Here are some important highlights from the meeting.
Supported by educational grants from Boehringer Ingelheim Pharmaceuticals, Inc., and Actelion.


Transbronchial Cryobiopsy for the Diagnosis of ILD
— Dr. Paul W. Noble

Fleischner Society Update in Classification of IPF
— Dr. David A. Lynch


ICD-10 Codes and Nomenclature for Classification of PAH
— Dr. Ronald J. Oudiz

NTM and Treatment Strategies for Patients with Cavitary Disease
— Dr. Sandra G. Adams


Real World Experience in Patients with COPD
— Dr. Sandra G. Adams

Palliative Care for Patients with IPF
— Dr. Meena Kalluri



New Developments in the Diagnosis and Management of IPF
— Dr. Anoop M. Nambiar

Registry Data Provide Important Severity Indicators for PH
— Dr. Ronald J. Oudiz


The Latest in IPF: Implications for Clinical Practice
— Drs. Lisa H. Lancaster &  Maria L. Padilla

Perspectives on Side Effect Management
— Drs. Lisa H. Lancaster &  Maria L. Padilla


IPF Management Considerations
— Drs. Lisa H. Lancaster &  Maria L. Padilla

Questions for the experts | Transcript






Conference Coverage Q&A


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Dr. Lynch discussed the new Fleischner Society diagnostic criteria for IPF. What are the new diagnostic categories of usual interstitial pneumonia (UIP), based on CT patterns?

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The following table includes the distribution and features associated with the 4 different diagnostic categories of UIP based on CT patterns from the new Fleischner Society guidelines.

Lynch DA, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6:138-153.

Dr. Nambiar said that combination studies with pirfenidone and nintedanib have shown that treatment with the antifibrotic agents combined is well tolerated. What were the most common adverse events, and is there any information yet on the efficacy of combination treatment?

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Vancheri et al have recently published results from an open-label trial of nintedanib with add-on pirfenidone in patients with IPF (the INJOURNEY Trial). In this study, patients with IPF and FVC ≥50% predicted at screening who completed a 4–5 week run-in with nintedanib 150 mg bid without dose reduction or treatment interruption were randomized to nintedanib 150 mg bid with add-on pirfenidone (titrated to 801 mg tid) (n = 53), or nintedanib 150 mg bid alone (n = 52), open-label for 12 weeks. The primary endpoint was the percentage of patients with on-treatment gastrointestinal adverse events from baseline to week 12. Analyses were descriptive and exploratory. GI AEs were reported for 69.8% of patients treated with nintedanib with add-on pirfenidone and in 52.9% of patients treated with nintedanib alone. Diarrhea, nausea and vomiting were the most frequent AEs, occurring in 37.7%, 41.5% and 28.3% respectively, with combination treatment, compared with 31.4%, 11.8%, and 11.8% with nintedanib treatment alone. At 12 weeks, the mean absolute change from baseline in FVC was -13.3±17.4 mL in the nintedanib + pirfenidone group compared with -40.9 ± 31.4 mL for nintedanib treatment alone.

Vancheri C, et al. Am J Respir Crit Care Med. 2017 Sep 10. [Epub ahead of print] Nintedanib with Add-on Pirfenidone in Idiopathic Pulmonary Fibrosis: Results of the INJOURNEY Trial. Available at Accessed November 30, 2017.

An open-label, single arm, 24 week safety and tolerability study of pirfenidone in combination with nintedanib has also been conducted in patients with IPF (NCT02598193), however the results of this study have not been published to date.

Conference Coverage Q&A

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