TY - JOUR T1 - Prevalence and Impact of WHO Group 3 Pulmonary Hypertension in Advanced Idiopathic Nonspecific Interstitial Pneumonia JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00545-2018 SP - 1800545 AU - Christopher S. King AU - A. Whitney Brown AU - Oksana A. Shlobin AU - Nargues Weir AU - Matthew Libre AU - Domingo Franco-Palacios AU - Shahzad Ahmad AU - Steven D. Nathan Y1 - 2018/01/01 UR - http://erj.ersjournals.com/content/early/2018/04/12/13993003.00545-2018.abstract N2 - Pulmonary hypertension (PH) is associated with impaired quality of life, worsened functional status, and increased mortality in idiopathic pulmonary fibrosis (IPF).1 The prevalence and clinical impact in other idiopathic interstitial pneumonias (IIP) has not been previously reported. Given the prognostic significance of PH in IPF, we sought to determine the prevalence and severity of World Health Organization (WHO) Group 3 PH in a cohort of patients with biopsy-proven idiopathic NSIP. We recognized that the prevalence of PH in this population of patients with advanced NSIP would be an overestimation of the NSIP population at large, but felt that mandating surgical lung biopsy and right heart catheterization (RHC) were the only way to ensure accurate diagnosis of NSIP and PH, respectively.Group 3 Pulmonary Hypertension commonly complicates idiopathic NSIP and is associated with adverse outcomesFootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. King reports personal fees from Boehringer Ingelheim, personal fees from France Foundation for PILOT program, outside the submitted work.Conflict of interest: Oksana A. ShlobinConflict of interest: Dr. Nargues Weir has no conflicts of interest to disclose.Conflict of interest: Dr. Libre has nothing to disclose.Conflict of interest: Dr. Franco-Palacios has nothing to disclose.Conflict of interest: Dr. Ahmad has nothing to disclose.Conflict of interest: SN is a consultant for, has received research funding and is on the speakers bureau for Boerhinger-Ingelheim, Gilead Sciences, Roche-Genentech, Bayer Pharmaceuticals and United Therapeutics. He is also a consultant for Bellerophon.Conflict of interest: Dr. Brown has nothing to disclose. ER -