Idiopathic Pulmonary Fibrosis: New Guidelines Released

Lara C. Pullen, PhD

July 17, 2015

The American Thoracic Society (ATS) has partnered with an international group of leading respiratory societies to create clinical practice guidelines for the treatment of idiopathic pulmonary fibrosis (IPF). The update of the 2011 guidelines has been issued by the ATS as well as the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association and was published in the July 15 issue of the American Journal of Respiratory and Critical Care Medicine.

The guidelines summarize the significant advances that have been achieved in the clinical management of IPF. They do not, however, recommend one treatment regimen over another.

The update was prompted by publication of the Prednisone, Azathioprine, and N-Acetylcysteine: A Study That Evaluates Response in Idiopathic Pulmonary Fibrosis (PANTHER-IPF) trial data. The trial investigated the efficacy of triple therapy with prednisone, azathioprine, and N-acetylcysteine as a treatment for idiopathic pulmonary fibrosis. The study found that triple therapy "increased the death rate by about 10 times," explained Kevin C. Wilson, MD, from the American Thoracic Society in New York City, to Medscape Medical News.

The new guidelines thus have a strong recommendation against triple therapy. The 2011 guideline had a weak recommendation against triple therapy.

Dr Wilson explained that the other noteworthy changes to the 2011 guidelines include conditional recommendations for the use of nintedanib and pirfenidone. These agents are new, and the updated guidelines cite clinical trials that support their efficacy.

The panel assessed evidence using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach, in which recommendations are scored as either "strong" or "conditional."

The new guidelines also give a strong recommendation against the use of anticoagulation, imatinib, and ambrisentan. Specifically, warfarin anticoagulation should not be used in patients with IPF if the patients do not have a known alternative indication for its use.

The guidelines also give a conditional recommendation against the use of sildenafil, macitentan, and bosentan.

The panel has continued the 2011 conditional recommendation against the use of N-acetylcysteine monotherapy for IPF. They have also continued the 2011 conditional recommendation for the use of antacid therapy in patients without symptoms of gastroesophageal reflux.

"Our systematic review of the available evidence on IPF treatments points to the need for additional research and long-term studies of their safety and efficacy," Ganesh Raghu, MD, from the University of Washington Medical Center in Seattle, who was chair of the committee that produced the guidelines, said in a society news release. "This is especially true for treatments that received conditional recommendations in the guidelines. The guidelines empower the clinician to make the most appropriate treatment choices for the patient confronted with IPF and encourage shared decision-making with the well informed patient to choose the most appropriate treatment options tailored to the individual patient's needs."

Dr Raghu consulted for Actelion, Bayer, Biogen, Boehringer Ingelheim, Centocor/Johnson & Johnson/Janssen, Celgene, FibroGen, GlaxoSmithKline, GeNO, Gilead, InterMune International, Promedior, sanofi-aventis, Stromedix, Takeda, Kadmon, Roche Genentech, UCB Celltech, and Veracyte and served on a data and safety monitoring board of MedImmune and an adjudication committee of Degge. Full conflict-of-interest information is available in the guideline. Dr Wilson has disclosed no relevant financial relationships.

Am J Respir Crit Care Med. 2015;192:e3-e19. Abstract

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