CPF applauds new guidelines for treatment of idiopathic pulmonary fibrosis

Guidelines Update 11-Year-Old Consensus Statement for Deadly Lung Disease

The Coalition for Pulmonary Fibrosis (CPF) applauds the joint statement issued by the American Thoracic Society for the first evidence-based clinical guidelines for the treatment of idiopathic pulmonary fibrosis (IPF).  The guidelines represent progress in understanding of the disease and a milestone for patients.  The devastating and deadly disease also called simply "Pulmonary Fibrosis" or "PF" has no FDA approved treatment and no cure.

The new guidelines replace the ATS consensus statement on PF issued in 2000 and reflect the progress in the disease that has changed medical understanding of the complex disease.

"We are pleased to see the new guidelines and hope it will not only help physicians to provide better care to PF patients, but will help them to empower their patients and family members to take an active role in their treatment decisions," said Mishka Michon, CEO of the Coalition for Pulmonary Fibrosis. 

The statement reviews 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.  It appears in the March 15, 2011, American Journal of Respiratory and Critical Care Medicine.

"In the decade since the publication of the previous statement on IPF, studies have used the criteria for the diagnosis of IPF and recommendations published in the previous consensus-based statement to further our understanding of the clinical manifestations and course of IPF, and there has been an increasing body of evidence pertinent to its clinical 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 accumulated data and observations made in these studies are substantial and have allowed us to provide new guidelines for the diagnosis and management of IPF to the global pulmonary community-at-large, to the patients and to all concerned based on evidence for the very first time."

The new guidelines represent a collaborative effort between the ATS, the European Respiratory Society (ERS), the Japanese Respiratory Society (JRS) and the Latin American Thoracic Association (ALAT).

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," Dr. Raghu, who also serves on the CPF's scientific advisory board, said.

To assist clinicians in their interactions with IPF patients and their families, the ATS has developed a companion patient information series piece on IPF that is also published in the March 15 journal. This education piece is also posted on the ATS Web site (www.thoracic.org), as well as the Web site of the Coalition for Pulmonary Fibrosis (www.coalitionforpf.org), an ATS Public Advisory Roundtable member organization, whose focus is on research, advocacy, support and education for the pulmonary fibrosis community.