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Definition & Statistics
- diopathic pulmonary fibrosis (IPF) is a progressive and generally fatal disease characterized by scarring of the lungs that thickens the lining of the lungs, causing an irreversible loss of the tissue’s ability to transport oxygen. IPF ultimately robs a patient of the ability to breathe.
- IPF affects about 128,100 people in the United States, with about 48,000 new cases diagnosed annually. (1) 40,000 people die each year to IPF, the same as to breast cancer.
- Scarring, or fibrosis, caused by IPF thickens and stiffens the interstitium, causing an irreversible loss of the tissues’ ability to transport oxygen.
- IPF is five times more common than cystic fibrosis and Lou Gehrig’s Disease (or ALS), yet the disease remains virtually unknown (to general public and even among some physicians) and IPF receives a fraction of the research funding (IPF: approx. $18 million per year; Cystic Fibrosis and ALS: $85 million and $48 million per year respectively.
- There is no known cause, no FDA approved treatments and no cure for IPF. IPF is one of the few remaining diseases in which this is the case.
- IPF is one of about 200 disorders called interstitial lung diseases (ILDs). IPF is the most common form of ILD
- Although other ILDs may be attributed to exposure to asbestos or certain medications, IPF has no known or proven cause.
- Potential risk factors for IPF include cigarette smoking and exposure to wood or metal dust. In addition, researchers are exploring a potential genetic predisposition for the disease.
- About two-thirds of IPF patients pass away within five years.
- IPF occurs more often in men than women. Men tend to be diagnosed at a later stage in the disease.
- A recent study found that IPF may be 5 to 10 times more prevalent than previously thought. It is unknown whether this may be due to an increased prevalence of the disease or to a previous lack of definitive guidelines for diagnosing IPF (2)
- Dry, persistent cough lasting longer than 30 days
- Chronic shortness of breath, also known as dyspnea
- Crackling sound in the lungs that can only be heard through a doctor’s stethoscope.
- Until recently there were no uniform diagnostic standards for IPF. Other diseases with similar symptoms have often been diagnosed as IPF, despite widely varying prognoses. In addition, IPF is known by a number of other names (e.g. cryptogenic fibrosing alveolitis), which has caused confusion for patients and physicians.
- Diagnosis of IPF requires a multidisciplinary approach, usually involving a pulmonologist, pathologist and radiologist, to exclude other known causes of ILD and other diseases that mimic IPF.
- Physicians must take a complete patient history, perform a thorough physical exam, assess pulmonary function, and examine chest X-rays and high-resolution computed tomographic images. Lung biopsy, with or without bronchoalveolar lavage (a “lung washing” technique used for the examination of cells and proteins from inside the lung), is frequently required to rule out alternative diagnoses.
Treatment & Therapies
- IPF patients typically are treated with anti-inflammatory drugs, including corticosteroids and cytotoxic agents, despite the fact that there is no evidence that they have any effect on long-term patient survival.
- Patients may require supplemental oxygen to help reduce breathlessness and allow the patient to be more active.
- Lung transplantation is recommended for consideration in patients with severe functional impairment, dependency on oxygen and continued, rapid deterioration despite optimal medical management.
- Researchers are exploring a number of drug treatments for IPF. A complete list of all clinical trials and research in the U.S. is available at www.coalitionforpf.org
- The CPF is the definitive resource for information on treatments and investigational research in IPF in the United States. Contact us at 888-222-8541 or visit www.coalitionforpf.org to learn more.
- (1) Respir Crit Care Med : Incidence and Prevalence of Idiopathic Pulmonary Fibrosis: Ganesh Raghu, M.D., Derek Weycker, Ph.D., John Edelsberg, M.D., M.P.H., Williamson Z. Bradford, M.D., Ph.D., Gerry Oster, Ph.D.: Published on June 29, 2006 as doi:10.1164/rccm.200602-163OC
- (2) Archives of Internal Medicine June 2005 Vol. 142 Number 12 – The Clinical Course of Patients with Idiopathic Pulmonary Fibrosis: Fernando J. Martinez, MD; Sharon Safrin, MD; Derek Weycker, PhD; Karen M. Starko, MD; Williamson Z. Bradford, MD, PhD; Talmadge E. King Jr., MD; Kevin R. Flaherty, MD; David A. Schwartz, MD; Paul W. Noble, MD; Ganesh Raghu, MD