Pulmonary Fibrosis

New Idiopathic Pulmonary Fibrosis Guideline Published

A new international clinical practice guideline has been developed and published to help clinicians diagnose idiopathic pulmonary fibrosis (IPF), a rare and often fatal lung disease of unknown etiology.

The guideline was developed by an expert committee with members from 4 major respiratory societies—the American Thoracic Society (ATS), the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Society. The 29-member committee included international clinical and scientific experts and an IPF patient. The committee discussed the findings of all accumulated evidence pertinent to IPF, rated the strength of those findings using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) system, and made recommendations.

The guideline was published online and in the American Journal of Respiratory and Critical Care Medicine.1

IPF is the most common and deadly form of a group of more than 200 conditions known broadly as interstitial lung disease (ILD). Most often, IPF is diagnosed in adults older than 60 and more often in men than in women. Symptoms include dyspnea, a dry cough, and low oxygen saturation. The median survival is 3 to 5 years after the IPF diagnosis.2

“Diagnosing IPF is challenging because these symptoms are nonspecific: they occur with all other interstitial lung diseases and with other respiratory problems,” said Ganesh Raghu, MD, in an ATS press release.2 Dr Raghu is the chair of the guideline committee and professor of medicine and director of the Center for Interstitial Lung Disease at the University of Washington in Seattle. “Because drugs may slow the progression of IPF, an early and accurate diagnosis is essential for prompt and appropriate treatment for this fatal disease,” he said.

This newly published guideline updates the IPF diagnostic guidelines that the 4 respiratory societies published in 2011. Chief among the refinements is the use of 4 diagnostic categories based on high-resolution tomography (HRCT) of the lung: usual interstitial pneumonia (UIP) pattern, probable UIP pattern, indeterminate pattern, and alternative diagnosis.

The committee recommended the same terminology for images produced using HRCT of the lung and for microscopic analysis of biopsied tissue. Consistent categorization should facilitate multidisciplinary discussions (MDD) between a pulmonologist (and in some cases, a rheumatologist), radiologist and pathologist of a diagnosis, the committee wrote. The guideline also features new following recommendations for diagnosing IPF in all adult patients with newly detected ILD of unknown cause.

—Michael Gerchufsky

References:

  1. Raghu G, Remy-Jardin M, Myers JL, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. https://doi.org/10.1164/rccm.201807-1255ST.
  2. New guideline aids in diagnosing idiopathic pulmonary fibrosis [press release]. New York, NY: American Thoracic Society. August 31, 2018.