Almost four years since the last guidance on the diagnosis and management of idiopathic pulmonary fibrosis, the American Thoracic Society – in collaboration with the European Respiratory Society, Japanese Respiratory Society and Asociacion Latinoamericana de Torax – has issued new guidelines for clinicians. The guidelines are available online in the May 1 issue of 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:
Recommendations for IPF
- Question: Should patients with newly detected ILD of undetermined type who are clinically suspected of having IPF undergo transbronchial lung cryobiopsy to obtain samples to make a histopathological diagnosis?
We suggest transbronchial lung cryobiopsy be regarded as an acceptable alternative to surgical lung biopsy for making a histopathological diagnosis in patients with ILD of undetermined type in medical centers with experience performing and interpreting transbronchial lung cryobiopsy (conditional recommendation, very low-quality evidence).
- Question: Should genomic classifier testing be performed for the purpose of diagnosing usual interstitial pneumonia in patients with ILD of undetermined type who are undergoing transbronchial forceps biopsy?
We make no recommendation for or against the addition of genomic classifier testing for the purpose of diagnosing usual interstitial pneumonia in patients with ILD of undetermined type who are undergoing transbronchial forceps biopsy, due to insufficient agreement among the committee members.
Treating Abnormal Gastroesophageal Reflux to Improve Respiratory Outcomes
- Question: Should patients with IPF and confirmed abnormal gastroesophageal reflux (GER), with or without symptoms of GERD, be treated with antacid medications to improve respiratory outcomes?
We suggest NOT treating patients with IPF with antacid medication for the purpose of improving respiratory outcomes (conditional recommendation, very low-quality evidence).
- Question: Should patients with IPF and confirmed abnormal GER, with or without symptoms of GERD, be referred for anti-reflux surgery to improve respiratory outcomes?
We suggest NOT referring patients with IPF for anti-reflux surgery for the purpose of improving respiratory outcomes (conditional recommendation, very low-quality evidence).
Treatment Recommendations for Non-IPF Progressive Pulmonary Fibrosis
- Question: Should patients with PPF be treated with pirfenidone?
We recommend further research into the efficacy, effectiveness, and safety of pirfenidone in both 1) non-IPF progressive pulmonary fibrosis in general and 2) specific types of interstitial lung disease manifesting progressive pulmonary fibrosis.
- Question: Should patients with PPF be treated with nintedanib?
We suggest nintedanib for the treatment of progressive pulmonary fibrosis in patients who have failed standard management (conditional recommendation, low-quality evidence).
The guideline committee while acknowledging the suggestions and recommendations made were based on low quality evidence surfaced the need for further studies to address the questions in future updates of the guideline.