New Clinical Practice Guidelines on Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis released
The American Thoracic Society-in collaboration with the European Respiratory Society, Japanese Respiratory Society and Asociacion Latinoamericana de Torax-has issued new guidelines on the diagnosis and management of idiopathic pulmonary fibrosis for clinicians.
The guidelines are available online in the American Journal of Respiratory and Critical Care Medicine.
In addition to providing guidance to clinicians who care for adults with IPF- a fatal lung disease characterized by shortness of breath, cough and fatigue, the latest guideline addresses progressive pulmonary fibrosis in interstitial lung diseases, other than IPF , provides a new definition to recognize PPF, and offers insights and guidance on treatment for interstitial lung diseases other than IPF that manifest as PPF.
"Making an accurate diagnosis of IPF and monitoring disease progression in fibrotic ILD, other than IPF is essential for consideration of prompt treatment intervention for PPF," said Ganesh Raghu, MD, chair of the new clinical practice guidelines committee; professor of medicine, Laboratory Medicine and Pathology, University of Washington; and director of the Center for Interstitial Lung Disease, U of Washington Medical Center, Seattle, Washington.
In making a diagnosis, a multidisciplinary team of clinicians-typically chest physicians, radiologists and pathologists with expertise in interstitial lung disease-look for usual interstitial pneumonia or UIP, a hallmark feature of IPF.
"In the absence of a high-resolution computed tomography image pattern of definitive usual interstitial pneumonia subjecting patients to a surgical lung biopsy (SLB) to determine the histopathology features of UIP had been considered as an acceptable surgical procedure in patients who are not at risk for surgical complications to diagnose IPF," said Dr. Raghu.
Increasingly, the trend is to diagnose IPF-using clinical, bronchoalveolar cellular and radiological features; and in patients that need histopathology diagnosis, using smaller lung biopsies with less invasive procedures other than SLB. This trend prompted the guidelines committee to refine the patterns of UIP, provide the current evidence-based guidance for ascertaining the diagnosis of UIP in smaller transbronchial lung biopsies obtained without SLB.
Using the thorough, evidence-based Grading of Recommendations, Assessment, Development and Evaluation or GRADE framework, the expert panel made the following recommendations:
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