Researchers at two of Michigans largest medical centers have joined forces to track down the cause of one of the most deadly lung diseases.
Backed by a five-year, $5 million grant from the Heart, Lung and Blood Institute of the National Institutes of Health (NIH), researchers at the Medical Center and the Henry Ford Health System hope to dis-cover the cause of idiopathic pulmonary fibrosis, or IPF, and lay the groundwork for development of more effective treatments.
People in their 50s and those with connective tissue disease, such as rheu-matoid arthritis, appear to be the most likely to develop IPF, but those are the only known risk factors. The disease affects one in 100,000 people and can strike anyone, at any age, and for no apparent reason.
The primary symptom of the disease is shortness of breath, usually noticed during or after exercise, says Joseph P. Lynch III, associate professor of internal medicine and a pulmonary medicine specialist who has been researching IPF for more than a decade. As the disease progresses, the individual becomes short of breath while performing tasks around the house or walking. Finally, the person has difficulty breathing even when he or she is resting.
The NIH grant was awarded to the Division of Pulmonary and Critical Care Medicine. The U-M is one of only four institutions nationwide to receive a Specialized Center of Research grant to study the disease. U-M and Henry Ford researchers will study IPF patients at both institutions and compile clinical information related to the cause, treatment and prevention of the disease.
All of us may suffer pulmonary problems, such as pneumonia, bronchitis and the daily breathing of pollutants, which may lead to inflammation, explains John Popovich Jr., division head for Pulmonary and Critical Care Medicine at Henry Ford Hospital. But IPF is more severe.
So far, the only certain knowledge physicians have is that IPF is an immunological disease that apparently occurs when the lungs natural healing process somehow goes awry.
Galen B. Toews, professor and chief of the division of Pulmonary and Critical Care Medicine and grant director, says pulmonary fibrosis results from the highly interactive functions of several types of inflammatory cells. We further believe that regulatory influences of the different types of cells in the lungs are major factors in determining whether inflammation resolves or results in fibrosis of the lung tissue.
Fibrosis, or scarring, occurs when the mending process goes haywire. What starts out as a finely regulated, normal response to a foreign body turns into an unregulated, unchecked action in which inflammatory cells continue to flock to the alveoli, or tiny balloon-like cavities inside the lungs. Over time, multiple scars begin to change the texture and tone of the lungs.
Normally the lungs are like a toy balloon and can expand and contract almost effortlessly, Popovich says. But the lungs of people with IPF become coarse and hard like a football, and they have to expend much more energy than the rest of us just to be able to breathe.
Physicians treat the disease with two therapies: corticosteroids and immunosuppressive agents. About 20 percent to 30 percent of patients who receive treat-ment either improve or at least stabilize. The rest have progressive disease that leads to respiratory failure and death, usually within four to five years of diagnosis.
During the five-year study, researchers hope to learn enough about the cause of IPF to develop more effective treatments.
We are enthusiastic about increasing the clinical and research knowledge base of IPF. Studies will assess the effectiveness of newer diagnostic modalities such as high-resolution CT scanning, Lynch says.
If we can identify what mechanism allows scars to keep from forming in the lungs, we may be able to develop a treat-ment that will prevent it from happening, Popovich adds.
The research also may enable doctors to develop more effective treatments for other conditions that result in scarring of the lungs. Lung scarring can result from sarcoidosis, a disease common among Blacks; from chemotherapy for certain cancers; from breathing asbestos; and from rheumatoid arthritis.
Any IPF patient age 18 and older is eligible to enroll in the study, except those who are pregnant. For information, contact Lynch at 936-5040 or Popovich at 876-2428.