AJMS summarizes developments related to idiopathic pulmonary fibrosis

Jul 8 2011

Idiopathic pulmonary fibrosis (IPF) is a common and severe lung disease of unknown cause and with no proven therapies. But researchers are pushing forward in investigating new clues to the development and effective treatment of IPF, according to a special symposium presented in The American Journal of the Medical Sciences (AJMS), official journal of the Southern Society for Clinical Investigation (SSCI). The journal is published by Lippincott Williams & Wilkins, a part of Wolters Kluwer Health.

The papers summarize the presentations made at The President's Symposium of last year's SSCI Annual Meeting, at which noted experts discussed promising leads as to the cause of IPF, including new data on genetic factors; evolving research strategies; and current clinical management. "Together, these writings summarize the latest knowledge available about IPF, its pathogenesis and therapy," writes guest editor Dr. Jesse Roman of University of Louisville Health Sciences Center.

Idiopathic Pulmonary Fibrosis—Many Questions, but Promising Clues
Patients with IPF have relentlessly progressive fibrosis (scarring) of the lungs. "There is no proven cure for IPF, and only 50 percent of patients survive over 3 years after being diagnosed with this disorder," according to Dr. Roman. More than 160,000 Americans have IPF, and many more worldwide. Recent data suggest that IPF is becoming more common, making it even important to disseminate accurate information on new research and current clinical management.

At the Symposium, researchers and clinicians from Vanderbilt University, University of Alabama, Duke University and elsewhere presented their insights on some of the most important developments related to IPF. The experts were asked to summarize their presentations for publication in The AJMS.

Several presentations focus on possible causes and contributing factors to this complex condition. One line of investigation views IPF as an abnormality of tissue regeneration—similar to other types of disorders in which the normal scarring process goes out of control. The initiation and progression of IPF may involve dysregulation of the normal process of cell death in specific types of lung cells. Treatments that can restore normal cell death of fibrotic cells and normal regeneration of airway-lining epithelial cells are a major focus of current IPF research.

Genetic factors involved in IPF are another key area of research. Familial cases of pulmonary fibrosis are relatively uncommon—known mutations far account for no more than 20 percent of all cases of IFP. However, in-depth study of familial pulmonary fibrosis may provide new clues as to how the idiopathic form of the disease arises. New genetic techniques will likely lead to identification of new gene abnormalities.

Researchers are also working to develop better animal models for IPF. Although various approaches have been used, developing a condition in animals that more closely resembles human IPF will not only help to evaluate new treatments, but also provide clues to disease development.

The symposium also presents a report of a typical patient with IPF and his treatment. There is currently no cure for IPF, and no treatment that is proven effective in prolonging survival. Oxygen therapy and pulmonary rehabilitation can help patients to live with IPF; lung transplantation is an option for some patients. Meanwhile, new treatment approaches are being evaluated—currently there are more than 90 ongoing or recently completed trials of treatments for IPF.

The special IPF symposium highlight's the SSCI's ability to bring together expert clinicians and researchers to address important clinical topics, integrating information collected at the bedside and the research laboratory. Dr. Roman hopes the symposium will lead to further collaborative efforts, encourage patients with IPF to consider enrolling in clinical trials, and stimulate younger professionals to further pursue research in this critical area.