Diagnosis and Care of Patients with Idiopathic Pulmonary Fibrosis

Mayo Clinic researchers conducted an international electronic survey among lung specialists to document current practice patterns concerning the diagnosis and management of patients with idiopathic pulmonary fibrosis (IPF). The results were presented this week at the CHEST 2007 meeting in Chicago.

Idiopathic pulmonary fibrosis (IPF) is a disease of unknown cause that results in progressive lung scarring (fibrosis) and ultimately affects the ability to breathe and obtain adequate oxygen. It occurs in approximately 43 in 100,000 people. Diagnosing and treating patients with idiopathic pulmonary fibrosis (IPF) is extremely challenging. The primary symptoms of this disease can mimic the effects of a lingering cold and other lung disorders, and they often don’t appear until the disease is advanced. Besides lung transplantation, no current treatments have been proven effective in halting the progress of this devastating disease. In 2000, the American Thoracic Society and European Respiratory Society published practice guidelines to assist physicians with diagnosis and management of IPF. Since then, research has yielded many advances in this field. Recognizing these developments, Mayo Clinic researchers conducted an international electronic survey of lung specialists to document current practice patterns concerning the diagnosis and management of patients with IPF, to assess familiarity and use of the society’s guidelines, and to identify other issues to address in future practice guidelines. “In light of the recent advances and the remaining challenges in managing IPF, it is essential to evaluate whether practicing pulmonologists accept and implement the existing practice guidelines. This information is extremely valuable as we assess the need for and develop updated guidelines for this devastating disorder,” explains Mayo Clinic Rochester pulmonologist Tobias Peikert, M.D., lead researcher on this project. Survey FindingsMayo researchers surveyed 6,443 members of the American College of Chest Physicians. Seventy-two percent of respondents were familiar with the societys’ consensus statement. Sixty-three percent found the statement to be clinically useful. However, a similar number of respondents indicated that an update is needed. Forty-five percent of pulmonary physicians advocate providing only supportive care for patients outside of clinical trials. Among physicians who offer patients treatment with medications, there was a lack of consensus regarding the specific drugs that should be used. Despite physician awareness of clinical trials (79 percent), only 54 percent of respondents consistently refer interested patients to clinical trials. Most respondents (61 percent) felt that lung transplantation represents the only effective therapy for IPF, and 86 percent refer their patients to lung transplant centres. Survey ConclusionsThe survey results demonstrate a substantial variability among pulmonary physicians in the diagnosis and management of IPF. According to Dr. Peikert, the observed variability may, in part, reflect the current lack of effective drug therapy. “Our results indicate that new practice guidelines incorporating recent developments regarding the diagnosis and therapy of IPF are clearly needed. Furthermore, to enhance our understanding of IPF and develop new therapies, treating physicians should be encouraged to discuss and offer interested patients’ referral to clinical trials,” says Dr. Peikert. Collaboration and SupportThe research team also included Mayo pulmonologists Craig Daniels, M.D., and Jay Ryu, M.D.; Mayo survey researcher Timothy Beebe, Ph.D.; and pulmonologist Keith Meyer, M.D., from the University of Wisconsin. This survey was supported by the American College of Chest Physicians Interstitial Lung Diseases Network. (Source: CHEST 2007 Meeting, October 2007 : Mayo Clinic : December 2007)