enlist the help of scientists studying cirrhosis, Alzheimer’s, renal failure, and cardiac fibrosis to join our mission, recognizing that many of these individuals are not in the pulmonary community?

One of the interesting aspects of biology is that human biology is often studied, but animal biology is often best understood. Is it time for the veterinarians to begin to understand why some species of dogs get pulmonary fibrosis and others seem immune? And are we prepared to understand the many different patterns of lung histology and computed tomography that result in the many diseases called interstitial lung diseases. The study of rare disease often leads to better understanding of the common diseases. But a better understanding of rare diseases must begin with natural history registries and easily accessible biorepositories.

As the patient community tries to access some of the treatments for IPF that are commercially available in Europe and Asia, my optimistic view is that we need to continue well designed United States studies to better define the efficacy of these drugs. And if a medication is active in IPF, no study has suggested that this would preclude more than one effective drug. The pace of science is rapid; the translation of science to our patients is slower. My optimistic view is that significant advances are in the pipeline and that our partners in drug development share the vision that "fibrosis" is a relative term.

Source:

Charlie Strange, MD 
Medical University of South Carolina
Chair, ATS Assembly on Clinical Problems


Quick Facts About Pulmonary Fibrosis

Every day in America:

  • 128,000 people are suffering from PF

  • 130 people will be diagnosed with PF

  • 110 will die from PF

 (Source: Coalition for Pulmonary Fibrosis)