As treatments and research into idiopathic pulmonary fibrosis (IPF) advance rapidly, guidelines for treatment of the disease must also be advanced. That is why pulmonary fibrosis (PF) organizations and healthcare providers are lauding the update of the Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis
, which was last updated in 2011.
The updated Guideline, developed by a multidisciplinary panel from the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society and the Latin American Thoracic Association, provides an evidence-based rationale for specific treatments for patients with IPF.
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Cosgrove“The community is coming together and recognizing the rapid evolution of information and, rather than relying on just expert opinion, the exciting aspect is that we have guidelines that are evidence-based,” said Gregory Cosgrove, MD, chief medical officer of the Pulmonary Fibrosis Foundation
(PFF). “As researchers and clinicians, we can validate the information we have based on robust scientific information, and identify areas in which we need more information.”
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For the first time, a patient with IPF was included in the panel as they revised the Guideline. “That is a really important step forward. The insight that patients have…is dramatically different than that of researchers and clinicians,” Cosgrove said. “While we hope we understand, we are not placed in the same context they are. It is a much more well-rounded approach to the guide recommendations.”
Among the top additions to the IPF guidelines are:
The conditional recommendation for the use of nintedanib (OFEV). Manufactured by Boehringer Ingelheim, it is one the first major treatments for IPF, and was approved by FDA last year. The tyrosine kinase inhibitor targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor and PDGF receptors. The updated IPF treatment guidelines also provide a conditional recommendation for pirfenidone (Esbriet, Genentech).
The strong recommendation against the use of certain therapies in patients with IPF. For example, the researchers issued a “strong recommendation against” the use of the anti-coagulant warfarin. The combination of prednisone, Azathioprine and N-acetylcysteine should also not be used, based on the “low confidence in effect estimates”. The selective tyrosine kinase inhibitor Imatinib and the selective endothelin receptor antagonist ambrisentan should also not be used in IPF therapy.
While the scientists reviewed the 2011 Guidelines to determine if they should chance anti-acid therapy recommendations the treatment of gastro-esophageal reflux (GURD) disease in IPF, those guidelines remained unchanged. The group issued a “conditional recommendation for use” of anti-acid therapy.
Meanwhile, the panel deferred recommendations on anti-pulmonary hypertension therapy in IPF – as well as a discussion on single versus bilateral lung transplantation – until the next update.
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