We wish you, your staff and families a happy, healthy New Year. Thank you for your ongoing support and confidence in our Program.

Transcription

1 January 2016 Dear Colleague, The Inova Advanced Lung Disease and Transplant Program has enjoyed another successful year with 19 lung transplants in This is a bit of a drop-off in terms of volumes for us, the reasons for which are probably multifactorial, but mostly driven by donor supply. The great equalizer of transplant statistics is The Scientific Registry of Transplant Recipients (SRTR) which supports ongoing evaluation of the status of solid organ transplantation in the United States. It is administered by the Chronic Disease Research Group of the Minneapolis Medical Research Foundation. This was accessed on 12/1/2015 at Our survival statistics continue to be very good and with our most recently reported one year survival at 91.64% versus the expected survival of 86.54% for the period 01/01/ /31/2014 (based on 66 transplants between 01/01/ /30/2014). Our early outcomes remain excellent with a one month survival of 98.44% versus expected of 96.85% (for period 01/01/ /30/2014). Our 3 year survival is a little lower than what we would like at 64.81% versus the national average 68.23% (Based on N=54 transplants performed between 07/01/2009 and 12/31/2011). The median wait time for a lung transplant at our Facility remains very short at 45 days (vs 36 days in 2014) with a range varying from days for The median age of our recipients in 2015 was 63 years (range:21-70 years) reflecting the fact that 16 of the 19 had some form of interstitial lung disease, most commonly IPF. We are underrepresented by COPD (N=2) and cystic fibrosis (N=1). The severity of our patients underlying lung disease is captured through their lung allocation scores which averaged 50.6 (vs 61.1 in 2014). The national average LAS is ~40. Our Advanced Lung Disease Program continues to grow and evolve. We are now an accredited Comprehensive Care Center for Pulmonary Hypertension, an accredited Pulmonary Fibrosis Foundation Care Center Network site and are an Alpha-one antitrypsin Center of Excellence. We are actively working on our application to become a Cystic Fibrosis Care Center, which will be one of our goals for These designations reflect the increasingly complex nature and management options for these patients. In this regard, we do also run two monthly multidisciplinary meetings for ILD and PH respectively, to discuss our more complex cases. It is my belief that advanced lung disease will evolve to be a distinct subspecialty. In lieu of this, Inova has approved us to offer a one year Advanced Lung Disease and Transplant fellowship beginning July We are therefore actively recruiting for this PGY7 position. We continue to welcome and encourage the referral of any patients with all forms of interstitial lung disease and pulmonary hypertension. Although we are an advanced lung disease program, we value seeing these patients as early as possible. We also continue to value all your COPD, cystic fibrosis, sarcoidosis and diagnostic dilemma referrals. From January 1 st 2015 to December 1st, 2015, we received 508 new referrals, which is a 7.2% increase over the previous year. Of these, we evaluated 342 new patients (vs.348 over same period in 2014). The median wait time for an appointment for a new patient in 2015 was 55 days, which is exactly the same as last year and remains an aspect of our program we continue to try and address. If any patients need to be seen more expeditiously, then please call or one of us directly and we will accommodate them earlier. About one third of our new evaluations travel>50 miles to see us and about 10% come from more than 200 miles away, reflecting our standing as a regional and National referral center. Between all our Programs, we follow over 1,000 patients (defined by at least two visits per year). We therefore enjoy, need and welcome co-managing these complex patients with our referring physicians. From a personnel standpoint on the surgical side of the Program, we have an outstanding team of four surgeons Dr.Toni Rongioni (Surgical Director), Dr. Linda Bogar, Dr. Liam Ryan and

2 Dr.Ramesh Singh, who perform all of our transplants. On the Pulmonologist side of the Program, Dr. Chris King s time is slated to be increased from 50% to 100% (whoopie!) All other docs stay the same. Between the current four (soon to be five full-timers), we have been with the Program an average of 12 years (SN-19; SA-14, OS-10 and AWB-5). Unfortunately, we have lost one of NP s (Kara Germano) to a little known program in the Bay area (Stanford or something like that ). Meg Fregoso continues with us (phew!) and we have added Heather Cook to help her manage our posttransplant patients. We are fully staffed on the Nurse Coordinator side having added two new Coords this year, Melissa Bowen and Angela Scully. On the research side, we are now up to 6 Clinical Research Coordinators and one Research Assistant. We place high value and enjoy the relationships we have established with our referring physicians. We pride ourselves on our ready availability. Please feel free to directly call or any of the five Pulmonologists with new referrals, questions, issues, or updates on existing patients. Our contact information is: steven.nathan@inova.org (tel: ) shahzad.ahmad@inova.org (tel: ) oksana.shlobin@inova.org (tel: ) anne.brown@inova.org (tel: ) christopher.king@inova.org (tel: ) nargues.weir@inova.org We wish you, your staff and families a happy, healthy New Year. Thank you for your ongoing support and confidence in our Program. With all good wishes Steven D. Nathan Steven Nathan, MD Medical Director Lung Transplant Program Advanced Lung Disease Program Referrals Phone Fax Deanne Starbird Deanne.starbird@inova.org 2

3 Salient Program Statistics 3

4 INOVA ADVANCED LUNG DISEASE & TRANSPLANT RESEARCH: MULTIFACETED ****RECENT, CURRENT AND FUTURE TRIALS**** Idiopathic Pulmonary Fibrosis (IPF) Clinical Trial Protocol Number: GB28547 Study Name: RIFF Title: A Phase II, Randomized, Double-Blind, Placebo-Controlled Study to Assess the Efficacy and Safety of Lebrikizumab in Patients with Idiopathic Pulmonary Fibrosis Select Inclusion: Diagnosis of IPF within the previous 5 years; DLCO > 25% and < 90%; ability to walk > 100 meters; post-bronchodilator FVC 50 90% Investigational Product: Lebrikizumab, subcutaneous Idiopathic Pulmonary Fibrosis (IPF) Clinical Trial Protocol Number: GS-US Study Name: RAINIER Title: A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Multi-Center Study to Assess the Efficacy and Safety of GS-6624 in Subjects with Idiopathic Pulmonary Fibrosis (RAINIER) Select Inclusion: Diagnosis of IPF; 6MWD 50 meters; Able to perform complete breath hold for diffusing lung capacity.investigational Product: Simtuzumab, subcutaneous Study Status: Closed to enrollment; 7 year follow-up status 4

5 Idiopathic Pulmonary Fibrosis (IPF) Clinical Trial Protocol Number: BIPF Study Name: BIBF-1120 Title: A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Multi-Center, Parallel-Group Study to Evaluate the Efficacy and Safety of BIBF-1120 in Subjects with Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension Investigational Product: Nintedanib, oral Study Status: Closed to enrollment; 7 year follow-up status Reproducibility of Pulmonary Function Tests in Patients with Idiopathic Pulmonary Fibrosis Clinical Trial Study Name: PFT s in IPF Title: Reproducibility of Pulmonary Function Tests in Patients with Idiopathic Pulmonary Fibrosis Select Inclusion: Diagnosis of IPF, 18 years and older, ability to perform PFTs Idiopathic Pulmonary Fibrosis (IPF) Clinical Trial Study Name: Pirfenidone/Ofev Combination Study Title: An Exploratory Multicenter, Open-label, Single Arm Study of the Safety and Tolerability of Pirfenidone (Esbriet ) in Combination with Nintedanib (Ofev ) in Patients with Idiopathic Pulmonary Fibrosis Select Inclusion: On pirfenidone for 16 weeks and stable dose for 28 days, diagnosis of IPF, FVC 50% and DLco 30% at screening, age Study Status: In enrollment approval process; Tentative enrollment start date of February, 2016 Cough in Idiopathic Pulmonary Fibrosis (IPF) Clinical Trial Protocol Number: AF Study Name: Cough in IPF Title: A Randomized Placebo-Controlled Study to Assess the Efficacy and Safety of AF-219, a P2X3 Receptor Antagonist, in Subjects with Idiopathic Pulmonary Fibrosis (IPF) with Persistent Cough Select Inclusion: IPF diagnosis, >40 years of age, Have a score of 40mm on the Cough Severity VAS at Screening. Investigational Product: AF-219, oral Pulmonary Hypertension (PH) associated with Idiopathic Interstitial Pneumonias (IIP) Clinical Trial Protocol Number: BAY / Study Name: Rise-IIP Title: A randomized, double-blind, placebo-controlled phase II study to investigate the efficacy and safety of riociguat (0.5 mg, 1.0 mg, 1.5 mg, 2.0 mg and 2.5 mg TID) in patients with symptomatic pulmonary hypertension associated with idiopathic interstitial pneumonias (IIP). Select Inclusion: Major IIPs diagnosis; PH diagnosis confirmed by right heart catheter (RHC); FVC 45 %; Systolic blood 5

6 pressure (SBP) 95 mmhg Investigational Product: Riociguat, oral Pulmonary Arterial Hypertension (PAH) Clinical Trial Protocol Number: GS- US Study Name: ARROW Title: A Phase 2, Dose Ranging, Randomized, Double-Blind, Placebo-Controlled Study of GS-4997 in Subjects with Pulmonary Arterial Hypertension Investigational Product: GS-US 4997, oral Study Status: Closed to enrollment; in long term follow-up Pulmonary Arterial Hypertension (PAH) Clinical Registry Protocol Number: AC Study Name: OPUS Study Title: Opsumit (macitentan) USers Registry Select Inclusion: Patients newly treated with Opsumit defined as a new user of Opsumit therapy, initiated 30 days prior to enrollment visit or at enrollment Pulmonary Arterial Hypertension (PAH) Registry Protocol Number: CHRC-002 Study Name: Queri study Title: Pulmonary Arterial Hypertension (PAH) Quality Enhancement Research Initiative (QUERI) Extension Program Select Inclusion: Diagnosis of PAH based on RCH within 3 years; Receiving treatment for PH Study Status: Closed to enrollment; in follow-up status until 2018 Pulmonary Arterial Hypertension (PAH) Biobank Study Title: National Biological Sample and Data Repository for Pulmonary Arterial Hypertension Select Inclusion: Must be 18 years and above; World Health Organization (WHO) diagnostic Group 1,mean pulmonary arterial pressure (mpap)>25 mm Hg at rest, pulmonary capillary wedge pressure (PCWP) 18 mm Hg Pulmonary Arterial Hypertension Registry Study Title: Multi-Center Registry of Patients with Group 1 or Group 5 Pulmonary Arterial Hypertension Select Inclusion: new patients to a PHA Care Center with either newly diagnosed or established PAH or CTEPH within 6 months of first visit 6

7 Sarcoidosis Associated Pulmonary Hypertension (SAPH) Clinical Trial Study Name: RioSAPH Title: A double blind, placebo controlled trial of oral Riociguat for Sarcoidosis Associated Pulmonary Hypertension (RioSAPH) Select Inclusion: Patients with diagnosis of sarcoidosis based on ATS/WASOG criteria Study Status: In approval process Non-Cystic Fibrosis Bronchiectasis Clinical Trial Study Name: Orbit 3 Title: A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety and Efficacy of Pulmaquin in the Management of Chronic Lung Infections with Pseudomonas aeruginosa in Subjects with Non-Cystic Fibrosis Bronchiectasis, including 28 Day Open-Label Extension and Pharmacokinetic Substudy (Orbit 3) Select Inclusion: confirmed diagnosis of non-cf bronchiectasis per computerized tomography; Have FEV1 25% of predicted values Investigational Product: Pulmaquin, inhaled Study Status: In follow-up status Systemic Sclerosis Associated Interstitial Lung Disease (ILD) Clinical Trial Protocol Number: BI Title: A double-blind, randomized, placebo-controlled trial evaluating efficacy and safety of oral nintedanib for at least 52 weeks in patients with systemic sclerosis associated interstitial lung disease (SSc-ILD) Select Inclusion: Onset of sclerosis < 5 years; ILD diagnosis by HRCT within 1 year and extent of fibrotic disease in lung 10%; FVC 40%; DLco 30%-89% Hb corrected Investigational Product: Nintedanib, oral Study Status: In approval process; Tentative enrollment start date of March, 2016 Development of diagnostic test for IPF Study Name: BRAVE-1 Title: Bronchial Sample Collection for a Novel Genomic Test Select Inclusion: Undergoing a diagnostic lung biopsy at IFMC for suspected or known ILD; able to tolerate bronchoscopy sampling Tissue/Biospecimens Bank Title: Gene Expression Profiling of Lung Explant and Biopsy Tissue in Subjects with Advanced Lung Disease Select Inclusion: Must be listed for lung transplant at the Inova Transplant Center or be undergoing a diagnostic lung biopsy at IFMC. 7

8 Blood/Biospecimens Bank Title: Collection and Storage of Blood/Biospecimens for Research in Patients Referred to the Advanced Lung Diseases Program Select Inclusion: Must be 18 years and above; patients referred to the Inova Advanced Lung Disease Program Transplant Rejection/Cell Free DNA Clinical Trial Title: Genome Transplant Dynamics (GRAFT) Select Inclusion: Lung and heart transplant candidates on the transplant waitlist at the Inova Transplant Center; 18 years and older Aerobic Exercise and Interstitial Lung Disease Clinical Trial Title: The Exercise Therapy for Advanced Lung Disease Trials: Response and Adaptation to Aerobic in Patients with Interstitial Lung Disease Select Inclusion: must be years, diagnosis of ILD, WHO functional Class II or III (Class I and IV with certain exceptions, no syncope or chest pain, no pulmonary Rehabilitation with in the last 6 month, echocardiogram showing the absence of Pulmonary hypertension 8

9 Drug & Bug Updates IPF Last year marked a watershed year in the field of IPF with two new drugs being approved. Many questions remain with regards to the use of these agents. For your reading pleasure and as a resource I have included one of the articles we had published in 2015 which provides a review on the use of these new antifibrotics for IPF (see enclosed). PAH No new meds in 2015, but a new oral prostacyclin analogue, Selexipeg, might be approved in Stay tuned! Cystic Fibrosis Here we have something new!the recent FDA approval of a new CFTR modulator, Ivacaftor/lumacaftor (Orkambi ) targeted at patients who are homozygous for F508 mutation now means that ~50% or more of CF patients in the USA are candidates for modulator therapy addressing the underlying CFTR defect. This translates to fewer pulmonary exacerbations, a modest improvement in lung function and an improvement in BMI in some cases. Although there were no new drugs approved for either IPF or PAH during 2015, it is interesting to reflect on what has happened in terms of drug development and availability over the past decade for some of the major forms of advanced lung disease. No bug updates, just thought that would look good in the title. The advent of all these agents, while extremely beneficial to our patients has also certainly added a layer of complexity to their management. We are here to help you navigate through the appropriate diagnosis, management options and timely follow-up of patients with all forms of advanced lung disease. One of the values of our integrated model of care for ALD patients, is that most of these patients cannot be pigeon-holed into one category of disease. A good example of this are our connective tissue disease patients with PH who invariably have a component of ILD or our IPF patients who frequently have PH/HFpEF. 9

10 Inova Advanced Lung Disease and Transplant Program Education One aspect of our program that I have neglected to highlight through the years is our role in education. Aside from our individual presentations at Grand Rounds venues, National and International meetings, we have also always enjoyed a steady flow of Fellows and Residents who have rotated through with us on a monthly basis. A rotation through our Advanced Lung Disease and Transplant Program is a now a requirement for all Pulmonary Fellows at the major Academic Centers in the DC Metro area (and beyond). For the academic year (July through June), we have had or will have 7 Medical Residents and 15 Fellows spend time with us from Clarilion Health System (Roanoke), Eastern Virginia Medical School (Norfolk), Georgetown, George Washington, Howard, Walter Reed, Washington Hospital Center and Capitol Hospice. This past year to three of our Fellows/residents presented cases at National meetings including; 2 at the American College of chest physicians meeting in Montréal and one at the cystic fibrosis meeting in Phoenix, Arizona Patient Education & Support Groups Transplantation. We hold a monthly transplant support group to which all our pre-transplant and post-transplant patients are welcomed. It is an expectation that our listed patients attend this as it also functions as an education forum with specific topics and speakers on a monthly basis. IPF. The Pulmonary Fibrosis Support Group of Metropolitan Washington DC is a monthly forum for not only IPF patients, but also those patients with any form of pulmonary fibrosis or interstitial lung disease. Patients do not have to be our clinic patients in order to attend. This support group is now supported by the Pulmonary Fibrosis Foundation and takes place the 4th Tuesday of every month at 1pm in the Physician Conference Center lower level at Inova Fairfax Hospital. See page 17 for schedule. This year we also hosted a combined patient/provider one day seminar on IPF. This was supported by the PILOT initiative that is run through an independent CME Company, The France Foundation. Speakers included four of our own, Drs. Ahmad, Brown, King and Nathan. We had a visiting Professor, Dr.Mark Rumbak, who is the Pulmonary Division Chief at the University of South Florida. Pulmonary Hypertension. We also have a patient run PH support group for all patients with any form of pulmonary hypertension. There are two Pulmonary Hypertension support groups in the area; one in Virginia (NOVA@PHASupportGroups.org) and one in Maryland (MD- SouthernMD@PHASupportGroups.org) Physician Support group. It s called lunch! For any information pertaining to our Support Groups, please contact our Social Worker, Jane Harrison at jane.harrison@inova.org 10

11 Academic Accomplishments 2015 For PDF copies of any publications, please JOURNAL ARTICLES 1. Brown AW, Fischer CP, Shlobin OA, Buhr RG, Ahmad S, Weir NA, Nathan SD. Outcomes after Hospitalization in Idiopathic Pulmonary Fibrosis: a cohort study. Chest 2015;147: Keyser RE, Woolstenhulme J, Chin L, Nathan SD, Connors G, Drinkard B Lamberti J, Chan L. Cardiorespiratory Function Before and After Aerobic Exercise Training in Patients with Interstitial Lung Disease. J Cardiopulm Rehabil Prev Jan-Feb;35(1): Keyser RE, Christensen EJ, Chin LMK, Woolstenhulme JG, Drinkard B, Quinn A, Connors G, Weir N, Nathan SD, Chan LE. Fatigability after Intense Aerobic Exercise Training Patients with Interstitial Lung Disease. Res Med 2015;109: Kirillov V, Siler JT, Ramadass M, Ge L, Grant G, Nathan SD, Jarai G, Trujillo G. Sustained Activation of Toll-like Receptor 9 Induces an Invasive Phenotype in Lung Fibroblasts. Possible Implications in Idiopathic Pulmonary Fibrosis. Am J Pathol Apr;185(4): doi: /j.ajpath Lederer DJ, Bradford WZ, Fagan EA, Glaspole I, Glassberg MK, Glasscock KF, Kardatzke D, King TE, Lancaster L, Nathan SD, Pereira CA, Sahn SA, Swigris JJ, Noble PW. Sensitivity Analyses of the Change in Forced Vital Capacity in a Phase 3 Trial Evaluating Pirfenidone in Adults with Idiopathic Pulmonary Fibrosis. Chest. 2015;148: Nathan SD, du Bois RM, Albera, C, Bradford WZ, Costabel U, Kartashov A, Noble PW, Sahn SA, Valeyre D, Weycker D, King TE. Validation of test performance characteristics and minimal clinically important difference of the 6-minute walk test in patients with idiopathic pulmonary fibrosis. Respir Med 2015;109: Kim SY, Diggans J, Pankratz D, Huang J, Pagan M, Sindy N, Tom E, Anderson J, Choi Y, Lynch DA, Steele M, Flaherty KR, Brown KK, Farah H, Bukstein MJ, Pardo A, Selman M, Wolters P, Nathan SD, Colby TV, Myers JL, Katzenstein AA, Raghu G, Kennedy GC. Differentiating Interstitial Lung Diseases Using Machine Learning on High-Dimensional Transcriptional Data and Surgical Lung Biopsies. Lancet Respir Med. 2015;3: doi: /S (15)00140-X. 8. Raghu G, Nathan SD, Behr J, Brown KK, Egan JJ, Kawut SM, Flaherty KR, Martinez FJ, Wells AU, Shao L, Zhang H, Henig N, Szwarcberg J, Gillies H, Montgomery AB, O Riordan TG. Pulmonary hypertension in idiopathic pulmonary fibrosis with mild to moderate restriction. Eur Respir J (5): doi: / Esposito DB, Lanes SF, Donneyong M, Holick CN, Lasky JA, Lederer D, Nathan SD, O Quinn S, Parker J, Tran TN. Idiopathic pulmonary fibrosis in US automated claims: incidence, prevalence and algorithm validation. Am J Respir Crit Care Med : PMID: Noble PW, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, Lancaster L, Lederer DJ, Leff JA, Nathan SD, Pereira CA, Sahn SA, Swigris JJ, Valeyre D, King TE. Pirfenidone for idiopathic pulmonary fibrosis: Analysis of pooled data from three multinational Phase 3 trials. ERJ Express. Published on December 2, 2015 as doi: / Reichmann WM, Yu YF, Macaulay DS, Wu EQ, Nathan SD. Change in Forced Vital Capacity and Associated Subsequent Outcomes in Patients with Newly Diagnosed Idiopathic Pulmonary Fibrosis. Accepted to BMC Pulm Med September Lancaster L, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, King TE, Lederer DJ, Lin Z, Nathan SD, Pereira CA, Swigris JJ, Valeyre D, Noble PW. Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis: Integrated Analysis of Cumulative Data from 5 Clinical Trials. Accepted to BMJ Open Respiratory Research 10/08/ Yu YF, Macaulay DS, Reichmann WM, Wu EQ, Nathan SD. Association of early suspected acute exacerbations of idiopathic pulmonary fibrosis with subsequent clinical outcomes and healthcare resource utilization. Respir Med Nov 6. pii: S (15) doi: /j.rmed Shin S, King CS, Puri N, Shlobin OA, Brown AW, Ahmad S, Weir N, Nathan SD. Pulmonary artery size as a predictor of outcomes in idiopathic pulmonary fibrosis. Accepted ERJ Dec 7 th

12 REVIEWS 1. Nathan SD. The Future of Lung Transplantation. Chest 2015;147: Meyer KC, Danoff S, Lancaster L, Nathan SD. Management of Idiopathic Pulmonary Fibrosis in the Elderly Patient: Addressing Key Questions. Chest 2015;148: Brown AW, Nathan SD. Lung Transplantation in Interstitial Idiopathic Pneumonia: A Review. Respirology Dec 3. doi: /resp King CS, Nathan SD. Practical Considerations in the Pharmacologic Treatment of Idiopathic Pulmonary Fibrosis. Curr Opin Pulm Med 2015;21: EDITORIALS 1. Nathan SD, Corris PA. Upfront combination therapy: does the Ambition study herald a new era in the treatment of pulmonary arterial hypertension. Thorax Nov 23. pii: thoraxjnl doi: /thoraxjnl BOOK CHAPTERS AND BOOKS 1. King CS, Nathan SD. Treatment of Pulmonary Hypertension in Interstitial Lung Disease. For Pulmonary Hypertension and Interstitial lung disease. Edited by Robert P. Baughman, Roberto G. Carbone and Steven D. Nathan. 2. Shlobin OA, Nathan SD. Rare ILD and PH. For Pulmonary Hypertension and Interstitial lung disease. Edited by Robert P. Baughman, Roberto G. Carbone and Steven D. Nathan. 3. Nathan SD. Interstitial Lung Disease and Pulmonary Hypertension. Submitted 1/20/15 for Pulmonary Circulation: Diseases and their treatment, Fourth Edition. Editors, Peacock, Naeije and Rubin. CRC Press. ORIGINAL RESEARCH ABSTRACTS & PRESENTATIONS TO INTERNATIONAL MEETINGS 1. Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan E, Glaspole I, Glassberg MK, Gorina E, Kardatzke D, King TE, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Spirig D, Swigris JJ, Valeyre D, Noble PW. Pirfenidone is efficacious in patients with idiopathic pulmonary fibrosis (IPF) and mild or more pronounced physiological impairment. Presented at ATS Berry DA, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan E, Glaspole I, Glassberg MK, King TE, Lancaster L, Lederer DJ, Lin Z, Nathan SD, Pereira CA, Sahn SA, Swigris JJ, Valeyre D, Noble PW. Pirfenidone Impact on Mortality in IPF patients: Bayesian Analysis. Presented at ATS Nathan SD, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Glaspole I, Glassberg MK, King TE, Lancaster L, Lederer DJ, Lin Z, Pereira CA, Sahn SA, Swigris JJ, Valeyre D,. Noble PW. Efficacy of Continued Treatment with Pirfenidone Following a Clinically Meaningful Decline in Percent Predicted Forced Vital Capacity in Patients with Idiopathic Pulmonary Fibrosis (IPF). Presented at ATS Lancaster L, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Glaspole I, Glassberg MK, King TE, Lederer DJ, Lin Z, Nathan SD, Pereira CA, Sahn SA, Swigris JJ, Valeyre D, Noble PW. Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (IPF): Integrated Analysis of Cumulative Data from 5 Clinical Trials. Presented at ATS Shin S, King C, Puri N, Shlobin AO, Brown AW, Nathan SD. Utilization of pulmonary arterial size and coronary artery calcification on HRCT imaging for prognostic stratification in patients with IPF. Presented at ATS Raza M, Hostler D, Shlobin OA, Brown AW, Ahmad S, Nathan SD. The Incidence of Heart Failure with Preserved Ejection Fraction among Lung Transplant Recipients. Presented at ATS Kennedy GC, Choi Y, Ellers, S, Huang J, Pankratz D, Kim S-Y, Imtiaz U, Sindy N, Steele M, Brown K, Flaherty K, Lynch D, Myers J, Katzenstein A-L, Nathan SD, Colby T. Diagnosis of Idiopathic Pulmonary Fibrosis on Transbronchial biopsies Using Machine Learning and High Dimensional Transcriptional Data. Presented at ATS AlGhamdi A, Daouk S, Lemma M, Collard H, Johannson K, Lederer DJ, Rosen G, Nathan SD Association of Air Pollution on Survival in patients with Idiopathic Pulmonary Fibrosis. Presented at 12

13 ATS Rodriguez L, Nathan SD, Chhina MK, Leema M, Grant GM. A novel role for Thymosin beta4 in lung fibrosis via TGF-β mediated epithelial mesenchymal transition. Presented at ATS King CS, Ahmad S, Valentino V, Shlobin OA, Brown AW, Cattamanchi A, Singh R, Nathan SD. An International Survey of Early Post-operative Management Practice Patterns Following Lung Transplantation. Presented at ATS Bui S, Chhina MK, Khandhar S, Mani H, Nathan SD, Grant GM. Effect of in-vitro combination Treatment with Curcumin, Aspirin and Sulforaphane on Idiopathic Pulmonary Fibrosis fibroblasts. Presented at ATS Ahmad S, Shafagati N, Shlobin OA, King C, Nathan SD, Brown AW. Association of Coagulation Protein Levels in Development of Deep Venous Thrombosis in Lung Transplant Recipients. Presented at ATS Kaya H, Brown AW, Amdur R, Nathan SD. Distance supplemental oxygen (DSO): An index to predict the survival in IPF. Presented at ATS Scholand MB, Baughman RP, Nathan SD, Barney JB, Cordova FC, Van Den Blink B, Shlobin OA, Engel P, Culver DA. Clinical Features of Patients with Moderate to Severe Sarcoidosis Associated Pulmonary Hypertension. Presented at ATS Culver DA, Baughman RP, Cordova FC, Barney JB, Shlobin OA, Engel P, Van DenBlink B, Nathan SD. Six Minute Walk Testing in Patients with Sarcoidosis Associated Pulmonary Hypertension. Presented at ATS Reichmann WM, Yu Y, Macaulay D, Nathan SD. Association of change in forced vital capacity with healthcare resource utilization in patients with newly diagnosed idiopathic pulmonary fibrosis. Submitted to International society of pharmacoeconomics and outcomes research. ISPOR 20th Annual International Meeting to be held May 16-20, Kaya H, Nathan SD, Ahmad S, Shlobin OA, King CS, Weir N, Brown AW. An Index to Predict the Survival of Pulmonary Hypertension. Presented at ATS Nathan SD, Reichmann WM, Macaulay D, Yu Y. Decline in Forced Vital Capacity and Clinical Outcomes in Newly Diagnosed Idiopathic Pulmonary Fibrosis Patients. Presented at ATS Nathan SD, Reichmann WM, Macaulay D, Yu Y. Impact of Suspected IPF Acute Exacerbations and on Subsequent Clinical and Healthcare Resource Utilization Outcomes Newly Diagnosed Idiopathic Pulmonary Fibrosis Patients. Presented at ATS. 20. Tetteh H, Burton NA, Lefrak EA, Ahmad S, Shlobin OA, Nathan SD. Double Donor Lobectomy Following Bilateral Sequential Single Lung Transplantation: A Case for Split Bilateral Lung Transplants. Presented at ISHLT April Nathan SD, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, Glasscock KF, King TE, Lancaster L, Lederer DJ, Lin Z, Pereira CA, Swigris JJ, Valeyre D, Noble PW, Wells A. Benefit of continued pirfenidone treatment following hospitalisation within the first 6 months of treatment ad hoc analysis from three Phase 3 trials in patients with idiopathic pulmonary fibrosis. Presented at ERS Nathan SD, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Glaspole I, Glassberg MK, Kardatzke D, King TE, Kirchgaessler K, Lancaster LH, Lederer DJ, Pereira CA, Swigris JJ, Valeyre D, Noble PW. Effect of Pirfenidone (PFD) on Treatment-emergent (TE) All-cause Mortality (ACM) in Patients with Idiopathic Pulmonary Fibrosis (IPF): Pooled Analysis of Data from ASCEND and CAPACITY. Presented at ERS Ahmad S, Brown AW, King C, Shlobin OA, Weir N, Stayrook S, Nathan SD. The Six Minute Walk Test Comparison to a Stair Climb Test. Poster at ERS Nathan SD, Albera C, Bradford WZ, Costabel U, Daigl M, du Bois RM, Fagan EA, Glaspole I, Glassberg MK, Kardatzke D, King TE, Kirchgaessler K, Lancaster LH, Lederer DJ, Pereira CA, Swigris JJ, Valeyre D, Noble PW. Effect of Pirfenidone on All-Cause Mortality in Patients with Idiopathic Pulmonary Fibrosis (IPF): Comparison of Pooled Analysis with Meta-Analysis from the ASCEND and CAPACITY Trials.. Presented October 26th 2015 Chest Montreal. 25. Nathan SD, Albera C, Bradford WZ, Costabel U du Bois RM, Fagan EA, Glaspole I, Glassberg MK, Kardatzke D, King TE, Kirchgaessler K, Lancaster LH, Lederer DJ, Pereira CA, Swigris JJ, Valeyre D, Noble PW. Effect of Pirfenidone on IPF-related Mortality Outcome Measures in Patients with Idiopathic Pulmonary Fibrosis (IPF): Pooled Data Analysis from the ASCEND and CAPACITY 13

14 Trials. Presented October 28th 2015 Chest Montreal. 26. Dave K, Brown AW, King C, Nathan SD. Lung mass associated with Cystic Lung disease An evasive diagnosis in a patient with Primary Sjögren s. Presented October Montreal Chest PRESENTATIONS AT NATIONAL/INTERNATIONAL MEETINGS 1. When to treat PH in association with IPF. International Society for Heart and Lung Transplantation, Nice, France. April 15th, (SN) 2. Lung Transplantation: The Medically Challenging CF Patient International Society for Heart and Lung Transplantation, Nice, France. April 15th, 2015 (AWB) 3. Pulmonary hypertension in Lung Disease. 10th annual Bayer Pulmonary Hypertension Symposium, Berlin, Germany April 25th, 2015 (SN) 4. Idiopathic Pulmonary Fibrosis in Evolution: Proactive Recognition, Early Diagnosis, and an Advancing Standard of Care. Chairperson Symposium. American College of Physicians 4/30/2015. Boston, MA. (SN) 5. Medical Therapy for Group 3 PH: Could you, should you, how would you? ATS Symposium May 20th, Denver, CO. (SN) 6. Role of lung transplantation in connective tissue diseases. May 2015 ATS 2015, Denver, USA (OS) 7. Sarcoidosis-associated Pulmonary Hypertension. World Association for Sarcoidosis and other Granulomatous Diseases. Sao Paulo, Brazil June 6th, (SN) 8. Benefit of continued pirfenidone treatment following hospitalisation within the first 6 months of treatment ad hoc analysis from three Phase 3 trials in patients with idiopathic pulmonary fibrosis. Podium presentation at European Respiratory Society meeting, Amsterdam September 29th, (SN) 9. Effect of Pirfenidone (PFD) on Treatment-emergent (TE) All-cause Mortality (ACM) in Patients with Idiopathic Pulmonary Fibrosis (IPF): Pooled Analysis of Data from ASCEND and CAPACITY. Podium presentation at European Respiratory Society meeting, Amsterdam September 29th, (SN) 10. Chairperson session on Pulmonary Hypertension in ILD at American College of Chest Physicians October 27th, Montreal Canada. (SN) 11. Pulmonary Hypertension in ILD. American College of Chest Physicians October 27th, Montreal Canada. (SN) 14

15 Inova Lung Transplant and Advanced Lung Disease Team Members (December 2015) Back row: Princess Waring (Medical Tech); Stephanie Toczylowski (Research Coordinator); Deanne Starbird (Referral Coordinator); Sarah Scott (Office Manager); Carlos Coronel (Admin Assistant); Adam Cochrane (Transplant Pharmacist); Chris King, MD; Shahzad Ahmad, MD; Sarah Kelly (Dietician); Sydney Stayrook (Research Assistant); Renee Brenner (Research Coordinator); Middle row: Jennifer Cumberland (Medical Tech); Margaret Fregoso (NP, Post-Transplant Coordinator); Jane Harrison (Social Worker); Heather Cook (Post-Transplant Coordinator); Maria Altan Mejia (Clinic Nurse); Astrid Julieth Munoz (Admin Assistant); Denise Lewis (ALD and Transplant Coordinator); Tina Thronson (Quality Manager); Lori Hill (Financial Coordinator); Edwinia Battle (Research Manager). Front Row: Debbie Campbell (Transplant Director); Angela Scully (ALD Coordinator); Alicia Banks (Patient Registration); Steven Nathan, MD; Nargues Weir, MD; Whitney Brown, MD; Oksana Shlobin, MD; Keisha Cardenas (Patient Registration); Melissa Bowen (Pretransplant Coordinator) Absent: Interventional Pulmonologist: Amit Bobby Mahajan, MD Surgeons: Linda Bogar, MD, Sandeep Khandhar, MD; Liam Ryan, MD; Tony Rongione, MD; Ramesh Singh, MD. Infectious Disease: Shalika Katugaha, MD Research: Lori Schlegel; Merte Lemme; Melodie Wuorinen. 15

16 Other team and fun photos: Dr..Whitney Brown presenting at the International Society for Heart and Lung Transplantation, Nice, France. April 2015 Molly K 5k fundraiser for IPF (Burke Lake, VA Nov 2015). Team Inova - Katrina Breese (Inova Foundation), Sarah Scott (Office manager), Steve Nathan, Merte Lemme (Research Coordinator) IPF cake of appreciation from a research subject. If you can t beat it, eat it Saturday night party and a transplant at the same time! Denise Lewis and Kara Germano double teaming it. Bobby Mahajan, Steve Nathan, Oksana Shlobin, Sandeep Khandhar at ATS Denver May Inova ALD outpatient clinic with Nargues Weir, Oksana Shlobin and Whitney Brown 16

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