Researchers discover novel pathway of inflammation that contributes to lung damage in CF patients

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.

Dr. Kolls' team is investigating gene-based strategies to probe the immune system's ability to fight infection in the T-cell depleted setting. In mice when T-cells are depleted, the mice are able to protect themselves against infections when given an experimental therapy that takes certain proteins secreted by T-cells the growth factor, IL-17, for example and reconstitutes them into the system. Such an approach might someday treat infections which do not respond well to antibiotics, such as pneumocystis.

The investigation of cytokines is also leading to better understanding of their role in inflammation in the lungs of patients with cystic fibrosis.

Dr. Kolls said, "I think that if we can understand the inflammation processes, we can make an impact on their lives." Dr. Kolls and his team's discovery could also have applications with Multiple Sclerosis, as well as Inflammatory Bowel Disease.


Contact: Melanie Finnigan
Children's Hospital of Pittsburgh

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