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1.
Chest ; 2024 Jan 04.
Article in English | MEDLINE | ID: mdl-38184168

ABSTRACT

BACKGROUND: Cognitive load theory asserts that learning and performance degrade when cognitive load exceeds working memory capacity. This is particularly relevant in the learning environment of ICU rounds, when multidisciplinary providers integrate complex decision-making and teaching in a noisy, high-stress environment prone to cognitive distractions. RESEARCH QUESTION: What features of ICU rounds correlate with high provider cognitive load? STUDY DESIGN AND METHODS: This was an observational, multisite study of multidisciplinary providers during ICU rounds. Investigators recorded rounding characteristics and hourly extraneous cognitive load events during rounds (defined as distractions, episodes of split-attention or repetition, and deviations from standard communication format). After rounds, investigators measured each provider's cognitive load using the provider task load (PTL), an instrument derived from the National Aeronautics and Space Administration Task Load Index survey that assesses perceived workload associated with complex tasks. Relationships between rounding characteristics, extraneous load, and PTL score were evaluated using mixed-effects modeling. RESULTS: A total of 76 providers were observed during 32 rounds from December 2020 to May 2021. The mean rounding census ± SD was 12.5 ± 2.9 patients. The mean rounding time ± SD was 2 h 17 min ± 49 min. The mean extraneous load ± SD was 20.5 ± 4.5 events per hour, or one event every 2 min 51 s. This included 8.6 ± 3.4 distractions, 8.2 ± 4.2 communication deviations, 1.9 ± 1.4 repetitions, and 1.8 ± 1.3 episodes of split-attention per hour. Controlling for covariates, the hourly extraneous load events, number of new patients, and number of higher acuity patients were each associated with increased PTL score (slope, 2.40; 95% CI, 0.76-4.04; slope, 5.23; 95% CI, 2.02-8.43; slope, 3.35; 95% CI, 1.34-5.35, respectively). INTERPRETATION: Increased extraneous load, new patients, and patient acuity were associated with higher cognitive load during ICU rounds. These results can help direct how the ICU rounding structure may be modified to reduce workload and optimize provider learning and performance.

2.
Chest ; 165(3): 645-652, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37852435

ABSTRACT

BACKGROUND: Massive hemoptysis is a rare, high-acuity presentation, which requires the integration of both cognitive and procedural skills. Simulation has been recommended to improve preparation for high-acuity, low-occurrence procedures; however, the effect of a simulation curriculum for massive hemoptysis management has never been investigated. RESEARCH QUESTION: Does simulation for hemoptysis management improve competence? STUDY DESIGN AND METHODS: Kern's six steps for medical education curriculum design were used iteratively to develop a simulation curriculum for the management of massive hemoptysis. Pulmonary and critical care medicine fellows from the University of Colorado participated in a local needs assessment and a massive hemoptysis simulation curriculum. Using a manikin-based massive hemoptysis simulator developed for this curriculum, the simulation session used repetitive practice, clinical variation, a range of difficulties, and directed feedback in a group practice setting. Time to management and performance were assessed for each management attempt; competence was assessed using a combined metric of management-related priorities and global entrustment. RESULTS: During the needs assessment, fellows viewed massive hemoptysis management skills as important, while expressing their current confidence as low. Nineteen fellows participated in a 90-min case-based hemoptysis simulation during which each was exposed to five different cases and acted as the primary manager for two cases. There was significant improvement in performance from the first to final simulation attempts measured by time to successful management (14.24 vs 10.26 min, P = .0067) and entrustment (Global Assessment Scale, 1 [should not perform] to 5 [independent]; 4.11 vs 4.61; P = .015). Fellow self-assessed knowledge and confidence in hemoptysis management and endobronchial blocker placement improved significantly after the simulation. INTERPRETATION: Hemoptysis simulation experience improves fellow confidence and skill for management of this high-acuity, low-occurrence presentation.


Subject(s)
Education, Medical , Simulation Training , Humans , Hemoptysis/diagnosis , Hemoptysis/etiology , Hemoptysis/therapy , Clinical Competence , Curriculum , Simulation Training/methods
3.
Chest ; 165(3): 636-644, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37852436

ABSTRACT

BACKGROUND: Simulation for the management of massive hemoptysis is limited by the absence of a commercially available simulator to practice procedural skills necessary for management. RESEARCH QUESTION: Is it feasible to create and validate a hemoptysis simulator with high functional task alignment? STUDY DESIGN AND METHODS: Pulmonary and critical care medicine (PCCM) attending physicians from four academic institutions in the Denver, Colorado, area and internal medicine residents from the University of Colorado participated in this mixed-methods study. A hemoptysis simulator was constructed by connecting a 3-D-printed airway model to a manikin that may be intubated. Attending PCCM physicians evaluated the simulator through surveys and qualitative interviews. Attendings were surveyed to determine simulation content and appropriate assessment criteria for a hemoptysis simulation. Based on these criteria, expert and novice performance on the simulator was assessed. RESULTS: The manikin-based hemoptysis simulator demonstrated adequate physical resemblance, high functional alignment, and strong affective fidelity. It was universally preferred over a virtual reality simulator by 10 PCCM attendings. Twenty-seven attendings provided input on assessment criteria and established that assessing management priorities (eg, airway protection) was preferred to a skills checklist for hemoptysis management. Three experts outperformed six novices in hemoptysis management on the manikin-based simulator in all management categories assessed, supporting construct validity of the simulation. INTERPRETATION: Creation of a hemoptysis simulator with appropriate content, high functional task alignment, and strong affective fidelity was successful using 3-D-printed airway models and existing manikins. This approach can overcome barriers of cost and availability for simulation of high-acuity, low-occurrence procedures.


Subject(s)
Hemoptysis , Physicians , Humans , Hemoptysis/diagnosis , Hemoptysis/therapy , Clinical Competence , Equipment Design , Surveys and Questionnaires , Computer Simulation
4.
ATS Sch ; 3(2): 197-203, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35924192

ABSTRACT

Massive hemoptysis is a high-risk, low-frequency clinical scenario, and teaching the management of this emergency should extend beyond reliance on clinical exposure. Massive hemoptysis requires emergent intervention to avoid asphyxiation and death. Practitioners need both cognitive and procedural skills to intervene in a high-stress situation. Cognitive aids have demonstrated benefits in other emergency settings, but no such tool exists for massive hemoptysis. Using expert recommendations, we developed the ABCDE Approach for Massive Hemoptysis, a cognitively accessible, prioritized toolbox of interventions designed to assist learners in organizing an approach to these high-risk and time-sensitive patient cases. Herein we present the elements and use of the ABCDE approach. Providing a cognitive approach to massive hemoptysis is an important first step in improving education for this potentially fatal clinical scenario.

5.
ERJ Open Res ; 7(2)2021 Apr.
Article in English | MEDLINE | ID: mdl-34109243

ABSTRACT

Hypersensitivity pneumonitis (HP) is an immunologically mediated form of lung disease resulting from inhalational exposure to any of a large variety of antigens. A subgroup of patients with HP develops pulmonary fibrosis (fibrotic HP; FHP), a significant cause of morbidity and mortality. This study will evaluate the safety and efficacy of the antifibrotic pirfenidone in treating FHP. This single-centre, randomised, double-blind, placebo-controlled trial is enrolling adults with FHP (ClinicalTrials.gov: NCT02958917). Study participants must have fibrotic abnormalities involving ≥5% of the lung parenchyma on high-resolution computed tomography scan, forced vital capacity (FVC) ≥40% and diffusing capacity of the lung for carbon monoxide ≥30% of predicted values. Study participants will be randomised in a 2:1 ratio to receive pirfenidone 2403 mg·day-1 or placebo. The primary efficacy end-point is the mean change in FVC % predicted from baseline to week 52. A number of secondary end-points have been chosen to evaluate the safety and efficacy in different domains.

6.
Clin Med Insights Circ Respir Pulm Med ; 15: 11795484211006050, 2021.
Article in English | MEDLINE | ID: mdl-33854398

ABSTRACT

PURPOSE: Nintedanib is an approved treatment for idiopathic pulmonary fibrosis (IPF), which slows disease progression. Management of patients with IPF receiving nintedanib can be complicated by tolerability issues, comorbidities, and concomitant medications. We developed consensus recommendations on the management of dosing, adverse events and comorbidities in patients with IPF treated with nintedanib. METHODS: A modified Delphi process using 3 questionnaires was used to survey 14 pulmonologists experienced in using nintedanib. Panelists rated their agreement with statements on a Likert scale from -5 (strongly disagree) to +5 (strongly agree). Consensus was predefined as a mean score of ⩽-2.5 or ⩾+2.5 with a standard deviation not crossing zero. RESULTS: The panelists' recommendations were largely aligned with clinical trial data, real-world evidence, and the prescribing information, and provided additional guidance regarding minimizing gastrointestinal effects, periodic monitoring for liver dysfunction, caution with respect to concomitant administration of cytochrome P450 3A4 and P-glycoprotein 1 inhibitors and inducers and anticoagulants, and management of comorbidities. The panelists unanimously agreed that adverse event management should be individualized, based on careful consideration of the risks and benefits of each possible intervention and discussion with the patient. CONCLUSIONS: These consensus recommendations provide additional guidance on the appropriate management of IPF with nintedanib, for use alongside evidence-based literature and the prescribing information.

7.
ERJ Open Res ; 7(1)2021 Jan.
Article in English | MEDLINE | ID: mdl-33644223

ABSTRACT

Age of ILD onset is similar in patients with RA-UIP and RA-NSIP but duration of RA before ILD onset differs https://bit.ly/3lgjfDJ.

8.
Ann Am Thorac Soc ; 17(6): 699-705, 2020 06.
Article in English | MEDLINE | ID: mdl-32040340

ABSTRACT

Rationale: Progression of idiopathic pulmonary fibrosis (IPF) is accompanied by worsening of symptoms, exercise capacity, and health-related quality of life. However, the utility of patient-reported outcomes as predictors of mortality remains uncertain.Objectives: To assess whether patient-reported outcomes are independently associated with mortality beyond clinical risk factors in patients with IPF.Methods: Data from the observational IPF Prospective Outcomes Registry were used to examine associations between patient-reported outcomes at enrollment and the composite outcome of death or lung transplant in the following year. Associations were examined using univariable models and models adjusted for age and clinical variables that have been associated with death or lung transplant in patients with IPF in this cohort (oxygen use, forced vital capacity % predicted, and diffusing capacity of the lungs for carbon monoxide % predicted at enrollment).Results: Among 662 patients, 45 died and 12 underwent lung transplant over 1 year. In the model adjusted for age and clinical variables that were associated with death or lung transplant, worse scores on the St. George's Respiratory Questionnaire (SGRQ) total score (hazard ratio [HR], 1.22 [95% confidence interval (CI), 1.01-1.48] per 10-point increase), SGRQ activity score (HR, 1.25 [95% CI, 1.02-1.54] per 10-point increase) and SGRQ symptoms score (HR, 1.17 [95% CI, 1.01-1.36] per 10-point increase) were associated with death or lung transplant over 1 year.Conclusions: Patient-reported outcomes that assess symptoms and physical activity are independently associated with mortality in patients with IPF.


Subject(s)
Idiopathic Pulmonary Fibrosis/mortality , Idiopathic Pulmonary Fibrosis/surgery , Lung Transplantation/mortality , Patient Reported Outcome Measures , Aged , Disease Progression , Exercise , Female , Humans , Idiopathic Pulmonary Fibrosis/diagnosis , Lung Transplantation/trends , Male , Proportional Hazards Models , Prospective Studies , Registries , Surveys and Questionnaires , United States/epidemiology
9.
ATS Sch ; 1(4): 384-394, 2020 Aug 13.
Article in English | MEDLINE | ID: mdl-33870309

ABSTRACT

Background: Whether graduating pulmonary and critical care medicine (PCCM) fellows feel adequately trained in interstitial lung disease (ILD) remains unknown. In addition, there are no published data describing the current approach to educating trainees about ILD. Objective: To characterize the present state of ILD training during fellowship and to determine graduating PCCM fellows' perceived abilities to diagnose and manage ILD. Methods: We surveyed PCCM fellowship program directors nationwide and compared their perceptions of graduating fellows' abilities to diagnose, provide initial management to, and offer longitudinal care to patients with ILD using a series of unpaired t tests. We also inquired about existing practices for educating fellows about ILD. We then surveyed graduating PCCM fellows from 19 different preselected programs to assess comfort level with ILD in comparison with other core clinical domains. Results: Program director respondents (n = 74, 40% response rate) rated graduating fellows' abilities to establish specific ILD diagnoses and to provide initial management similarly (4.3 ± 0.8 on five-point Likert scale), whereas the ability to provide longitudinal expert care was rated significantly lower (3.8 ± 0.9, P = 0.001). Most respondents (n = 52, 70.3%) reported having dedicated outpatient ILD specialists with whom fellows could rotate, but only half required this rotation. In addition, very few (n = 17, 23.0%) reported that a majority of patients with suspected or newly diagnosed ILD were scheduled in fellow clinics, many of whom received subsequent longitudinal care from dedicated ILD specialists. Among 71 third-year fellow respondents, confidence in managing ILD was rated poorly (3.2 ± 1.0 on a five-point Likert scale) in contrast to more common diseases like chronic obstructive pulmonary disease (4.4 ± 0.7, P < 0.001) and asthma (4.2 ± 0.8, P < 0.001). Conclusion: Trainee exposure to ILD in both clinical and educational settings varied across PCCM fellowships nationwide. Fellows nearing graduation were significantly less confident in their ability to manage ILD compared with other more common pulmonary diseases.

10.
ATS Sch ; 1(4): 416-435, 2020 Oct 08.
Article in English | MEDLINE | ID: mdl-33870311

ABSTRACT

The American Thoracic Society Core Curriculum updates clinicians annually in adult and pediatric pulmonary disease, medical critical care, and sleep medicine in a 3- to 4-year recurring cycle of topics. The topics of the 2020 Pulmonary Core Curriculum include pulmonary vascular disease (submassive pulmonary embolism, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension) and pulmonary infections (community-acquired pneumonia, pulmonary nontuberculous mycobacteria, opportunistic infections in immunocompromised hosts, and coronavirus disease [COVID-19]).

11.
Br J Radiol ; 92(1099): 20181003, 2019 Jul.
Article in English | MEDLINE | ID: mdl-31084494

ABSTRACT

Radiologists have a critical role in the evaluation and diagnosis of suspected idiopathic pulmonary fibrosis (IPF). Accurate pattern identification on imaging is key in the multidisciplinary diagnostic process and frequently obviates the need for a surgical lung biopsy. In this review, we describe the clinical and imaging features of IPF in the context of recently revised international guidelines; contrast findings in other diseases that may inform differential diagnosis of fibrotic lung disease; and highlight common complications associated with pulmonary fibrosis.


Subject(s)
Idiopathic Pulmonary Fibrosis/diagnostic imaging , Tomography, X-Ray Computed/methods , Diagnosis, Differential , Humans , Lung/diagnostic imaging , Positron Emission Tomography Computed Tomography , Radiologists
13.
Am J Respir Crit Care Med ; 200(2): 160-167, 2019 07 15.
Article in English | MEDLINE | ID: mdl-31034241

ABSTRACT

Over the past decade, several large registries of patients with idiopathic pulmonary fibrosis (IPF) have been established. These registries are collecting a wealth of longitudinal data on thousands of patients with this rare disease. The data collected in these registries will be complementary to data collected in clinical trials because the patient populations studied in registries have a broader spectrum of disease severity and comorbidities and can be followed for a longer period of time. Maintaining the quality and completeness of registry databases presents administrative and resourcing challenges, but it is important to ensuring the robustness of the analyses. Data from patient registries have already helped improve understanding of the clinical characteristics of patients with IPF, the impact that the disease has on their quality of life and survival, and current practices in diagnosis and management. In the future, analyses of biospecimens linked to detailed patient profiles will provide the opportunity to identify biomarkers linked to disease progression, facilitating the development of precision medicine approaches for prognosis and therapy in patients with IPF.


Subject(s)
Idiopathic Pulmonary Fibrosis , Registries , Biological Specimen Banks , Comorbidity , Disease Progression , Humans , Observational Studies as Topic , Severity of Illness Index
15.
Am J Physiol Lung Cell Mol Physiol ; 313(5): L930-L939, 2017 Nov 01.
Article in English | MEDLINE | ID: mdl-28775099

ABSTRACT

A key physiological feature of acute respiratory distress syndrome (ARDS) is inflammation. Toll-like receptor (TLR) signaling is required to combat the infection that underlies many ARDS cases but also contributes to pathological inflammation. Several TLR signaling pathway genes encoding positive effectors of inflammation also produce alternatively spliced mRNAs encoding negative regulators of inflammation. An imbalance between these isoforms could contribute to pathological inflammation and disease severity. To determine whether splicing in TLR pathways is altered in patients with ARDS, we monitored alternative splicing of MyD88 and IRAK1, two genes that function in multiple TLR pathways. The MyD88 and IRAK1 genes produce long proinflammatory mRNAs (MyD88L and IRAK1) and shorter anti-inflammatory mRNAs (MyD88S and IRAK1c). We quantified mRNA encoding inflammatory cytokines and MyD88 and IRAK1 isoforms in peripheral blood mononuclear cells (PBMCs) from 104 patients with ARDS and 30 healthy control subjects. We found that MyD88 pre-mRNA splicing is altered in patients with ARDS in a proinflammatory direction. We also observed altered MyD88 isoform levels in a second critically ill patient cohort, suggesting that these changes may not be unique to ARDS. Early in ARDS, PBMC IRAK1c levels were associated with patient survival. Despite the similarities in MyD88 and IRAK1 alternative splicing observed in previous in vitro studies, there were differences in how MyD88 and IRAK1 alternative splicing was altered in patients with ARDS. We conclude that pre-mRNA splicing of TLR signaling genes is altered in patients with ARDS, and further investigation of altered splicing may lead to novel prognostic and therapeutic approaches.


Subject(s)
Alternative Splicing/genetics , Leukocytes, Mononuclear/metabolism , RNA Splicing/genetics , RNA, Messenger/metabolism , Respiratory Distress Syndrome/metabolism , Signal Transduction , Toll-Like Receptors/metabolism , Cytokines/metabolism , Humans , Inflammation/genetics , Inflammation/metabolism , RNA Precursors/genetics , RNA, Messenger/genetics , Respiratory Distress Syndrome/genetics
16.
Respir Med ; 126: 100-104, 2017 05.
Article in English | MEDLINE | ID: mdl-28427540

ABSTRACT

PURPOSE: Interstitial lung disease is a common extra-articular manifestation of rheumatoid arthritis (RA-ILD) and is associated with significant morbidity and mortality. However, limited data exist regarding predictors of mortality. We sought to examine the prognostic value of the high-resolution computed tomography (HRCT) patterns in patients with RA-ILD. MATERIALS AND METHODS: RA-ILD patients with HRCT patterns of usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) were identified among a longitudinal cohort of individuals evaluated at National Jewish Health. A total of 158 subjects were included in the study. For each subject, the earliest available HRCT was reviewed independently by two expert thoracic radiologists blinded to clinical data. HRCT patterns were classified as demonstrating definite UIP, possible UIP, or NSIP. Kaplan-Meier curves were generated and survival was compared among the three patterns using a log rank test for trend. RESULTS: One hundred subjects (63%) had HRCT findings classified as definite UIP, 23 (15%) as possible UIP and 35 (22%) as NSIP. No difference in survival was seen between subjects with definite UIP versus those with possible UIP. The combined group of subjects with either definite- or possible UIP had significantly worse survival than those with NSIP (log-rank p = 0.03). CONCLUSIONS: In patients with RA-ILD, patients with either definite UIP or possible UIP have equally poor survival when compared to those with an NSIP pattern.


Subject(s)
Arthritis, Rheumatoid/diagnostic imaging , Idiopathic Pulmonary Fibrosis/diagnostic imaging , Lung Diseases, Interstitial/diagnostic imaging , Tomography, X-Ray Computed/methods , Aged , Arthritis, Rheumatoid/complications , Female , Humans , Idiopathic Interstitial Pneumonias/complications , Idiopathic Pulmonary Fibrosis/mortality , Idiopathic Pulmonary Fibrosis/physiopathology , Lung Diseases, Interstitial/complications , Lung Diseases, Interstitial/mortality , Lung Diseases, Interstitial/physiopathology , Male , Middle Aged , Prognosis , Pulmonary Diffusing Capacity , Rheumatoid Factor , Smoking/epidemiology , Survival Analysis , Vital Capacity
17.
Ann Am Thorac Soc ; 13(7): 1042-9, 2016 07.
Article in English | MEDLINE | ID: mdl-27064856

ABSTRACT

RATIONALE: Granulomatous-lymphocytic interstitial lung disease (GLILD) has emerged as a major cause of morbidity in patients with common variable immunodeficiency (CVID). While GLILD is among the most serious noninfectious pulmonary complications of CVID, risk factors for this condition have not been reported. OBJECTIVES: To identify clinical, physiologic, and serologic risk factors for GLILD in adults with CVID. METHODS: Of 345 consecutive adult patients with CVID, we identified 34 in the National Jewish Health research database who had a radiographic-pathologic diagnosis of GLILD evaluated between 2002 and 2014. Each case was age and sex matched to 52 CVID control subjects. We used logistic regression to determine independent predictors of GLILD. A mixed effects model was used to estimate the longitudinal change in percent predicted FVC. MEASUREMENTS AND MAIN RESULTS: The mean time from CVID diagnosis to GLILD detection was 7.8 years. Compared with matched control subjects, cases were more likely to have a history of autoimmune cytopenia, hypersplenism, polyarthritis, lower marginal zone and switched memory B cells, and restrictive lung function. Multivariate analysis revealed that hypersplenism (odds ratio [OR], 24; 95% confidence interval [CI], 4.5-179.1), polyarthritis (OR, 19; 95% CI, 2.3-206.8), and percent predicted FVC (OR, 0.93; 95% CI, 0.87-0.98) were independently associated with the development of GLILD. The rate of change of percent predicted FVC (slope, P = 0.48) did not vary significantly in patients with GLILD over a mean follow-up of 7 years after diagnosis. CONCLUSIONS: Hypersplenism and polyarthritis are strong risk factors for GLILD in patients with CVID. Percent predicted FVC remained stable over time in patients with GLILD.


Subject(s)
Common Variable Immunodeficiency/complications , Granuloma/pathology , Lung Diseases, Interstitial/diagnostic imaging , Adult , Arthritis/complications , Case-Control Studies , Databases, Factual , Female , Humans , Hypersplenism/complications , Leukopenia/complications , Logistic Models , Lung/pathology , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/pathology , Male , Middle Aged , Multivariate Analysis , Radiography , Risk Factors , United States
18.
Eur Respir J ; 47(2): 588-96, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26585429

ABSTRACT

Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a "definite" or "possible" usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.


Subject(s)
Arthritis, Rheumatoid/complications , Idiopathic Pulmonary Fibrosis/mortality , Aged , Cohort Studies , Disease Progression , Female , Forced Expiratory Volume , Humans , Idiopathic Pulmonary Fibrosis/diagnostic imaging , Idiopathic Pulmonary Fibrosis/etiology , Idiopathic Pulmonary Fibrosis/physiopathology , Kaplan-Meier Estimate , Lung Diseases, Interstitial/diagnostic imaging , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/mortality , Lung Diseases, Interstitial/physiopathology , Male , Middle Aged , Multivariate Analysis , Proportional Hazards Models , Pulmonary Diffusing Capacity , Survival Rate , Tomography, X-Ray Computed , Vital Capacity
19.
Respir Investig ; 53(3): 88-92, 2015 May.
Article in English | MEDLINE | ID: mdl-25951093

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial lung disease of unknown etiology characterized by progressive lung scarring and a median survival of 3-5 years from the time of diagnosis. The most recent consensus guidelines adopt a diagnostic process that characterizes patients as having a final diagnosis of IPF, probable IPF, or possible IPF determined from a combination of the clinical context and specific chest imaging and histologic disease patterns. Based on currently available data, the enrollment criteria for treatment trials could be expanded to include not only patients with IPF but also those with probable and possible IPF without adversely affecting trial design or outcomes.


Subject(s)
Clinical Trials as Topic , Idiopathic Pulmonary Fibrosis , Humans , Idiopathic Pulmonary Fibrosis/diagnosis , Idiopathic Pulmonary Fibrosis/pathology , Idiopathic Pulmonary Fibrosis/therapy , Medical History Taking , Radiographic Image Enhancement , Radiography, Thoracic , Tomography, X-Ray Computed
20.
Chest ; 146(3): 775-785, 2014 Sep.
Article in English | MEDLINE | ID: mdl-24700149

ABSTRACT

BACKGROUND: The usual interstitial pneumonia (UIP) pattern of lung injury may occur in the setting of connective tissue disease (CTD), but it is most commonly found in the absence of a known cause, in the clinical context of idiopathic pulmonary fibrosis (IPF). Our objective was to observe and compare longitudinal changes in pulmonary function and survival between patients with biopsy-proven UIP found in the clinical context of either CTD or IPF. METHODS: We used longitudinal data analytic models to compare groups (IPF [n = 321] and CTD-UIP [n = 56]) on % predicted FVC (FVC %) or % predicted diffusing capacity of the lung for carbon monoxide (Dlco %), and we used both unadjusted and multivariable techniques to compare survival between these groups. RESULTS: There were no significant differences between groups in longitudinal changes in FVC % or Dlco % up to diagnosis, or from diagnosis to 10 years beyond (over which time, the mean decrease in FVC % per year [95% CI] was 4.1 [3.4, 4.9] for IPF and 3.5 [1.8, 5.1] for CTD-UIP, P = .49 for difference; and the mean decrease in Dlco % per year was 4.7 [4.0, 5.3] for IPF and 4.3 [3.0, 5.6] for CTD-UIP, P = .60 for difference). Despite the lack of differences in pulmonary function, subjects with IPF had worse survival in unadjusted (log-rank P = .003) and certain multivariable analyses. CONCLUSIONS: Despite no significant differences in changes in pulmonary function over time, patients with CTD-UIP (at least those with certain classifiable CTDs) live longer than patients with IPF--an observation that we suspect is due to an increased rate of mortal acute exacerbations in patients with IPF.


Subject(s)
Idiopathic Pulmonary Fibrosis/mortality , Idiopathic Pulmonary Fibrosis/physiopathology , Lung Diseases, Interstitial/mortality , Lung Diseases, Interstitial/physiopathology , Lung/physiopathology , Aged , Biopsy , Female , Humans , Kaplan-Meier Estimate , Longitudinal Studies , Lung/pathology , Male , Middle Aged , Multivariate Analysis , Pulmonary Diffusing Capacity/physiology , Respiratory Function Tests , Survival Rate , Vital Capacity/physiology
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