BACKGROUND:It is unclear whether continuing anti-fibrotic therapy until the time of lung transplant increases the risk of complications in patients with idiopathic pulmonary fibrosis.OBJECTIVES:To investigate whether the time between discontinuation of anti-fibrotic therapy and lung transplant in patients with idiopathic pulmonary fibrosis affects the risk of complications.METHODS:We assessed intra-operative and post-transplant complications among patients with idiopathic pulmonary fibrosis who underwent lung transplant and had been treated with nintedanib or pirfenidone continuously for ⩾ 90 days at listing. Patients were grouped according to whether they had a shorter (⩽ 5 medication half-lives) or longer (> 5 medication half-lives) time between discontinuation of anti-fibrotic medication and transplant. Five half-lives corresponded to 2 days for nintedanib and 1 day for pirfenidone.RESULTS:Among patients taking nintedanib (n = 107) or pirfenidone (n = 190), 211 (71.0%) had discontinued anti-fibrotic therapy ⩽ 5 medication half-lives before transplant. Anastomotic and sternal dehiscence occurred only in this group (anastomotic: 11 patients [5.2%], p = 0.031 vs patients with longer time between discontinuation of anti-fibrotic medication and transplant; sternal: 12 patients [5.7%], p = 0.024). No differences were observed in surgical wound dehiscence, length of hospital stay, or survival to discharge between groups with a shorter versus longer time between discontinuation of anti-fibrotic therapy and transplant.CONCLUSION:Anastomotic and sternal dehiscence only occurred in patients with idiopathic pulmonary fibrosis who discontinued anti-fibrotic therapy < 5 medication half-lives before transplant. The frequency of other intra-operative and post-transplant complications did not appear to differ depending on when anti-fibrotic therapy was discontinued.REGISTRATION:clinicaltrials.gov NCT04316780: https://clinicaltrials.gov/ct2/show/NCT04316780.

Introduction:Non-specific pattern (NSP) is a subgroup of preserved ratio impaired spirometry (PRISm) that requires a normal total lung capacity measurement. NSP has been historically classified as being an obstructive lung disease pattern. There has been heightened interest and investigation into PRISm recently as it has been associated with an increased likelihood of developing chronic obstructive pulmonary disease (COPD). Given the inherent challenges of understanding the clinical significance of the NSP, the aim of this study was to further explore the clinical characteristics of patients with this pulmonary function test pattern.Material and methods:We identified 111 and 79 subjects using pre-bronchodilator (pre-BD) and post-bronchodilator (post-BD) values, respectively, that met criteria for NSP. The outpatient medical records were retrospectively reviewed for associated diagnoses that were then clustered into 'obstructive' or 'non-obstructive' groups based on the treating physician's primary pulmonary clinical diagnosis.Results:Within this NSP cohort, cough, wheezing and sputum production were documented more frequently in those with an obstructive lung disease diagnosis. Whether identified using pre-BD or post-BD spirometric values, those with NSP and a positive BD response were more likely to carry an obstructive lung disease diagnosis.Conclusion:Approximately one third of patients with NSP in this study were not given an obstructive lung disease diagnosis by their clinician, which supports the classification of NSP as not an exclusively obstructive lung disease pattern. However, the presence of supporting clinical symptoms, such as cough with sputum production and wheeze, and/or a positive BD response on PFT, support a diagnosis of obstruction in patients with NSP.

SESSION TITLE: Lung Cancer Case Report Posters 11 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: While cervical cancer is the fourth most common cancer of women in the world, the number of cases has declined in regions where screening is implemented. However, in resource-limited areas, the incidence and mortality rates remain disproportionately high. Lung metastases in cervical cancer occur in up to 8%. We present an unfortunate case of lung metastases of a cervical squamous cell carcinoma. CASE PRESENTATION: A 43-year-old woman was admitted for vision loss, diagnosed with multifocal acute and subacute embolic strokes of the left occipital cortex. She was found to have a lower extremity DVT along with multiple segmental pulmonary emboli. The CT also showed bibasilar ground glass and consolidative infiltrates. The strokes were felt to be a consequence of an inter-atrial shunt. She was started on anticoagulation and then underwent IVC filter placement as her DVT showed proximal propagation with an eventual plan for atrial septal defect closure. She was discharged though returned 1 month later with severe vaginal bleeding. A CT abdomen/pelvis showed pelvic lymphadenopathy and a cervical mass invading the bladder wall. She required high-flow oxygen and a chest CT showed resolving thrombi, but progressive bilateral lower lobe consolidative infiltrates. Endometrial biopsy confirmed an exophytic squamous cell carcinoma. Her anticoagulation was continued as her bleeding allowed and she was treated with broad antibiotics for possible pneumonia. Despite this she progressed to respiratory failure and was intubated. Her course complicated by increased intra-cardiac shunting post-intubation. She underwent bronchoalveolar lavage of the right middle lobe which grew oral flora, the cytology showed squamous cell carcinoma from the cervix. Her hypoxemia and shock worsened and care was ultimately withdrawn. DISCUSSION: Lung metastases typically appear as single or multiple, rounded nodules scattered throughout both lungs. Atypical features like consolidation or cavitation are rare. We present a case of a rapidly progressive stage 4 cervical cancer with metastases to both lungs in a pneumonia-like, consolidative pattern that progressed rapidly and led to hypoxemic respiratory failure. While lepidic growth resulting in imaging findings resembling infectious pneumonia is most frequently observed in lung adenocarcinoma, the pattern of rapidly progressive lepidic pulmonary metastases has also been described in tumors originating from the biliary system, GI tract and breast. CONCLUSIONS: While we did not have pathological confirmation of lepidic growth within the lung, the rapid progression of the metastases over 1 month in a pneumonia-like manner and the cancer cells captured in lavage fluid, strongly suggested lepidic growth which has not been described in cervical cancer. REFERENCE #1: Yohsuke Nagayoshi, Kazuko Yamamoto, Satoru Hashimoto, Keiko Hisatomi, Seiji Doi, Seiji Nagashima, Hirokazu Kurohama, Masahiro Ito, Takahiro Takazono, Shigeki Nakamura, Taiga Miyazaki, Shigeru Kohno, An Autopsy Case of Lepidic Pulmonary Metastasis from Cholangiocarcinoma, Internal Medicine, 2016, Volume 55, Issue 19, Pages 2849-2853, Released on J-STAGE October 01, 2016, Online ISSN 1349-7235, Print ISSN 0918 2918, https://doi.org/10.2169/internalmedicine.55.5972, https://www.jstage.jst.go.jp/article/internalmedicine/55/19/55_55.5972/_article/-char/en, Abstract: REFERENCE #2: https://doi.org/10.1016/j.lungcan.2018.01.013 DISCLOSURES: No relevant relationships by Saeed Abughazaleh No disclosure on file for Levindo Garcia No relevant relationships by Peter LaCamera No relevant relationships by Wasey Ali Yadullahi Mir No relevant relationships by Azib Shahid

BackgroundDespite the availability of COVID-19 vaccinations, there remains a need to investigate treatments to reduce the risk or severity of potentially fatal complications of COVID-19, such as acute respiratory distress syndrome (ARDS). This study evaluated the efficacy and safety of the transient receptor potential channel C6 (TRPC6) inhibitor, BI 764198, in reducing the risk and/or severity of ARDS in patients hospitalised for COVID-19 and requiring non-invasive, supplemental oxygen support (oxygen by mask or nasal prongs, oxygen by non-invasive ventilation or high-flow nasal oxygen).MethodsMulticentre, double-blind, randomised phase II trial comparing once-daily oral BI 764198 (n=65) with placebo (n=64) for 28 days (+2-month follow-up). Primary endpoint: proportion of patients alive and free of mechanical ventilation at day 29. Secondary endpoints: proportion of patients alive and discharged without oxygen (day 29); occurrence of either in-hospital mortality, intensive care unit admission or mechanical ventilation (day 29); time to first response (clinical improvement/recovery); ventilator-free days (day 29); and mortality (days 15, 29, 60 and 90).ResultsNo difference was observed for the primary endpoint: BI 764198 (83.1%) versus placebo (87.5%) (estimated risk difference –5.39%; 95% CI –16.08 to 5.30; p=0.323). For secondary endpoints, a longer time to first response (rate ratio 0.67; 95% CI 0.46 to 0.99; p=0.045) and longer hospitalisation (+3.41 days; 95% CI 0.49 to 6.34; p=0.023) for BI 764198 versus placebo was observed; no other significant differences were observed. On-treatment adverse events were similar between trial arms and more fatal events were reported for BI 764198 (n=7) versus placebo (n=2). Treatment was stopped early based on an interim observation of a lack of efficacy and an imbalance of fatal events (Data Monitoring Committee recommendation).ConclusionsTRPC6 inhibition was not effective in reducing the risk and/or severity of ARDS in patients with COVID-19 requiring non-invasive, supplemental oxygen support.Trial registration numberNCT04604184.

Asbestos is a naturally occurring mineral with a wide range of application in various industries. It has been linked with respiratory and non-respiratory illnesses. We aim to discuss the various pathologies caused due to asbestos exposure with succinct coverage of all areas of asbestosis study. Asbestos continues to be used in many industries. Currently, cosmetic products contaminated by asbestos raise the problem of continuous exposure to a proven carcinogen. Various conditions like effusions, plaques, pneumoconiosis, mesothelioma, and lung cancer are associated with it. The development of respiratory problems has a dose–response relationship with asbestos. Currently, no pharmacological therapy has demonstrated efficacy in the treatment of asbestosis. The diagnosis of asbestos-related illnesses should have a clear history of exposure to the mineral and suggestive clinical and imaging findings. As the therapy of asbestos-related illnesses is not curative, the main aim should be to limit asbestos exposure. Besides, alternative materials should be promoted instead of asbestos to reduce its usage further.

BACKGROUND: Bronchial thermoplasty is a device-based treatment for subjects >-18 years of age with severe asthma poorly controlled with inhaled corticosteroids and long-acting beta-agonists. The Post-FDA Approval Clinical Trial Evaluating Bronchial Thermoplasty in Severe Persistent Asthma (PAS2) study collected data on patients with severe asthma undergoing this procedure. RESEARCH QUESTION: What are the 5-year efficacy and safety results in patients with severe asthma who have undergone bronchial thermoplasty? STUDY DESIGN AND METHODS: This was a prospective, open-label, observational, multicenter study conducted in the United States and Canada. Subjects 18 to 65 years of age who were taking inhaled corticosteroids >= 1,000 mg/d (beclomethasone or equivalent) and long-acting beta-agonists >= 80 mg/d (salmeterol or equivalent) were included. Severe exacerbations, hospitalization, ED visits, and medication usage were evaluated for the 12 months prior to and at years 1 through 5 posttreatment. Spirometry was evaluated at baseline and at years 1 through 5 posttreatment. Spirometry was evaluated at baseline and at years 1 through 5 posttreatment. RESULTS: A total of 284 subjects were enrolled at 27 centers; 227 subjects (80%) completed 5 years of follow-up. By year 5 posttreatment, the proportion of subjects with severe exacerbations, ED visits, and hospitalizations was 42.7%, 7.9%, and 4.8%, respectively, compared with 77.8%, 29.4%, and 16.1% in the 12 months prior to treatment. The proportion of subjects on maintenance oral corticosteroids decreased from 19.4% at baseline to 9.7% at 5 years. Analyses of subgroups based on baseline clinical and biomarker characteristics revealed a statistically significant clinical improvement among all subgroups. INTERPRETATION: Five years after treatment, subjects experienced decreases in severe exacerbations, hospitalizations, ED visits, and corticosteroid exposure. All subgroups demonstrated clinically significant improvement, suggesting that bronchial thermoplasty improves asthma control in different asthma phenotypes.

Bronchial thermoplasty is a device-based treatment for subjects ≥ 18 years of age with severe asthma poorly controlled with inhaled corticosteroids and long-acting beta-agonists. The Post-FDA Approval Clinical Trial Evaluating Bronchial Thermoplasty in Severe Persistent Asthma (PAS2) study collected data on patients with severe asthma undergoing this procedure.What are the 5-year efficacy and safety results in patients with severe asthma who have undergone bronchial thermoplasty?This was a prospective, open-label, observational, multicenter study conducted in the United States and Canada. Subjects 18 to 65 years of age who were taking inhaled corticosteroids ≥ 1,000 μg/d (beclomethasone or equivalent) and long-acting beta-agonists ≥ 80 μg/d (salmeterol or equivalent) were included. Severe exacerbations, hospitalization, ED visits, and medication usage were evaluated for the 12 months prior to and at years 1 through 5 posttreatment. Spirometry was evaluated at baseline and at years 1 through 5 posttreatment.A total of 284 subjects were enrolled at 27 centers; 227 subjects (80%) completed 5 years of follow-up. By year 5 posttreatment, the proportion of subjects with severe exacerbations, ED visits, and hospitalizations was 42.7%, 7.9%, and 4.8%, respectively, compared with 77.8%, 29.4%, and 16.1% in the 12 months prior to treatment. The proportion of subjects on maintenance oral corticosteroids decreased from 19.4% at baseline to 9.7% at 5 years. Analyses of subgroups based on baseline clinical and biomarker characteristics revealed a statistically significant clinical improvement among all subgroups.Five years after treatment, subjects experienced decreases in severe exacerbations, hospitalizations, ED visits, and corticosteroid exposure. All subgroups demonstrated clinically significant improvement, suggesting that bronchial thermoplasty improves asthma control in different asthma phenotypes.ClinicalTrials.gov; No.: NCT01350336; URL: www.gov.

BackgroundManagement of patients with interstitial lung disease (ILD) requires subspecialized, comprehensive, multidisciplinary care. The Pulmonary Fibrosis Foundation established the Care Center Network (CCN) in 2013 with identified criteria to become a designated CCN site. Despite these criteria, the essential components of an ILD clinic remain unknown.Research QuestionsHow are ILD clinics within the CCN structured? What are the essential components of an ILD clinic according to ILD physician experts, patients, and caregivers?Study Design and MethodsThis study had three components. First, all 68 CCN sites were surveyed to determine the characteristics of their current ILD clinics. Second, an online, three-round modified Delphi survey was conducted between October and December 2019 with 48 ILD experts participating in total. Items for round 1 were generated using expert interviews. During rounds 1 and 2, experts rated the importance of each item on a 5-point Likert scale. The a priori threshold for consensus was more than 75% of experts rating an item as important or very important. In round 3, experts graded items that met consensus and ranked items deemed essential for an ILD clinic. Third, ILD patient and caregiver focus groups were conducted and analyzed for content to determine their perspectives of an ideal ILD clinic.ResultsForty items across four categories (members, infrastructure, resources, and multidisciplinary conference) achieved consensus as essential to an ILD clinic. Patient and caregiver focus groups identified three major themes: comprehensive, patient-centered medical care; expanded access to care; and comprehensive support for living and coping with ILD.InterpretationThe essential components of an ILD clinic are well-aligned between physician experts and patients. Future research can use these findings to evaluate the impact of these components on patient outcomes and to inform best practices for ILD clinics throughout the world. Management of patients with interstitial lung disease (ILD) requires subspecialized, comprehensive, multidisciplinary care. The Pulmonary Fibrosis Foundation established the Care Center Network (CCN) in 2013 with identified criteria to become a designated CCN site. Despite these criteria, the essential components of an ILD clinic remain unknown. How are ILD clinics within the CCN structured? What are the essential components of an ILD clinic according to ILD physician experts, patients, and caregivers? This study had three components. First, all 68 CCN sites were surveyed to determine the characteristics of their current ILD clinics. Second, an online, three-round modified Delphi survey was conducted between October and December 2019 with 48 ILD experts participating in total. Items for round 1 were generated using expert interviews. During rounds 1 and 2, experts rated the importance of each item on a 5-point Likert scale. The a priori threshold for consensus was more than 75% of experts rating an item as important or very important. In round 3, experts graded items that met consensus and ranked items deemed essential for an ILD clinic. Third, ILD patient and caregiver focus groups were conducted and analyzed for content to determine their perspectives of an ideal ILD clinic. Forty items across four categories (members, infrastructure, resources, and multidisciplinary conference) achieved consensus as essential to an ILD clinic. Patient and caregiver focus groups identified three major themes: comprehensive, patient-centered medical care; expanded access to care; and comprehensive support for living and coping with ILD. The essential components of an ILD clinic are well-aligned between physician experts and patients. Future research can use these findings to evaluate the impact of these components on patient outcomes and to inform best practices for ILD clinics throughout the world. Interstitial lung diseases (ILD) represent a rare and heterogeneous group of more than 100 diseases. Among them, idiopathic pulmonary fibrosis is the most common and carries significant morbidity and mortality, with a median survival of 41 months.1Nathan S.D. Shlobin O.A. Weir N. et al.Long-term course and prognosis of idiopathic pulmonary fibrosis in the new millennium.Chest. 2011; 140: 221-229Abstract Full Text Full Text PDF PubMed Scopus (277) Google Scholar The scope of care for patients with ILD can be challenging and complex, ranging from obtaining accurate diagnoses, initiating disease-modifying treatments, providing supportive care, including oxygen therapy, and managing comorbidities to discussing lung transplantation and providing palliative and end-of-life care. The multidimensional, complex, and longitudinal needs of this patient population has led to the emergence of specialized, comprehensive ILD clinics in the last two decades. The Pulmonary Fibrosis Foundation (PFF) is a nonprofit organization dedicated to providing support to those living with pulmonary fibrosis. In 2013, the PFF started the Care Center Network (CCN), which now consists of 68 designated medical centers across the United States recognized for having expertise in the diagnosis and treatment of ILD through multidisciplinary care and patient engagement in education, support, and research. Although the PFF CCN delineates the criterion to qualify and maintain designated site status,2Pulmonary Fibrosis FoundationWorking together to improve patient outcomes: PFF Care Center Network. Updated March 1, 2019. Pulmonary Fibrosis Foundation website.https://www.pulmonaryfibrosis.org/docs/default-source/medical-community-documents/pff-care-center-criteria-2019-v-03-updated_03-01-2019.pdf?sfvrsn=a02d918d_2Google Scholar little is known about the composition, structure, and function of an ideal ILD clinic. Because of a lack of clinical practice guidelines to inform the structure of an ILD clinic, we identified three objectives for this study: first, to assess the current structure of ILD clinics within the PFF CCN; second, to determine the essential components of an ILD clinic using a three-round modified Delphi survey administered to a group of ILD experts, all of whom are directors of PFF CCNs; and third, to identify the essential components of an ILD clinic from the perspectives of ILD patients and caregivers using a series of focus groups. The Colorado Multiple Institutional Review Board approved the study (Identifier: COMIRB 19-0841, 19-1583). A 60-item, web-based survey using Research Electronic Data Capture (REDCap) was sent to all 68 PFF CCN directors via e-mail to determine baseline characteristics and composition of their ILD clinics. Items included in the first round of the modified Delphi survey were compiled based on content analysis of transcripts from individual, semistructured telephone interviews with PFF CCN directors (e-Appendix 1). Nine directors were selected based on their clinical expertise and background to ensure representation with respect to sex, geography, and size of ILD clinic. All interviews were conducted by C. H., digitally recorded, and transcribed verbatim. All PFF CCN directors, including those who had participated in the telephone interviews, were invited via e-mail to participate in the modified Delphi survey. We conducted a three-round Delphi survey using a secure, online REDCap database between October and December 2019. In rounds 1 and 2, the Delphi collaborators ranked items by degree of importance on a five-point Likert scale (very important, important, less important, not important, and not sure). In the third round, participants were instructed to rank items on a five-point Likert scale based on if items were thought to be essential to an ILD clinic (strongly agree, agree, disagree, strongly disagree, and not sure). Results were analyzed anonymously and reported according to the defined methodologic criteria for Delphi studies.3Diamond I.R. Grant R.C. Feldman B.M. et al.Defining consensus: a systematic review recommends methodologic criteria for reporting of Delphi studies.J Clin Epidemiol. 2014; 67: 401-409Abstract Full Text Full Text PDF PubMed Scopus (1586) Google Scholar The a priori threshold for an item to be considered important to an ILD clinic in Delphi rounds 1 and 2 was defined as more than 75% consensus among experts rating an item as "very important" or "important," and the a priori threshold for an item to be considered not important to an ILD clinic was defined as more than 75% consensus among experts rating an item as "not important" or "less important." The same thresholds were used for the third Delphi round, but in relationship to level of agreement with an item being "essential" for an ILD clinic. We conducted focus groups with patients with ILD and self-identified caregivers. We chose a qualitative study design using content analysis as our methodologic framework to gain a comprehensive understanding of the experiences, wants, and needs of patients and caregivers seeking medical care at ILD clinics. Eligible participants had to reside currently in the United States. Participants were not excluded if they were not currently or had not previously received care at a PFF CCN site. Participants were recruited at the PFF 2019 Summit at an informational table and through a recruitment flyer distributed electronically to the PF Warrior community e-mail distribution list. Informed consent was obtained from all participants at the time of the focus group. Participants were not compensated for their time. Real-time focus groups were conducted using a secure, web-based meeting interface and facilitated by authors (B. A. G., C. H., and M. M.) from November 2019 through January 2020. The focus groups followed a semistructured, open-ended approach to elicit participants' opinions and experiences of ILD clinics (e-Appendix 2). All focus groups were audio recorded and transcribed verbatim. Three focus groups initially were planned, but new themes emerged from the second and third groups. Therefore, two additional focus groups were conducted, after which thematic saturation was achieved based on debriefing after group meetings and note comparison by the moderator (B. A. G.) and comoderators (C. H. or M. M.). This number of groups is consistent with existing literature examining the relationship between number of focus groups conducted and thematic saturation.4Hennink M.M. Kaiser B.N. Weber M.B. What influences saturation? Estimating sample sizes in focus group research.Qual Health Res. 2019; 29: 1483-1496Crossref PubMed Scopus (429) Google Scholar,5Saunders B. Sim J. Kingstone T. et al.Saturation in qualitative research: exploring its conceptualization and operationalization.Qual Quant. 2018; 52: 1893-1907Crossref PubMed Scopus (5179) Google Scholar Transcripts were entered into Atlas.ti version 8.0 software (ATLAS.ti Scientific Software Development GmbH) and analyzed for content using a general inductive approach. Analysis was completed by organizing the data through open coding and repeated comparisons to identify key themes. The primary coders (B. A. G. and M. M.) met regularly to discuss coded data, to reconcile differences, and to achieve consensus. Both coders double-coded all focus groups. Coded data were analyzed within and across groups to identify the emergent themes. A total of 36 of 68 (53%) PFF CCN directors completed the survey (Table 1 and e-Table 1). The median number of new patient visits per year was 250 (interquartile range, 150-383), and the median number of total patient visits per year was 1000 (interquartile range, 500-1500). Sixty-seven percent of clinics were staffed with two to five physicians. Nearly all clinics (92%) had nursing support, with 56% of sites having dedicated ILD nurses. Available ancillary support staff varied across clinics. Every clinic participated in research and clinical trials. All clinics, except for one, held a multidisciplinary conference and conferences at least once per month.Table 1Baseline Characteristics of ILD Clinics in the PFF CCNItemResultMembers... No. of ILD-focused pulmonologists working in the ILD clinic...12 (6)2-524 (67)> 510 (27) Nursing support for the ILD clinic33 (92) Nurses who are dedicated solely to ILD clinic (in the clinics with nurses)18 (56) Advanced practice providers working in the ILD clinic18 (50)Infrastructure... New patient visits per y250 (150-383) Total patient visits per y1000 (500-1,500) No. of half days of ILD clinic per wk7 (4-8) Average wait time from referral to clinic visit...1 wk-1 mo15 (42)1-3 mo16 (44)3-12 mo4 (11)Unknown1 (3) Most patients traveling > 60 min to get to clinic19 (53) Use of telemedicine3 (8)Resources... Access to social worker11 (31) Access to respiratory therapists24 (67) Access to a palliative care program33 (92) Access to a dedicated ILD palliative care program3 (8) Access to a local pulmonary rehabilitation program36 (100) Access to a local support group36 (100) Participate in clinical trialsaOnly 35 respondents answered this question.35 (100) Participate in patient registries32 (89) Participate in biobanking of specimens31 (86)Multidisciplinary conference... Participation in a multidisciplinary conference35 (97) Frequency of multidisciplinary conference...> Once/wk1 (3)Once/wk17 (49)Once/2 wks9 (26)Once/mo8 (23)Data are presented as No. (%) or median (interquartile range). CCN = Care Center Network; ILD = interstitial lung disease; PFF = Pulmonary Fibrosis Foundation.a Only 35 respondents answered this question. Open table in a new tab Data are presented as No. (%) or median (interquartile range). CCN = Care Center Network; ILD = interstitial lung disease; PFF = Pulmonary Fibrosis Foundation. Eight of the 9 identified PFF CCN directors participated in the telephone interviews. Eighty-seven total items were generated for inclusion in the first round of the modified Delphi survey. Items were divided into four categories: members of an ILD team, infrastructure for an ILD clinic, resources for an ILD clinic, and multidisciplinary conference. Of the 67 PFF CCN directors invited to participate (J. S. L., as PFF CCN Director at the University of Colorado, was not invited to participate), the response rate for the three rounds of modified Delphi was 48 (72%) for the first round, 42 (63%) for the second round, and 40 (60%) for the third round (Fig 1). The number of items in each round is summarized in Figure 2, and detailed results of all three rounds can be found in the online Supplemental Materials (e-Tables 2-4). At completion of the third round, 40 unique items achieved consensus as essential for an ILD clinic (Table 2).Figure 2Flow diagram showing items through each round of the modified Delphi process. The Delphi collaborators were asked to rank items in degree of importance for interstitial lung disease (ILD) clinics on a five-point Likert scale during each of the three rounds. During round 1, participants were given the opportunity to add any additional items they found important for an ILD clinic that were not included already in the first-round items. In the second round, the amended list of items (including the items generated in round 1) and results of the first round that did not achieve consensus were presented. Participants were asked to rate this amended list on the same five-point Likert scale. In the final round, participants ranked consensus items on a five-point Likert scale on whether these items were not only important, but also essential, for an ILD clinic. ∗Threshold of importance: 75% of respondents selected "very important" or "important." †Item: If advanced practice providers are present in ILD clinic, their role mainly should be diagnosis of new ILD patients. ‡One item duplicated–total of 52 unique items in round 3.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Table 2Items That Achieved Consensus in the Modified Delphi Survey as Essential or Important for an ILD ClinicItems That Are Essential to Have for an ILD ClinicItems That Are Important to Have for an ILD ClinicMembers of the ILD team PhysiciansHaving expertise in ILD (a certain number of years working in ILD patient care)Having at least 2 or more pulmonologists working the ILD clinic NursesDedicated ILD nurse If a clinic has advanced practice providersClose supervision by physiciansTheir role should be mainly longitudinal care of ILD patients Research coordinatorsClinic coordinatorsFellows and traineesInfrastructure of ILD clinics GeneralILD clinic sees a minimum number of patients per yearILD clinic sees a minimum of 100 unique patients/yaIf threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic.Minimum number of clinics per weekEase of access to clinicTriaging and rerouting referrals to ILD clinics from general pulmonary clinicsTriaging of ILD patients before new patient visit to avoid multiple visits (prescheduling tests before the visit, obtaining prior records and imaging, and so forth)The maximum time from referral to new appointment is less than 2 mo for a standard new patient visitbMaximum time to new patient visit < 1 mo or < 3 mo for a standard new patient visit were important, but not essential.Maximum time from referral to new appointment is less than 7-10 d for an urgent patient visit Patient management strategiesProviding a mix of primary management, collaborative/shared care, and consultative managementProviding primary management of ILD care Exposure historyObtain a structured occupational and environmental exposure history for all new ILD patientsResources for ILD clinics Pulmonary rehabilitationA pulmonary rehabilitation facility in close proximity (within a 30-60-min drive from the center) to the ILD clinicaIf threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic. Ancillary services within the same institutionGeneral rheumatologyAccess to rheumatologists with expertise in ILDThoracic radiologyAccess to sleep clinicPulmonary pathologyaIf threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic.Thoracic surgeonPulmonary hypertensionaIf threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic.CardiologyPalliative careExperience in treating patients with advanced lung diseasesAvailability in outpatient, inpatient, and hospice care Pulmonary function testingSame-day appointments as the ILD clinic visit RadiologyDedicated ILD HRCT protocolHRCT available same day or next day of clinic visitaIf threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic. ResearchParticipation in researchParticipation in clinical trialsParticipation in patient registriesaIf threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic. Patient educationPatient education is delivered by physiciansaIf threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic.Patient education is delivered by nursesILD clinic participates in local patient support groupsMultidisciplinary conference Having a multidisciplinary conferenceFrequency is at least once/2 wks Staff (routinely participates in ILD conference)PulmonologistsTraineesRadiologistsPathologists Discussing the following types of patients at multidisciplinary conferenceComplex cases with diagnostic or therapeutic dilemmasPatients who already have undergone surgical lung biopsyPatients in whom a surgical lung biopsy is being consideredILD = interstitial lung disease.a If threshold for agreement were increased to > 80%, these items would have been considered important, but not essential, for an ILD clinic.b Maximum time to new patient visit < 1 mo or < 3 mo for a standard new patient visit were important, but not essential. Open table in a new tab ILD = interstitial lung disease. In the third round, if we changed the threshold for essential to 70%, this would have added six items to the list of essential items for an ILD clinic, including inclusion of more specific personnel (clinic and research coordinators, access to rheumatologists with expertise in ILD) and a minimum frequency of multidisciplinary conference (e-Table 4). In contrast, if the threshold were more stringent, only allowing for those with more than 80% agreement, seven items would be excluded from the essential list, including a minimum number of unique patients seen yearly, specific ancillary services at the same institution, and proximity of a pulmonary rehabilitation center (Table 2). A total of 21 individuals participated: 16 patients and five family caregivers. We conducted five focus groups with a range of three to seven participants per group. Among the patient participants, 69% were men and 31% were women. Among the caregiver participants, 20% were men and 80% were women. Additional demographics were not obtained to maintain participant confidentiality; some participants provided details of their diagnoses voluntarily. We identified three major themes from the focus groups that encompass patients' and caregivers' perspectives of an ideal ILD clinic: comprehensive patient-centered medical care, expanded access to care, and support for living and coping with ILD. See Table 3 for the three major themes, with subthemes and representative quotations.Table 3Major Themes and Subthemes With Representative Quotations From the Patient and Caregiver Focus GroupsThemeSubthemesComprehensive, Patient-Centered Medical CareAll-InclusivePatient-CenteredTimely and EfficientAssistance With Prescriptions and Insurance CompaniesExcerpts"They have in one building just about everything necessary. All of the diagnostic equipment; they have a blood lab; they have all kinds of scanning equipment. Of course, they have the equipment to test your breathing. Of course, they have the physicians there to meet with you after your tests are completed, so they go over your diagnosis and discuss a treatment plan . . . everything is in one building for all of your pulmonary diagnoses and treatments.""To me, to be able to go somewhere that they have all of the necessary specialties to provide complete diagnostic and treatment-option discussions, that really should be done in a day because of the psychological impact . . . the more quickly that you can get a definitive diagnosis, to me would be very important.""Very, very integrated and focused on the patient.""Being a patient-centered facility rather than a doctor-centered facility.""The idea of doctors being cued in to compassion and not just talking about the data or the interest in data in their research . . . also to remember that these are patients.""I think if I had had a place to go, and no matter how long it took me . . . but just to be able to stay in one spot and be able to get it done and know, 'Okay. This is it. This is what's happening' to me is very important.""For us, because we fatigue so easily and get so short of breath, the least amount of travel and appointment times would be ideal.""Maybe somebody who would go through insurance or check your insurance for the different medications.""It would be great if there was somebody that can look into your insurance benefits.""I think that a good clinic would have a more competent staffing level to deal with prescriptions for medication, prescriptions for oxygen, and prescriptions for rehab."Expanded Access to CareCommunicationGeographic Considerations and TravelPulmonary RehabilitationClinical Trials ParticipationExcerpts"I would advise people to sign up for the . . . patient portal, to get familiar with that process, it makes things a lot easier in contacting doctors and/or their staff . . . its just an easy, easy way of communicating and not having to be on the phone for minutes at a time waiting for someone to answer or someone to call you back.""I just feel like I can always reach out to her and ask her any questions, and she always gets back to me.""Its gonna go always to me location, location, location, you know what I mean? Wherever they're located at makes it easier. If I can get there within an hour, that's nice, but if I have to drive 6 hours to get to the place, once again, its just a hard thing.""I had to go out of state to get any help whatsoever.""But that could be just because I'm so far and remote from any facility. I'm so far away to the facility.""I think that would be my biggest complaint about any clinic is where is your pulmonary rehab and how can we get people there easy. These people don't travel well in the first place, and then they have to go so far to get—what I consider is one of the treatments for pulmonary fibrosis, is pulmonary rehab.""It took me actually 9 months to find a pulmonary rehab that was not too far away from us . . . most of 'em are [1] hour and a half, 2 hours away, which is kinda far when you're going two to three times a week.""I would love to get into a trial . . . I mean, that's how we're gonna beat this thing, you know, and to slow down the progression and to make things better for other people. Trials are extremely important.""I believe that most patients would be very happy to participate in trials if they were offered that participation in a hope that we might be able to, you know, help make some discoveries that will, if not prevent other people from having the condition in the future, at least to come up with some better treatment options."Comprehensive Support for Living and Coping With Interstitial Lung DiseasePatient AdvocateDisease EducationCounseling and Support ServicesPatient Support GroupsSupplemental OxygenExcerpts"First of all, they come in and tell that patient that they're not alone.""It would be somebody that you would feel comfortable going to with multiple questions . . . that would have access and obviously enough knowledge about the disease to know the next step that you're looking for or in need of.""Having somebody who essentially knows you, that maybe periodically would touch base with us if we haven't been in clinic in a month or two. Even if it's a phone call saying, 'Whad'ya need?' Or, you know, just, 'Let me know if you need something. I'll try to find it and tell you where to go and get it.'""If there was some sort of patient ambassador that people can have where there are so many questions that a patient will have afterwards . . . that coordinate the questions and to be able to pass on information to the patients or the families to get them pointed in the right direction when they have these different questions.""You always know you can call up this person and whether its getting a doctor's appointment scheduled, or getting a referral or getting a new medication or medication refill, if it's questions about insurance . . . to have somebody as a primary point of contact to coordinate all of these different things, whether its them being able to answer the question or just getting you pointed in the right direction to find that answer is something that would be very valuable.""I think on that first visit, there needs to be more information given, like that fact sheet of—you can get an IPF fact sheet. That needs to be handed out.""Things like where you can buy an oximeter and what oximeter is good to get, things like that just right off the bat are good things to get, and books to read . . . that they can start learning and preparing themselves. If they can be prepared right off the bat with stuff, I think, to me, is an ideal clinic.""As soon as you get a diagnosis like IPF, I know that my first inclination is to Google the disease and there's a lot of bad information out there, inaccurate information, and scary information . . . it's up to the patient to seek these things out most of the time, as opposed to maybe one center having all these things and not only providing the services but also giving an explanation of why these things are important." "The most important things they could do would be to make the patient aware of the organizations that exist to help support patients who have ILD.""There would be counsellors, counselling services there because you're being given a death sentence. That is the biggest shock in the entire world to have that happen.""There's got to be some help there, emotional help, as much as the physical-medical aspect of it.""A psychologist or a therapist. Whatever you wanna call it because, I mean, really and truly, you are filled with emotions of all different kinds. Panic, fear, sadness. You know? Just totally in awe about everything. And your life's been turned upside down.""The coordination of mental health . . . as part of the staff too where they can coordinate with the patient to, I guess, understand, cope with, get adjusted to this new way of life and being given this diagnosis.""This is the opportunity to interact very closely with other people who are similarly situated. You know, it's a great source of support as well as information . . . You can get together on a regular basis with a group of folks, who are similarly situated, and you'll find it to be a very beneficial just to be able to spend time together and compare notes. That, to me, I think is a very important part of the support that would be provided by this center.""I think a support group is critical in your area in some way. It doesn't have to be at your center, but I think also, what I'm hearing more an

Exercise limitation associated with most lung diseases is multifactorial and is due to complex interactions between impaired ventilatory, cardiovascular, and peripheral muscle responses. Cardiopulmonary exercise tests (CPETs) are often required to ascertain the primary cause of exercise limitation particularly in patients with multiple co-morbidities. CPETs are generally offered only at tertiary care medical centers.

AbstractPurposeAlcohol withdrawal syndrome (AWS) is commonly treated in medical ICUs and typically requires high resource utilization. Dexmedetomidine for AWS has not been extensively investigated, and guidelines regarding its use are lacking. We evaluated the association between dexmedetomidine use in AWS and ICU length of stay (LOS).MethodsWe performed a multi-institutional retrospective cohort study of patients in the ICU with the primary diagnosis of AWS. ICU LOS of those treated with benzodiazepines alone vs. benzodiazepines plus dexmedetomidine was compared. Negative binomial regression was performed to test whether dexmedetomidine use was associated with increased ICU LOS after adjustment for age, gender, body mass index, and the time between hospital and ICU admission.ResultsFour hundred thirty-eight patients from eight institutions were included. Patients treated with benzodiazepines plus dexmedetomidine had higher Clinical Institute Withdrawal Assessment for Alcohol scores at ICU admission, spent longer on the medical wards prior to ICU admission, and had longer unadjusted ICU LOS (p < 0.0001). After covariate adjustment, dexmedetomidine remained associated with longer ICU LOS (relative mean to non-dexmedetomidine group 2.14, 95% CI 1.78–2.57,p < 0.0001).ConclusionsCompared to benzodiazepines alone, dexmedetomidine for the treatment of AWS was associated with increased ICU LOS. These results provide evidence that dexmedetomidine may increase resource utilization.

The Boston Marathon is the most popular marathon in the New England region and attracts some of the most qualified athletes participating from the United States and abroad. The race occurs in April, a month in the northeast characterized by unpredictable weather. While there are a number of well described weather-related medical complications that occur during exercise, less is known about noncardiogenic pulmonary edema (NCPE) in marathon runners, a condition that most physician are unfamiliar with. This phenomenon has been described in the literature as a complication of severe hyponatremia and cerebral edema. Here, we describe the case of a healthy athlete who took part in the Boston Marathon in 2018 and presented afterwards with hypothermia and NCPE. We also review the normal cardiopulmonary physiology along with the physiological changes and external factors impacting the respiratory system during exercise. The combination of significant physical exertion, cold and rainy weather and subsequent hypothermia, perhaps along with other less understood factors may have increased the permeability of his lungs and caused NCPE.

Components of the hedgehog signaling pathway are upregulated in patients with idiopathic pulmonary fibrosis (IPF). Vismodegib, a small-molecule inhibitor of hedgehog signaling, when used in combination with currently available antifibrotic therapy, may be more efficacious than antifibrotics alone. The objective of this study was to evaluate the safety and tolerability of vismodegib plus pirfenidone in patients with IPF. Twenty-one patients were enrolled in a phase 1b open-label trial to receive vismodegib 150 mg plus pirfenidone 2403 mg/day once daily. Key endpoints were safety, tolerability, and pharmacokinetics. Exploratory endpoints included change from baseline to week 24 in % predicted forced vital capacity (FVC) and University of California, San Diego Shortness of Breath Questionnaire (UCSD-SOBQ) scores, as well as pharmacodynamic changes in hedgehog biomarker C-X-C motif chemokine ligand 14 (CXCL14). All patients reported at least one treatment-emergent adverse event (AE), most frequently muscle spasms (76.2%). Serious AEs were reported in 14.3% of patients; one event of dehydration was considered related to vismodegib. One patient died due to IPF progression, unrelated to either treatment. More patients discontinued vismodegib than pirfenidone (42.9% vs. 33.3%, respectively). Changes from baseline to week 24 in % predicted FVC and UCSD-SOBQ scores were within known endpoint variability. In contrast to findings in basal cell carcinoma, vismodegib had no effect on circulating CXCL14 levels. The safety profile was generally consistent with the known profiles of both drugs, with no new safety signals observed in this small cohort. There was no pharmacodynamic effect on CXCL14 levels. Future development of vismodegib for IPF may be limited due to tolerability issues. ClinicalTrials.gov NCT02648048. Plain language summary available for this article. F. Hoffmann-La Roche Ltd. and Genentech, Inc.

BACKGROUND:Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease characterized by irreversible loss of lung function and an unpredictable course of disease progression.METHODS:The safety data for patients with IPF who received placebo in 6 clinical trials were pooled to examine the categories and frequencies of serious adverse events (SAEs) in this population.RESULTS:In 1082 patients with IPF who received placebo, 673 SAEs were reported. Of these, 93 SAEs resulted in death (8.6% of patients). Respiratory-related conditions were the most frequently reported SAE (225 events, 16.33 per 100 patient-exposure years [PEY]), followed by infections and infestations (136 events, 9.87 per 100 PEY) and cardiac disorders (79 events, 5.73 per 100 PEY); these categories also had the most fatal outcomes (60, 10, and 10 deaths, respectively). The most frequently reported fatal respiratory-related SAEs were IPF and respiratory failure (38 and 11 patients, respectively), and the most frequently reported fatal infections and infestations and cardiac disorders were pneumonia (5 patients) and myocardial infarction (3 patients), respectively.CONCLUSIONS:This pooled analysis has value as a comparator for safety in future studies of IPF and provides insights in the natural evolution of both IPF and common comorbidities.

SESSION TITLE: IPF: From Diagnosis to Treatment SESSION TYPE: Original Investigations PRESENTED ON: 10/21/2019 01:30 pm - 02:30 pm PURPOSE: Pirfenidone is an approved treatment for idiopathic pulmonary fibrosis (IPF) that slows lung function decline. Although pirfenidone is generally well tolerated, some patients discontinue therapy due to adverse events (AEs). Temporary dose modifications may promote AE resolution and facilitate long-term treatment maintenance. To further understand which modification strategies may mitigate pirfenidone-related AEs in patients with IPF, a varied sample of US pulmonologists was surveyed. METHODS: An online, self-administered survey was fielded to pulmonologists between April 10 and May 17, 2017, by MedPanel, Inc. Pulmonologists were included if they spent >20% of their time in direct patient care and had ≥5 patients with IPF receiving antifibrotics. Pulmonologists answered questions about potential AE management in patients receiving pirfenidone maintenance therapy and rank ordered the steps they considered: temporary dose interruption, temporary dose reduction, prescribe/recommend additional agents, permanent discontinuation or unsure. Mean rankings were summarized by physician type (interstitial lung disease [ILD] specialists and community-based pulmonologists). RESULTS: A total of 169 pulmonologists responded; 69 (40.8%) and 100 (59.2%) were from ILD centers and community practices, respectively. Pulmonologists reported that a mean 58.6% (SD, 22.7%) of patients with IPF were receiving pirfenidone. For potential gastrointestinal (GI) AEs, such as nausea and vomiting, ILD pulmonologists ranked temporary dose reduction first followed by temporary dose interruption, prescribe/recommend additional agents and permanent discontinuation. Community pulmonologists also ranked temporary dose reduction first for potential GI AEs. For potential skin-related AEs, such as photosensitivity and rash, both groups ranked temporary dose interruption first before temporary dose reduction, followed by prescribe/recommend additional agents and permanent discontinuation of pirfenidone. Both ILD and community pulmonologist groups reported nearly half of patients (45% and 44%, respectively) achieved the maintenance dose of pirfenidone (2403 mg/d) after dose modification. Both pulmonologist groups had similar frequencies of patients discontinuing due to AEs, with the majority having CONCLUSIONS: Overall, pulmonologists typically use a temporary dose reduction first to manage pirfenidone-related GI AEs. For skin-related AEs, a temporary dose interruption is usually used first. Almost half of patients requiring dose modification can achieve the full maintenance dose of pirfenidone. CLINICAL IMPLICATIONS: Treatment persistence with pirfenidone can be maintained by effective dose-modification strategies. DISCLOSURES: Employee $100000 Added 03/14/2019 by Cindy Burg, source=Web Response, value=Salary Consultant relationship with Genentech, Inc. Please note: $1001 - $5000 Added 03/13/2019 by Tmirah Haselkorn, source=Web Response, value=Consulting fee Consultant relationship with Novartis Pharmaceuticals Corp. Please note: $1-$1000 Added 03/13/2019 by Tmirah Haselkorn, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Genentech, Inc. Please note: $1001 - $5000 Added 03/06/2019 by Peter LaCamera, source=Web Response, value=Consulting fee Consultant relationship with Genentech, Inc. Please note: $1001 - $5000 Added 03/06/2019 by Peter LaCamera, source=Web Response, value=Consulting fee Employee relationship with Roche Please note: >$100000 Added 03/14/2019 by John Stauffer, source=Web Response, value=Salary Employee $100000 Added 03/14/2019 by John Stauffer, source=Web Response, value=Ownership interest Speaker/Speaker's Bureau relationship with Genentech Please note: $1-$1000 Added 03/14/2019 by Mark Wencel, source=Web Response, value=Honoraria Employee relationship with Genentech, Inc. Please note: >$100000 Added 03/15/2019 by Ming Yang, source=Web Response, value=Salary

SESSION TITLE: Diffuse Lung Disease SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 pm - 02:00 pm PURPOSE: Pirfenidone is approved for the treatment of idiopathic pulmonary fibrosis (IPF), reduces lung function decline and improves progression-free survival. Although pirfenidone is generally well tolerated, a subset of patients discontinue therapy due to gastrointestinal- and fatigue-related adverse events (AEs). Physician characteristics and treatment strategies stratified by physician-reported patient discontinuation rates due to AEs are described in a varied sample of US pulmonologists. METHODS: An online, self-administered survey was fielded to pulmonologists between April 10 and May 17, 2017. Pulmonologists were included if they spend >20% of their time in direct patient care and had ≥5 patients with IPF receiving antifibrotic therapy. Physicians were stratified by their self-reported proportion of patients discontinuing pirfenidone (≤20% vs. >20%) after answering questions about initiating pirfenidone treatment and management of AEs. RESULTS: A total of 167 pulmonologists responded; 110 (65.9%) and 57 (34.1%) reported ≤20% and >20% of patients discontinuing pirfenidone due to AEs, respectively. In the ≤20% and >20% groups, 41.8% and 36.8%, respectively, practice in an interstitial lung disease center; in both groups, most spend >80% of their time in direct patient care and have 11 to 20 years of experience. More physicians in the ≤20% group practice at an academic medical center vs. the >20% group (63.6% vs. 45.6%, respectively). The mean (SD) number of patients with IPF in each practice was 70.3 (122.7) in the ≤20% group and 60.6 (80.3) in the >20% group. Most physicians in both groups specify when to take pirfenidone relative to a meal (e.g., before, during or after), with the majority counseling during the meal. However, a lower proportion in the ≤20% group recommend during a meal vs. those in the >20% group (56.7% vs. 71.9%, respectively), whereas a higher proportion in the ≤20% group recommend after a meal (28.3% vs. 18.8%, respectively). Physicians in the ≤20% group were less likely to suggest additional therapy to manage AEs, including anti-nausea and anti-diarrheal drugs. Physicians in the ≤20% group were more likely to report strategies to prevent fatigue as being effective than those in the >20% group (33.3% vs. 19.4%, respectively). CONCLUSIONS: Physicians who reported a lower rate of tolerability-related discontinuations (≤20%) had more patients with IPF in their practice. These physicians are more likely to recommend taking pirfenidone after a meal, use fewer additional therapies to manage AEs and reported more-effective strategies to prevent fatigue in their patients vs. physicians with a higher rate of discontinuations (>20%). CLINICAL IMPLICATIONS: Treatment persistence with pirfenidone can be maintained by effective dose titration and AE management strategies. DISCLOSURES: Consultant relationship with Genentech, Inc. Please note: $1001 - $5000 Added 02/21/2018 by Tmirah Haselkorn, source=Web Response, value=Consulting fee No relevant relationships by Peter LaCamera, source=Web Response Employee relationship with Genentech Please note: >$100000 Added 02/23/2018 by Susan Limb, source=Web Response, value=Salary Employee relationship with Genentech, Inc. Please note: >$100000 Added 02/22/2018 by Elizabeth Morgenthien, source=Web Response, value=Salary Stockholder relationship with Roche Please note: $5001 - $20000 Added 02/22/2018 by Elizabeth Morgenthien, source=Web Response, value=Stock Stockholder relationship with Bristol Myers Squibb Please note: $20001 - $100000 Added 02/22/2018 by Elizabeth Morgenthien, source=Web Response, value=Stock Stockholder relationship with Zimmer Please note: $20001 - $100000 Added 02/22/2018 by Elizabeth Morgenthien, source=Web Response, value=Stock Employee Removed 02/22/2018 by John Stauffer, source=Web Response Employee relationship with Genentech, Inc. Please note: $20001 - $100000 Added 02/22/2018 by John Stauffer, source=Web Response, value=Stock holder Speaker/Speaker's Bureau relationship with Genentech, Inc. Please note: $5001 - $20000 Added 02/27/2018 by Mark Wencel, source=Web Response, value=Consulting fee Employee relationship with Genentech, Inc. Please note: >$100000 Added 02/22/2018 by Ming Yang, source=Web Response, value=Salary

Pirfenidone is an oral antifibrotic agent approved for idiopathic pulmonary fibrosis (IPF). Real-world data on adverse event (AE) management for pirfenidone are limited. Strategies for managing potential antifibrotic therapy AEs were examined in a sample of US pulmonologists.

Dyspnea is a meaningful outcome to patients with IPF. Pamrevlumab, a mAb against connective tissue growth factor, has shown favorable safety, efficacy and QoL (SGRQ) results in PRAISE, a Phase 2, placebo-controlled trial. The UCSD-SOBQ is a respiratory questionnaire used in IPF trials, and in a subset of patients in PRAISE. The UCSD-SOBQ assesses dyspnea associated with activities of daily living (ADL). Subjects indicate severity of SOB on a 6-point scale in 21 ADL. Three additional questions ask about fear of harm from overexertion, limitations and fear caused by SOB. A total score ranges from 0 to 120, with higher scores indicating greater impairment. In a subset (N=42) of patients in PRAISE the UCSD-SOBQ was administered at baseline and every 12 weeks. Data were analyzed using ANCOVA, with missing data imputed using the predicted value from a random coefficient linear regression model. Baseline mean values for pamrevlumab and placebo were: age 68.6 and 65.9 years; FVC%-predicted 74.5 and 73.1%; GAP Score 3.5 and 3.2 and similar GAP Stage distribution. The mean baseline UCSD-SOBQ total score was 39.1 and 31.8 points. The between-groups absolute difference from baseline to Week 48 was statistically significant. These UCSD-SOBQ results suggest that pamrevlumab attenuates dyspnea progression in IPF patients in comparison to placebo.

Rationale. The impact of emergency department length of stay (EDLOS) upon sepsis outcomes needs clarification. We sought to better understand the relationship between EDLOS and both outcomes and protocol compliance in sepsis. Methods. We performed a retrospective observational study of septic patients admitted to the ICU from the ED between January 2012 and December 2015 in a single tertiary care teaching hospital. 287 patients with severe sepsis and septic shock were included. Study population was divided into patients with EDLOS < 6 hrs (early admission) versus ≥6 hours (delayed admission). We assessed the impact of EDLOS on hospital mortality, compliance with sepsis protocol, and resuscitation. Statistical significance was determined by chi-square test. Results. Of the 287 septic ED patients, 137 (47%) were admitted to the ICU in <6 hours. There was no significant in-hospital mortality difference between early and delayed admissions (p = 0.68). Both groups have similar compliance with the 3-hour protocol (p = 0.77). There was no significant difference in achieving optimal resuscitation within 12 hours (p = 0.35). Conclusion. We found that clinical outcomes were not significantly different between early and delayed ICU admissions. Additionally, EDLOS did not impact compliance with the sepsis protocol with the exception of repeat lactate draw.

Methods . This observational retrospective cohort study includes 318 ARDS patients extracted from an ICU database between the years of 2001 and 2008. Clinical factors including age, gender, comorbidity score, Sequential Organ Failure Assessment (SOFA) score, and PaO 2 /FiO 2 ratio were chosen for the base model to predict ICU mortality. The RDW value at the time of ARDS diagnosis was added to the base model to determine if it improved its predictive ability. Results . 318 subjects were included; 113 (36%) died in the ICU. AUC for the base model without RDW was 0.76, and 0.78 following the addition of RDW [p=0.048]. The NRI was 0.46 (p=0.001), indicating that, in 46% of patients, the predictive probability of the model was improved by the inclusion of RDW. Conclusions . Adding RDW at time of ARDS diagnosis improved discrimination in a model using 4 clinical factors to predict ICU mortality.