Researchers have identified a highly specific pathway that causes inflammation in lung tissue, a discovery that could help in the design of more targeted treatments for patients with various lung diseases, including cystic fibrosis.
Children's Hospital of Pittsburgh and University of Pittsburgh researchers have identified a highly specific pathway that causes inflammation in lung tissue, a discovery that could help in the design of more targeted treatments for patients with various lung diseases, including cystic fibrosis (CF).
Their results, which also confirmed this novel pathway may contribute to lung damage in adult patients with cystic fibrosis (CF), are being published in the July issue of The Journal of Immunology.
Jay K. Kolls, MD, who is the division chief of Pediatric Pulmonology, Laboratory of Lung Immunology and Host Defense at Children's Hospital of Pittsburgh, and professor of Pediatrics and Immunology at the University of Pittsburgh School of Medicine, and his research team measured mediators of inflammation in cystic fibrosis patients, focusing on interleukin 23 (IL-23) and interleukin-17 (IL-17).
"The results show that IL-23 and IL-17 may be good targets for neutralization and blocking the inflammatory response," said Dr. Kolls adding that "this research shows us that with the newly identified pathway of inflammation, it may be possible to treat the patient earlier and more effectively, which could prevent lung disease or give patients a better quality of life and longer lifespan."
Dr. Kolls added, "What's intriguing is that by targeting IL-17 we may also be able to inhibit IL-8, a well-known inflammatory instigator in CF, since our laboratory studies suggest expression of this cytokine is dependant on IL-17. A treatment that focuses on IL-17 instead of IL-8 may be the more rational approach."
Because in CF patients the inflammation is never "shut off" and infections cannot be resolved, researchers have been looking for other ways to treat the inflammation associated with the disease. Throughout most of the world, lung infections are a major cause of death and illness among children. Although death rates are low in the United States, lung infection is the leading reason children visit doctors.
"Studies over the last decade have suggested that an excessive inflammatory response to infection occurs in CF. Clinical trials of non-specific anti-inflammatory therapies have shown some clinical benefit, but significant side effects have limited their utility," said co-author Joseph Pilewski, MD, associate professor of Medicine, Pediatrics and Cell Biology and Physiology at the University of Pittsburgh School of Medicine. "This work identifies a target for more specific regulation of the inflammatory pathways that ultimately contribute to lung damage in CF. Modulating this pathway could lead to a safer and more effective anti-inflammatory therapy for CF and perhaps other inflammatory lung diseases."
Dr. Pilewski also is co-director of the Adult Cystic Fibrosis Program at the Antonio J. and Janet Palumbo Cystic Fibrosis Center at Children's Hospital of Pittsburgh, and Medical Director of the Lung Transplant Program at the University of Pittsburgh Medical Center.