The American Thoracic Society (ATS) has released new official clinical guidelines on the diagnosis and management of idiopathic pulmonary fibrosis (IPF). The new guidelines replace those published in 2000, and review current knowledge in the epidemiology, etiology, diagnosis, and management of IPF, as well as available treatment options, including pharmacologic and non-pharmacologic therapies and palliative care.
The statement appears in the American Journal of Respiratory and Critical Care.
The new guidelines represent a collaborative effort between ATS, the European Respiratory Society (ERS), the Japanese Respiratory Society (JRS), and the Latin American Thoracic Association (ALAT). The statement has also been formally endorsed by the Society of Thoracic Radiology and by the Pulmonary Pathology Society.
“The methodology used and the strength of the recommendations are all very transparent and thus the clinician confronted with the patient with IPF is empowered for the very first time to make the most appropriate decisions tailored to individual patient’s values and preferences, and to make appropriate decisions regarding all aspects of disease management,” said Ganesh Raghu, MD, director of the Interstitial Lung Disease/Sarcoid/Pulmonary Fibrosis Program at the University of Washington Medical Center in Seattle and chair of the collaborative committee that drafted the statement.
The recommendations in the guidelines are ranked from weak to strong to help the clinician understand the strength of the recommendations made in managing patients with IPF.
“For example, in the case of a weak recommendation clinicians are especially required to spend adequate time with patients to discuss patients’ values and preferences. This may lead a significant proportion of patients to choose an alternative approach,” said Raghu.
Teresa Barnes, ATS Public Advisory Roundtable Chair, points out that while fully informed patients are in the best position to make decisions that reconcile the evidence with their values and preferences, physicians still need to spend as much time as necessary to communicate to patients the risks and consequences of a treatment regimen.
To assist clinicians in their interactions with IPF patients and their families, the ATS has developed a companion patient information series on IPF that is also published in the journal. In addition, the series will be posted on the ATS Web site, as well as the Coalition for Pulmonary Fibrosis Web site.
In addition to offering guidelines for diagnosis and management of IPF patients, the statement also offers suggestions for future studies of the disease.
“It is hoped that with continued collaboration between basic and clinical scientists, the goals of finding the cause or causes of IPF, detecting disease in preclinical and early stages by appropriate biomarkers, genetic predisposition and/or other host susceptibility factors, improving outcomes and quality of life, prolonging survival and, ultimately, curing IPF will be realized,” said Raghu.
Genetic studies and preventive and regenerative strategies, including stem cell transplant research and gene therapy, hold promise and should be aggressively pursued, added Raghu.
“The committee believes that successful treatment of IPF will require a combination of therapies targeting multiple pathways involved in the fibro proliferations,” said Raghu. “Future clinical trials should incorporate endpoints of proven clinical value, utilize sophisticated study design and statistical methodology, investigate the impact of potential preventive measures and consider combinations of promising therapies that work through distinct mechanisms.”
Source: American Thoracic Society