Joshua Mooney, MD, MS, is a board certified pulmonologist and critical care physician who specializes in the care of interstitial lung disease and lung transplant patients. He performs health services and outcomes research focused on understanding and improving the lives and care of patients with advanced lung disease and is actively involved in clinical trials to improve outcomes in interstitial lung disease. He is the director of the Pulmonary Fibrosis Foundation Care Center at Stanford and serves on national and international committees related to pulmonary fibrosis and lung transplantation.
- Lung Transplantation
- Heart Lung Transplantation
- Interstitial Lung Disease
- Idiopathic Pulmonary Fibrosis
- Hypersensitivity Pneumonitis
- Pulmonary Disease
Clinical Assistant Professor, Medicine - Pulmonary & Critical Care Medicine
Director, Stanford Pulmonary Fibrosis Foundation Care Center (2016 - Present)
Honors & Awards
Medical Honor Society, Alpha Omega Alpha (AOA) (2008)
Medical Honor Society, Gold Humanism (2008)
KL2 Career Development Award, Spectrum, Stanford Center for Clinical and Translational Research and Education (2014)
Transplant Registry Early Career Award, International Society for Heart and Lung Transplantation (2016)
Boards, Advisory Committees, Professional Organizations
Editorial Board, Annals of the American Thoracic Society (2018 - Present)
Communications Committee, Pulmonary Fibrosis Foundation (2017 - Present)
Pulmonary Transplant Council Liason, Junior Faculty and Trainee Council, International Society for Heart and Lung Transplantation (2016 - Present)
Executive Committee, Junior Faculty Representative, American Society of Transplantation Thoracic and Critical Care Community of Practice (2015 - 2016)
Residency:UCSF Internal Medicine Residency (2012) CA
MS, Stanford University, Health Services Research (2016)
Board Certification: Critical Care Medicine, American Board of Internal Medicine (2015)
Fellowship:Stanford University (2015)
Board Certification: Pulmonary Disease, American Board of Internal Medicine (2014)
Board Certification: Internal Medicine, American Board of Internal Medicine (2012)
MD, University of Minnesota Medical School, Medicine (2009)
Intern, University of California San Francisco, Internal Medicine (2010)
Resident, University of California San Francisco, Internal Medicine (2012)
Fellow, Stanford University, Pulmonary and Critical Care Medicine (2015)
Current Research and Scholarly Interests
Outcomes and Health Services Research in Advanced Lung Disease & Lung Transplant
A Study to Evaluate the Efficacy and Safety of CC-90001 in Subjects With Idiopathic Pulmonary Fibrosis
This is a Phase 2, multicenter, multinational, randomized, double-blind, placebo-controlled study evaluating the efficacy, safety, pharmacokinetics (PK), quality of life and exploratory pharmacodynamics (PD) of two treatment doses of CC-90001, 200 mg and 400 mg, compared with placebo, when delivered once daily per os (PO) in subjects with idiopathic pulmonary fibrosis (IPF). This study is designed to assess response to treatment by using measures of lung function, disease progression, fibrosis on radiography, and patient-reported outcomes. It will also assess dose response.
A Trial to Compare Nintedanib With Placebo for Patients With Scleroderma Related Lung Fibrosis
Systemic Sclerosis (SSc) is a devastating disease of unknown etiology. Patients suffer from multiple organ fibrosis whereas lung fibrosis (interstitial lung disease, ILD) is one of the main driver for mortality. There is preclinical evidence for efficacy of nintedanib in SSc and associated ILD (SSc-ILD) and the anti-fibrotic efficacy of nintedanib was proven in idiopathic pulmonary fibrosis patients, who are presenting a similar pattern regarding lung fibrosis. Hence it is the purpose of the trial to confirm the efficacy and safety of nintedanib 150 mg bid in treating patients with SSc-ILD, compared with placebo. The trial will be conducted as a double blind, randomised, placebo-controlled trial with primary efficacy evaluation at week 52 and placebo-controlled treatment until last patient out (up to a maximum of 100 weeks). Respiratory function is globally accepted for assessment of treatment effects in patients with lung fibrosis. The chosen endpoint (Forced Vital Capacity (FVC) decline) is easy to obtain and is part of the usual examinations done in patients with SSc-ILD.
A Trial to Evaluate the Safety of Long Term Treatment With Nintedanib in Patients With Scleroderma Related Lung Fibrosis.
The main objective is to assess long term safety of treatment with oral nintedanib in patients with Systemic Sclerosis associated Interstitial Lung Disease (SSc-ILD).
CleanUP IPF for the Pulmonary Trials Cooperative
The purpose of this study is to compare the effect of standard care, versus standard of care plus antimicrobial therapy (co-trimoxazole or doxycycline), on clinical outcomes in patients diagnosed with idiopathic pulmonary fibrosis (IPF).
Detection of Integrin avb6 in Idiopathic Pulmonary Fibrosis Using PET/CT
The investigators wish to evaluate the feasibility of [18F]FP-R01-MG-F2 PET/CT scanning in patients with Idiopathic Pulmonary Fibrosis.
Pulmonary Fibrosis Foundation Patient Registry
The Pulmonary Fibrosis Foundation Patient Registry will collect data on at least 2,000 patients with interstitial lung disease (ILD) at approximately 40 clinical sites in the US. The Registry is targeting enrollment of approximately 60% of the 2,000 ILD participants to have idiopathic pulmonary fibrosis (IPF). The aim of the Registry is to create a cohort of well-characterized patients with interstitial lung disease (ILD) for participation in retrospective and prospective research
Safety and Tolerability Study of Pirfenidone in Combination With Nintedanib in Participants With Idiopathic Pulmonary Fibrosis (IPF)
This clinical study will evaluate the safety and tolerability of combination treatment of nintedanib and pirfenidone in participants with IPF. Eligible participants must have received pirfenidone for at least 16 weeks on a stable dose. Nintedanib will be added on Day 1 of the study as a combination treatment for IPF for 24 weeks.
- Potential Delays in Diagnosis of Idiopathic Pulmonary Fibrosis in Medicare Beneficiaries. Annals of the American Thoracic Society 2019
Multiple Listing in Lung Transplant Candidates: A Cohort Study.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
Lung transplant candidates can be waitlisted at more than one transplant center, a practice known as multiple listing. The factors associated with multiple listing and whether multiple listing modifies waitlist mortality or likelihood of lung transplant is unknown. US lung transplant waitlist candidates were identified as either single or multiple listed using data from the Scientific Registry of Transplant Recipients. Characteristics of single and multiple listed candidates were compared and multivariable logistic regression was used to estimate associations with multiple listing. Multiple listed candidates were matched to single listed candidates using a combination of exact and propensity score matching methods. Cox proportional hazard models were used to estimate the relationship of multiple listing on waitlist mortality and receiving a transplant. Multiple listing occurred in 2.3% of lung transplant waitlist candidates. Younger age, female gender, white race, short stature, high antibody sensitization, college or post-college education, lower lung allocation score, and a cystic fibrosis diagnosis were independently associated with multiple listing. Multiple listing was associated with an increased likelihood of lung transplant (adjusted hazard ratio [aHR] 2.74, 95% CI 2.37 to 3.16) but was not associated with waitlist mortality (aHR 0.99, 95% CI 0.68 to 1.44). This article is protected by copyright. All rights reserved.
View details for DOI 10.1111/ajt.15124
View details for PubMedID 30253057
Effect of broader geographic sharing of donor lungs on lung transplant waitlist outcomes.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
The United States lung allocation system prioritizes allocation based on medical urgency and benefit but does not address a federal mandate for broader geographic organ sharing. It is unknown whether broader geographic sharing of donor lungs would improve lung transplant waitlist outcomes.A discrete event microsimulation model simulated donor lung allocation according to different geographic lung-sharing policies, including the historic local donor service area (DSA)-based policy and hypothetical policies that prioritize candidates to donors within 500-mile or 1,000-mile geographic radii. Candidate waitlist mortality, number of transplants, and 1-year survival were compared across organ allocation policies. Waitlist mortality rates were further stratified by diagnosis, Lung Allocation Score (LAS) threshold, ABO blood type, and region.Under broader geographic lung sharing, the proportion of chronic obstructive pulmonary disease transplant recipients decreased, whereas the proportion of pulmonary fibrosis recipients increased. Waitlist mortality decreased with broader geographic lung sharing with a 21.3% decrease in waitlist mortality with 500-mile lung sharing and a 31.8% decrease in waitlist mortality with 1,000-mile lung sharing. The decrease in waitlist deaths occured across all U.S. geographic regions and was greatest in candidates with pulmonary fibrosis and/or high medical urgency.Broader geographic sharing of donor lungs could reduce waitlist mortality, particularly among pulmonary fibrosis and high-medical-urgency candidates.
View details for DOI 10.1016/j.healun.2018.09.007
View details for PubMedID 30344025
Racial and ethnic disparities in lung transplant listing and waitlist outcomes.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
The United States lung transplant registry data demonstrate differences in adult waitlist mortality by race/ethnicity. It is unknown whether these differences persist after risk adjustment or occur secondary to disparities in disease severity at the time of listing.Adult lung transplant waitlist candidates between May 4, 2005 and March 5, 2015 were identified and compared by non-Hispanic white (NHW), non-Hispanic black (NHB), Hispanic and Asian race/ethnicity. A competing risk proportional hazards model was used to assess the association of race/ethnicity with the unadjusted and adjusted risk of waitlist death or removal for too sick, transplant, or removal for other reason. Disease illness severity at transplant listing was compared by race/ethnicity.There were 20,684 lung transplant candidates identified (82% NHW, 9% NHB, 6% Hispanic, 2% Asian and 1% other). Non-white candidates had higher unadjusted waitlist mortality, which was fully mitigated by adjusting for other risk factors (NHB: hazard ratio [HR] 1.05, 95% confidence interval [CI] 0.93 to 1.18; Hispanic: HR 1.02, 95% CI 0.99 to 1.18; Asian: HR 0.90, 95% CI 0.70 to 1.16). Adjusted waitlist access to transplant was lower in non-white candidates (NHB: HR 0.88, 95% CI 0.83 to 0.94; Hispanic: HR 0.87, 95% CI 0.81 to 0.94; Asian: HR 0.83, 95% CI 0.73 to 0.96). NHW candidates with obstructive lung disease and pulmonary fibrosis were older with less illness severity at listing than non-white candidates.Within the current lung allocation system, there is no difference in risk-adjusted waitlist mortality by race/ethnicity, but non-white waitlist candidates have lower risk-adjusted access to lung transplant. Non-white candidates are generally younger with greater disease-specific illness severity at the time of lung transplant listing.
View details for DOI 10.1016/j.healun.2017.09.017
View details for PubMedID 29129372
- COUNTERPOINT: Should BAL Be Routinely Performed in the Diagnostic Evaluation of Idiopathic Pulmonary Fibrosis? No. Chest 2017
Effect of Transplant Center Volume on Cost and Readmissions in Medicare Lung Transplant Recipients.
Annals of the American Thoracic Society
2016; 13 (7): 1034-1041
While lung transplant recipient survival is better at higher volume centers, the effect of center volume on admission cost and early hospital readmission is unknown.To understand the association between transplant center volume and recipient risk-adjusted transplant admission cost, in-hospital mortality, and early hospital readmission in lung transplant recipients.Medicare lung transplant recipients from May 4, 2005 to December 31, 2011 were identified through linkage of transplant registry and Medicare administrative claims. Transplant admission cost was extracted, adjusted for regional price variation, and compared across low, intermediate, and high volume centers. A multivariable hierarchical generalized linear regression model was used to assess the effect of transplant center volume on recipient adjusted cost. Modified Poisson regression models were used to assess adjusted in-hospital mortality and early hospital readmission by transplant center volume.There were 3,128 Medicare lung transplant recipients identified. Unadjusted transplant cost was lower at high volume centers (mean $131,352, SD±$106,165; median $90,177, IQR $79,165-$137,915) than intermediate (mean $138,792, SD±$106,270; median $93,024, IQR $82,700-$154,857) or low volume (mean $143,609, SD±$123,316; median $95,234, IQR $83,052-$152,149) centers (p<0.0001). After adjusting for recipient health risk, low volume centers had an 11.66% greater transplant admission cost (p=0.040), a 41% greater risk for in-hospital mortality (p=0.015), and a 14% greater risk for early hospital readmission (p=0.033) compared to high volume centers. There was no significant difference in transplant cost, in-hospital mortality, or early hospital readmission between intermediate and high volume centers.Lung transplant admission cost, in-hospital mortality, and early hospital readmission rate are lower at high volume centers compared to low volume centers.
View details for DOI 10.1513/AnnalsATS.201601-017OC
View details for PubMedID 27064753
Lung Quality and Utilization in Controlled Donation After Circulatory Determination of Death Within the United States
AMERICAN JOURNAL OF TRANSPLANTATION
2016; 16 (4): 1207-1215
Although controlled donation after circulatory determination of death (cDCDD) could increase the supply of donor lungs within the United States, the yield of lungs from cDCDD donors remains low compared with donation after neurologic determination of death (DNDD). To explore the reason for low lung yield from cDCDD donors, Scientific Registry of Transplant Recipient data were used to assess the impact of donor lung quality on cDCDD lung utilization by fitting a logistic regression model. The relationship between center volume and cDCDD use was assessed, and the distance between center and donor hospital was calculated by cDCDD status. Recipient survival was compared using a multivariable Cox regression model. Lung utilization was 2.1% for cDCDD donors and 21.4% for DNDD donors. Being a cDCDD donor decreased lung donation (adjusted odds ratio 0.101, 95% confidence interval [CI] 0.085-0.120). A minority of centers have performed cDCDD transplant, with higher volume centers generally performing more cDCDD transplants. There was no difference in center-to-donor distance or recipient survival (adjusted hazard ratio 1.03, 95% CI 0.78-1.37) between cDCDD and DNDD transplants. cDCDD lungs are underutilized compared with DNDD lungs after adjusting for lung quality. Increasing transplant center expertise and commitment to cDCDD lung procurement is needed to improve utilization.
View details for DOI 10.1111/ajt.13599
View details for Web of Science ID 000373075400021
Increased Resource Use in Lung Transplant Admissions in the Lung Allocation Score Era
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
2015; 191 (3): 302-308
Rationale: In 2005, the lung allocation score (LAS) was implemented to prioritize organ allocation to minimize waiting-list mortality and maximize one-year survival. It resulted in transplantation of older and sicker patients without changing one-year survival. Its effect on resource utilization is unknown. Objective: To determine changes in resource utilization over time in lung transplant admissions Methods: Solid organ transplant recipients were identified within the Nationwide Inpatient Sample (NIS) data from 2000 to 2011. Joinpoint regression methodology was performed to identify a time point of change in mean total hospital charges amongst lung transplant and other solid organ transplant recipients. Two temporal lung transplant recipient cohorts identified by joinpoint regression were compared for baseline characteristics and resource utilization, including total charges for index hospitalization, charges per day, length of stay, discharge disposition, tracheostomy, and need for extracorporeal membrane oxygenation (ECMO). Measurements and Main Results: A significant point of increased total hospital charges occurred for lung transplant recipients in 2005, corresponding to LAS implementation, that was not seen in other solid organ transplant recipients. Total transplant hospital charges increased by 40% in the post-LAS cohort [$569,942 ($53,229) vs. $407,489 ($28,360)] along with an increased median length of stay, daily charges, and discharge disposition other than to home. Post-LAS recipients also had higher post-transplant utilization of ECMO (OR 2.35, 95% CI 1.56, 3.55) and higher incidence of tracheostomy (OR 1.52, 95% CI 1.22, 1.89). Conclusions: LAS implementation is associated with a significant increase in resource utilization during index hospitalization for lung transplant.
View details for DOI 10.1164/rccm.201408-1562OC
View details for Web of Science ID 000348827000014
View details for PubMedID 25517213
Impact of the lung allocation score on survival beyond 1 year.
American journal of transplantation
2014; 14 (10): 2288-2294
Implementation of the lung allocation score (LAS) in 2005 led to transplantation of older and sicker patients without altering 1-year survival. However, long-term survival has not been assessed and emphasizing the 1-year survival metric may actually sustain 1-year survival while not reflecting worsening longer-term survival. Therefore, we assessed overall and conditional 1-year survival; and the effect of crossing the 1-year threshold on hazard of death in three temporal cohorts: historical (1995-2000), pre-LAS (2001-2005) and post-LAS (2005-2010). One-year survival post-LAS remained similar to pre-LAS (83.1% vs. 82.1%) and better than historical controls (75%). Overall survival in the pre- and post-LAS cohorts was also similar. However, long-term survival among patients surviving beyond 1 year was worse than pre-LAS and similar to historical controls. Also, the hazard of death increased significantly in months 13 (1.44, 95% CI 1.10-1.87) and 14 (1.43, 95% CI 1.09-1.87) post-LAS but not in the other cohorts. While implementation of the LAS has not reduced overall survival, decreased survival among patients surviving beyond 1 year in the post-LAS cohort and the increased mortality occurring immediately after 1 year suggest a potential negative long-term effect of the LAS and an unintended consequence of increased emphasis on the 1-year survival metric.
View details for DOI 10.1111/ajt.12903
View details for PubMedID 25208599
Radiographic Fibrosis Score Predicts Survival in Hypersensitivity Pneumonitis
2013; 144 (2): 586-592
It is unknown if the radiographic fibrosis score predicts mortality in persistent hypersensitivity pneumonitis (HP) and if survival is similar to that observed in idiopathic pulmonary fibrosis (IPF) when adjusting for the extent of radiographic fibrosis.We reviewed records from 177 patients with HP and 224 patients with IPF whose diagnoses were established by multidisciplinary consensus. Two thoracic radiologists scored high-resolution CT (HRCT) scan lung images. Independent predictors of transplant-free survival were determined using a Cox proportional hazards analysis. Kaplan-Meier survival curves were constructed, stratified by disease as well as fibrosis score.HRCT scan fibrosis score and radiographic reticulation independently predicted time to death or lung transplantation. Clinical predictors included a history of cigarette smoking, auscultatory crackles on lung examination, baseline FVC, and FEV1/FVC ratio. The majority of HP deaths occurred in patients with both radiographic reticulation and auscultatory crackles on examination, compared with patients with only one of these manifestations (P < .0001). Patients with IPF had worse survival than those with HP at any given degree of radiographic fibrosis (hazard ratio 2.31; P < .01).Survival in patients with HP was superior to that of those with IPF with similar degrees of radiographic fibrosis. The combination of auscultatory crackles and radiographic reticulation identified patients with HP who had a particularly poor outcome.
View details for DOI 10.1378/chest.12-2623
View details for Web of Science ID 000323021400035
View details for PubMedID 23392130
Hospital cost and length of stay in idiopathic pulmonary fibrosis
JOURNAL OF MEDICAL ECONOMICS
2017; 20 (5): 518-524
To provide a detailed picture of the economic impact of hospitalization in idiopathic pulmonary fibrosis (IPF) and to identify factors associated with cost and length of stay (LOS).In this retrospective cross-sectional study using the Nationwide Inpatient Sample (NIS), we included hospitalizations for IPF (ICD-9-CM 516.3) with a principal diagnosis of respiratory disease (ICD-9-CM 460-519) from 2009-2011; lung transplant admissions were excluded. Total inpatient cost, LOS, in-hospital death, and discharge disposition were reported. Linear regression models were used to determine variables predictive of LOS and cost.From 2009-2011, 22,350 non-transplant IPF patients with a principal diagnosis of respiratory disease were admitted: mean (+/-SE) age was 70.0 (0.32), and 49.1% were female. While in-hospital, 11.4% of patients received mechanical ventilation and 8.9% received non-invasive ventilation. Mean (+/-SE) LOS was 7.4 (0.15) days overall (p < 0.001). The mean (+/-SD) admission cost was $16,042 (+/-631). Of hospitalized patients, 14.1% died, 20.6% transferred facilities, and 46.4% were routinely discharged. The adjusted LOS (95%CI) for patients with and without mechanical ventilation was 16.1 days (15 - 17.5) versus 6.3 (6 - 6.5); adjusted costs were $48,772 (43,979 - 53,565) versus $11,861 (11,292 - 12,431).The positive predictive value of the algorithm used to identify IPF is not optimal. The NIS database does not follow patients longitudinally and claims after admission are not available. Claims do not indicate whether listed diagnoses were present on admission or developed during hospitalization. The exclusion of transplant-related expenditures lead to underestimation of cost.Using a nationally-representative database, we found IPF respiratory-related hospitalizations represent a significant economic burden with approximately 7,000 non-transplant IPF admissions per year, at a mean cost of $16,000 per admission. Mechanical ventilation is associated with statistically significant increases in LOS and cost. Therapeutic advances that reduce rates and costs of IPF hospitalizations are needed.
View details for DOI 10.1080/13696998.2017.1282864
View details for Web of Science ID 000399655000010
View details for PubMedID 28092235
Delirium after lung transplantation: Association with recipient characteristics, hospital resource utilization, and mortality.
2017; 31 (5)
Delirium is associated with increased morbidity and mortality. The factors associated with post-lung transplant delirium and its impact on outcomes are under characterized.The medical records of 163 consecutive adult lung transplant recipients were reviewed for delirium within 5 days (early-onset) and 30 hospital days (ever-onset) post-transplantation. A multivariable logistic regression model assessed factors associated with delirium. Multivariable negative binomial regression and Cox proportional hazards models assessed the association of delirium with ventilator duration, intensive care unit (ICU) length of stay (LOS), hospital LOS and one-year mortality.Thirty six % developed early-onset and 44% - ever-onset delirium. Obesity (OR 6.35, 95% CI 1.61-24.98) and bolused benzodiazepines within the first post-operative day (OR 2.28, 95% CI 1.07-4.89) were associated with early-onset delirium. Early-onset delirium was associated with longer adjusted mechanical ventilation duration (p=0.001), ICU LOS (p<0.001), and hospital LOS (p=0.005). Ever-onset delirium was associated with longer ICU (p<0.001) and hospital LOS (p<0.001). After adjusting for clinical variables, delirium was not significantly associated with one-year mortality (early-onset HR 1.65, 95% CI 0.67-4.03; ever-onset HR 1.70, 95% CI 0.63-4.55).Delirium is common after lung transplant surgery and associated with increased hospital resources. This article is protected by copyright. All rights reserved.
View details for DOI 10.1111/ctr.12966
View details for PubMedID 28314081
Identification of Diagnostic Criteria for Chronic Hypersensitivity Pneumonitis: An International Modified Delphi Survey.
American journal of respiratory and critical care medicine
Current diagnosis of chronic hypersensitivity pneumonitis (cHP) involves considering a combination of clinical, radiological, and pathological information in multidisciplinary team discussions. However, this approach is highly variable with poor agreement between centers.We aimed to identify diagnostic criteria for cHP that reach consensus among international experts.A 3-round modified Delphi survey was conducted between April and August 2017. Forty-five experts in interstitial lung disease from 14 countries participated in the online survey. Diagnostic items included in round 1 were generated using expert interviews and literature review. During rounds 1 and 2, experts rated the importance of each diagnostic item on a 5-point Likert scale. The a priori threshold of consensus was ≥ 75% of experts rating a diagnostic item as very important or important. In the third round, experts graded the items that met consensus as important and provided their level of diagnostic confidence for a series of clinical scenarios.Consensus was achieved on 18 of the 40 diagnostic items. Among these, experts gave the highest level of importance to the identification of a causative antigen, time relation between exposure and disease, mosaic attenuation on chest imaging, and poorly formed non-necrotizing granulomas on pathology. In clinical scenarios, the diagnostic confidence of experts in cHP was heightened by the presence of these diagnostic items.This consensus-based approach for the diagnosis of cHP represents a first step towards the development of international guidelines for the diagnosis of cHP.
View details for DOI 10.1164/rccm.201710-1986OC
View details for PubMedID 29172641
- Hypersensitivity Pneumonitis: Lessons from the Great Imitator in Interstitial Lung Disease. Annals of the American Thoracic Society 2017; 14 (10): 1506–7
- Rebuttal from Drs Mooney and Collard. Chest 2017
- Changing the curve in chronic lung allograft dysfunction: Implications of chronic lung allograft dysfunction phenotypes in assessing treatment interventions. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2017
Mechanical ventilation in idiopathic pulmonary fibrosis: a nationwide analysis of ventilator use, outcomes, and resource burden.
BMC pulmonary medicine
2017; 17 (1): 84
Idiopathic pulmonary fibrosis (IPF) is associated with increased risk of respiratory-related hospitalizations. Studies suggest mechanical ventilation (MV) use in IPF does not improve outcomes and guidelines recommend against its general use. Our objective was to investigate MV use and association with cost and mortality in IPF.This retrospective study, using a nationwide sample, included claims with IPF (ICD-9-CM: 516.3) in 2009-2011 and principal respiratory disease diagnosis (ICD-9-CM: 460-519); excluding lung transplant. Regression models were used to determine predictors of MV and association with cost, LOS, and mortality. Domain analysis was used to account for use of subpopulation. Costs were adjusted to 2011. Data on patient severity not available.Twenty two thousand three hundred fifty non-transplant IPF patients were admitted with principal respiratory disease diagnosis: Mean age 70.0 (SD 13.9), 49.1% female, mean LOS 7.4 (SD 8.2). MV was used in 11.4% of patients with a non-significant decline over time. In regression models, MV was associated with an increased stay of 9.78 days (95% CI 8.38-11.18) and increased cost of $36,583 (95% CI $32,021-41,147). MV users had significantly increased mortality (OR 15.55, 95% CI 12.13-19.95) versus nonusers.Mechanical ventilation use has not significantly changed over time and is mostly used in younger patients and those admitted for non-IPF respiratory conditions. MV was associated with a 4-fold admission cost increase ($49,924 versus $11,742) and a 7-fold mortality increase (56% versus 7.5%), although patients who receive MV may differ from those who do not. Advances in treatment and decision aids are needed to improve outcomes in IPF.
View details for DOI 10.1186/s12890-017-0426-2
View details for PubMedID 28532459
View details for PubMedCentralID PMC5441011
Lung transplantation following death by drowning: a review of the current literature.
2016; 30 (10): 1195-1197
While multiple donor characteristics have been cited as ideal for lung transplantation, there are minimal widely accepted exclusion criteria. One criterion that many centers view with hesitation is death by drowning. However, recent literature suggests such donors may result in acceptable outcomes following transplation. This review highlights a case of a patient who underwent successful bilateral lung transplant from a donor following a drowning event. A review of the current literature is presented, concluding with a new proposed set of favorable donor criteria following death by drowning. This article is protected by copyright. All rights reserved.
View details for DOI 10.1111/ctr.12822
View details for PubMedID 27447443
Invasive mold infections in lung and heart-lung transplant recipients: Stanford University experience
TRANSPLANT INFECTIOUS DISEASE
2015; 17 (2): 259-266
Recipients of lung transplantation (LT) and heart-lung transplantation (HLT) are at increased risk of infection, including invasive mold infections (IMIs). The clinical presentation, radiographic correlates, and outcomes of Aspergillus and non-Aspergillus IMIs in this population have not been well documented.LT and HLT recipients diagnosed with IMIs between 1990 and 2012 were identified using the Stanford Translational Research Integrated Database Environment and Stanford LT and HLT clinical database. Recipient clinical and radiographic characteristics were obtained via retrospective review of medical records and compared between Aspergillus and non-Aspergillus mold recipients. Risk factors for mortality were identified using multivariate logistic regression analysis.During the study period, 87 (14%) transplant recipients were diagnosed with IMIs. Aspergillus species were isolated in 63 (72%) and non-Aspergillus molds in 24 (28%) recipients. No significant difference was seen in presenting symptoms or radiographic findings between Aspergillus and non-Aspergillus mold recipients. Median time to diagnosis was 363 days in the Aspergillus group and 419 days in the non-Aspergillus group, with dissemination occurring only within the non-Aspergillus group (12.5%). Overall 90-day and 1-year mortality following IMI was 24% and 44%. One-year mortality was increased in the non-Aspergillus group (39.5% vs. 60.5%, P = 0.03).There is significant overlap in risk factors, presentation, and radiographic patterns in IMI in LT or HLT recipients. Non-Aspergillus molds were more likely to present late, with disseminated disease, and portend increased 1-year mortality. This article is protected by copyright. All rights reserved.
View details for DOI 10.1111/tid.12362
View details for Web of Science ID 000352219400011
View details for PubMedID 25648194
Understanding the lung allocation score for the non-transplant pulmonologist
Current Pulmonology Reports
View details for DOI 10.1007/s13665-015-0113-9
Predicting Survival Across Chronic Interstitial Lung Disease The ILD-GAP Model
2014; 145 (4): 723-728
Risk prediction is challenging in chronic interstitial lung disease (ILD) because of heterogeneity in disease-specific and patient-specific variables. Our objective was to determine whether mortality is accurately predicted in patients with chronic ILD using the GAP model, a clinical prediction model based on sex, age, and lung physiology, that was previously validated in patients with idiopathic pulmonary fibrosis.Patients with idiopathic pulmonary fibrosis (n=307), chronic hypersensitivity pneumonitis (n=206), connective tissue disease-associated ILD (n=281), idiopathic nonspecific interstitial pneumonia (n=45), or unclassifiable ILD (n=173) were selected from an ongoing database (N=1,012). Performance of the previously validated GAP model was compared with novel prediction models in each ILD subtype and the combined cohort. Patients with follow-up pulmonary function data were used for longitudinal model validation.The GAP model had good performance in all ILD subtypes (c-index, 74.6 in the combined cohort), which was maintained at all stages of disease severity and during follow-up evaluation. The GAP model had similar performance compared with alternative prediction models. A modified ILD-GAP Index was developed for application across all ILD subtypes to provide disease-specific survival estimates using a single risk prediction model. This was done by adding a disease subtype variable that accounted for better adjusted survival in connective tissue disease-associated ILD, chronic hypersensitivity pneumonitis, and idiopathic nonspecific interstitial pneumonia.The GAP model accurately predicts risk of death in chronic ILD. The ILD-GAP model accurately predicts mortality in major chronic ILD subtypes and at all stages of disease.
View details for DOI 10.1378/chest.13-1474
View details for Web of Science ID 000334097700014
View details for PubMedID 24114524
- Surgical lung biopsy over bronchoalveolar lavage in chronic hypersensitivity pneumonitis. American journal of respiratory and critical care medicine 2014; 189 (3): 371-372
Lung Transplantation for Hypersensitivity Pneumonitis.
Background:Hypersensitivity pneumonitis (HP) is an inhaled antigen-mediated interstitial lung disease (ILD). Advanced disease may lead to lung transplantation. There are no published studies addressing lung transplant outcomes in HP. We characterized HP outcomes compared to referents undergoing lung transplantation for idiopathic pulmonary fibrosis (IPF). Methods:To identify HP cases, we reviewed records for all ILD lung transplantation cases at our institution from 2000-2013. We compared clinical characteristics, survival, and acute and chronic rejection for lung transplant recipients with HP to IPF referents. We also reviewed diagnoses of HP discovered only by explant pathology and looked for evidence of recurrent HP after transplant. Survival was compared using Kaplan-Meier methods and Cox proportional hazard modeling. Results:We analyzed 31 subjects with HP and 91 with IPF among 183 cases undergoing lung transplantation for ILD. Survival at 1, 3, and 5 years after lung transplant in HP compared to IPF was 96%, 89% and 89% vs. 86%, 67%, and 49%, respectively. HP subjects manifested a reduced adjusted risk of death compared to IPF subjects (HR 0.25, 95% CI 0.08-0.74; p=0.013). Of the 31 cases, the diagnosis of HP was unexpectedly made at explant in 5 (16%). Two subjects developed recurrent HP in their allografts. Conclusions:Overall, subjects with HP have excellent medium-term survival after lung transplantation and, relative to IPF, a reduced risk of death. HP may be initially discovered only by review of the explant pathology. Notably, HP may recur in the allograft.Hypersensitivity pneumonitis (HP) is an inhaled antigen-mediated interstitial lung disease (ILD). Advanced disease may lead to lung transplantation. There are no published studies addressing lung transplant outcomes in HP. We characterized HP outcomes compared to referents undergoing lung transplantation for idiopathic pulmonary fibrosis (IPF).To identify HP cases, we reviewed records for all ILD lung transplantation cases at our institution from 2000-2013. We compared clinical characteristics, survival, and acute and chronic rejection for lung transplant recipients with HP to IPF referents. We also reviewed diagnoses of HP discovered only by explant pathology and looked for evidence of recurrent HP after transplant. Survival was compared using Kaplan-Meier methods and Cox proportional hazard modeling.We analyzed 31 subjects with HP and 91 with IPF among 183 cases undergoing lung transplantation for ILD. Survival at 1, 3, and 5 years after lung transplant in HP compared to IPF was 96%, 89% and 89% vs. 86%, 67%, and 49%, respectively. HP subjects manifested a reduced adjusted risk of death compared to IPF subjects (HR 0.25, 95% CI 0.08-0.74; p=0.013). Of the 31 cases, the diagnosis of HP was unexpectedly made at explant in 5 (16%). Two subjects developed recurrent HP in their allografts.Overall, subjects with HP have excellent medium-term survival after lung transplantation and, relative to IPF, a reduced risk of death. HP may be initially discovered only by review of the explant pathology. Notably, HP may recur in the allograft.
View details for DOI 10.1378/chest.14-1543
View details for PubMedID 25412059
Prevalence and prognosis of unclassifiable interstitial lung disease
EUROPEAN RESPIRATORY JOURNAL
2013; 42 (3): 750-757
The aim of this study was to determine the prevalence, characteristics and outcomes of patients with unclassifiable interstitial lung disease (ILD) and to develop a simple method of predicting disease behaviour. Unclassifiable ILD patients were identified from an ongoing longitudinal cohort. Unclassifiable ILD was diagnosed after a multidisciplinary review did not secure a specific ILD diagnosis. Clinical characteristics and outcomes were compared with idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs. Independent predictors of mortality were determined using Cox proportional-hazards analysis to identify subgroups with distinct disease behaviour. Unclassifiable ILD was diagnosed in 10% of the ILD cohort (132 out of 1370 patients). The most common reason for being unclassifiable was missing histopathological assessment due to a high risk of surgical lung biopsy. Demographic and physiological features of unclassifiable ILD were intermediate between IPF and non-IPF disease controls. Unclassifiable ILD had longer survival rates when compared to IPF on adjusted analysis (hazard ratio 0.62, p = 0.04) and similar survival compared to non-IPF ILDs (hazard ratio 1.54, p = 0.12). Independent predictors of survival in unclassifiable ILD included diffusion capacity of the lung for carbon monoxide (p = 0.001) and a radiological fibrosis score (p = 0.02). Unclassifiable ILD represents approximately 10% of ILD cases and has a heterogeneous clinical course, which can be predicted using clinical and radiological variables.
View details for DOI 10.1183/09031936.00131912
View details for Web of Science ID 000326160500025
View details for PubMedID 23222877
Ninety-day mortality and major complications are not affected by use of lung allocation score
JOURNAL OF HEART AND LUNG TRANSPLANTATION
2008; 27 (2): 192-196
In May 2005 the Organ Procurement Transplant Network (OPTN) and United Network for Organ Sharing (UNOS) implemented the donor lung allocation score (LAS) system to prioritize organ allocation among prospective transplant recipients. The purpose of our study was to determine the impact of LAS implementation on 90-day survival, early complications and incidence of severe primary graft dysfunction (PGD) after the transplant procedure.Early outcomes among 78 patients receiving transplants after the initiation of the scoring system were compared with those of the 78 previous patients. Survival rates at 90 days and 1 year were the primary end-points of the study. Arterial blood-gas measurements were collected for all patients at the time of ICU arrival and at 12, 24 and 48 hours after surgery to determine the distribution of International Society of Heart and Lung Transplant (ISHLT) PGD grade. Major complications within 30 days post-transplant were recorded.We found a small but significant 1-year survival advantage among post-LAS implementation patients, which was largely due to decreased early mortality in comparison to the control cohort. The incidence of ISHLT Grade 3 PGD measured within the first 24 hours after transplant did not differ between groups, nor was there an increase in the rate of major post-operative complications.Implementation of the LAS system has not been associated with an increase in early mortality, immediate PGD or major complications.
View details for DOI 10.1016/j.healun.2007.11.001
View details for Web of Science ID 000253258800008
View details for PubMedID 18267226