New Guidelines Update Diagnostic Criteria for Idiopathic Pul

New Guidelines Update Diagnostic Criteria for Idiopathic Pulmonary Fibrosis (IPF)


Updated guidelines on the treatment of idiopathic pulmonary fibrosis (IPF) have been recently released by an international group of  leading respiratory societies. The new guidelines, issued by the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association, have been published in the American Journal of Respiratory and Critical Care Medicine. These guidelines are an update to the ones released in 2011.

The following recommendations are new or revised from the 2011 guidelines
The recommendation against the use of the following agents for the treatment of IPF is strong:
• Anticoagulation (warfarin)
• Imatinib, a selective tyrosine kinase inhibitor against platelet-derived growth factor (PDGF) receptors
• Combination prednisone, azathioprine, and N-acetylcysteine
• Selective endothelin receptor antagonist (ambrisentan)
The recommendation for the use of the following agents for the treatment of IPF is conditional:
• Nintedanib, a tyrosine kinase inhibitor that targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and PDGF receptors
• Pirfenidone
The recommendation against the use of the following agents for the treatment of IPF is conditional:
• Phosphodiesterase-5 inhibitor (sildenafil)
• Dual endothelin receptor antagonists (macitentan, bosentan)

Recommendations remaining unchanged from the 2011 guidelines
•  Conditional recommendation against the use of N-acetylcysteine monotherapy for IPF
•  Conditional recommendation for the use of antiacid therapy

Adult patients with newly detected interstitial lung disease (ILD) of apparently unknown cause are clinically suspected of having idiopathic pulmonary fibrosis (IPF) if they have unexplained symptomatic or asymptomatic patterns of bilateral fibrosis on a chest radiograph or chest computed tomography (CT), bibasilar inspiratory crackles, and an age typically older than 60 years. Rarely, middle-aged adults (40 yr and ,60 yr), especially those with risks for familial pulmonary fibrosis, may otherwise manifest the same clinical scenario as the typical patient older than 60 years. 

The recommendations in this guideline are for the patterns and distributions of images obtained by high-resolution CT (HRCT) and, thus, require that patients be subjected to HRCT of the chest for evaluation

1. For adult patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF:
• Take a detailed history of both medication use and environmental exposures at home, work, and other places the patient frequently visits to exclude potential causes of ILD 
• Serological testing to exclude connective tissue disease as a potential cause of the ILD 

2. For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and have an HRCT pattern of probable usual interstitial pneumonia (UIP), indeterminate, or an alternative diagnosis:
• 
Cellular analysis of their BAL fluid (conditional recommendation)
• 
Surgical lung biopsy (SLB) (conditional recommendation)
• 
No recommendation for or against 
a. Transbronchial lung biopsy (TBBx) 
b. Lung cryobiopsy

3. For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and have an HRCT pattern of UIP
• NOT performing cellular analysis of their BAL fluid is suggested (conditional recommendation)
• NOT performing SLB is recommended (strong recommendation)
• NOT performing TBBx is recommended (strong recommendation) 
• NOT performing lung cryobiopsy is recommended (strong recommendation)

4. For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF
• Multidisciplinary discussion (MDD) for diagnostic decision-making is suggested (conditional recommendation)
• NOT measuring serum MMP (matrix metalloproteinase)-7, SPD (surfactant protein D), CCL (chemokine ligand)-18, or KL (Krebs von den Lungen)- 6 for the purpose of distinguishing IPF from other ILDs are recommended (strong recommendation)

Idiopathic pulmonary fibrosis diagnosis based upon HRCT and biopsy patterns

Diagnostic algorithm for idiopathic pulmonary fibrosis (IPF)

 

 

About ATS
The American Thoracic Society (ATS) is the world's leading medical association dedicated to advancing our clinical and scientific understanding of pulmonary diseases, critical illnesses and sleep-related breathing disorders.


Note: This list is a brief compilation of some of the key points included in the guidelines and is not exhaustive and does not constitute medical advice. Kindly refer to the original publication here: https://pxmd.co/38MFu  


About Author
Dr. Prachi Chhimwal
Dr. Prachi Chhimwal is an Editor at PlexusMD and is a part of the Engagment Team. She curates the Technical Content posted daily on the news feed. She graduated from Army College of Dental Sciences (B.D.S) and went on to pursue her post-graduation (M.D.S) in Oral & Maxillofacial Pathology. After a decade in the field of dentistry she took a leap of faith and joined PlexusMD. A badminton enthusiast, when not working you can find her reading, Netflixing or enjoying stand-up comedy shows.
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Dr. M●●●●●●d S●●●i D●r
Dr. M●●●●●●d S●●●i D●r Internal Medicine
Thanks for getting updated and re-educated.
Sep 15, 2018Like