Identification and Prognosis of Patients With Interstitial Pneumonia With Autoimmune Features.

BACKGROUND/OBJECTIVE: Patients classified as interstitial pneumonia with autoimmune features (IPAF) have interstitial lung disease (ILD) and features of autoimmunity but do not fulfill criteria for connective tissue diseases (CTDs). Our goal was to identify patients classifiable as IPAF, CTD-ILD, and idiopathic pulmonary fibrosis (IPF) from a preexisting pulmonary cohort and evaluate the prognosis of patients with IPAF. METHODS: We reviewed the medical records of 456 patients from a single-center pulmonary ILD cohort whose diagnoses were previously established by a multidisciplinary panel that did not include rheumatologists. We reclassified patients as IPAF, CTD-ILD, or IPF. We compared transplant-free survival using Kaplan-Meier methods and identified prognostic factors using Cox models. RESULTS: We identified 60 patients with IPAF, 113 with CTD-ILD, and 126 with IPF. Transplant-free survival of IPAF was not statistically significantly different from that of CTD-ILD or IPF. Among IPAF patients, male sex (hazard ratio, 4.58 [1.77-11.87]) was independently associated with worse transplant-free survival. During follow-up, only 10% of IPAF patients were diagnosed with CTD-ILD, most commonly antisynthetase syndrome. CONCLUSION: Despite similar clinical characteristics, most patients with IPAF did not progress to CTD-ILD; those who did often developed antisynthetase syndrome, highlighting the critical importance of comprehensive myositis autoantibody testing in this population. As in other types of ILD, male sex may portend a worse prognosis in IPAF. The routine engagement of rheumatologists in the multidisciplinary evaluation of ILD will help ensure the accurate classification of these patients and help clarify prognostic factors.

The Virtual Interview Experience: Perspectives of Pulmonary and Critical Care Fellowship Applicants.

Background: Because of the coronavirus disease (COVID-19) pandemic, graduate medical education programs adopted virtual interviews (VIs) as the default modality for the 2020 recruitment season. It is unknown whether VIs allowed applicants to effectively evaluate programs, and the best interview format for the future is unclear. Objective: To 1) assess pulmonary and critical care applicants' perceived ability to evaluate programs using VIs, 2) determine the attitudes of applicants toward the components of VIs, and 3) identify applicants' preferences for the future fellowship interview format. Methods: After the National Residency Matching Program medical subspecialty match, an electronic survey was sent to 1,067 applicants to pulmonary and critical care medicine programs asking them to compare their fellowship VI experience with their residency in-person interview (IPI) experience. Results: Three hundred six (29%) applicants responded to the survey, and 289 completed it (27%). There were 117 (40%) women and 146 (51%) White individuals. Most respondents believed that VIs hindered their ability to evaluate programs' culture, faculty-fellow relationships, location, facilities, and their own fit within the program. They believed they were able to evaluate the clinical experience, curriculum, and potential for academic development equally well compared with IPIs. The most helpful elements of VIs were the interview with the program director, meetings with the fellows, and interviews with faculty members. Less helpful elements included conference access, prerecorded program director presentations, virtual hospital and city tours, and video testimonials. One hundred twenty-three respondents (43%) chose VIs with an optional visit as their preferred future interview format, 85 (29%) chose IPIs, 54 (19%) wanted a choice between VIs and IPIs, and 27 (9%) chose VIs only. Conclusion: Most pulmonary and critical care medicine applicants preferred future interviews to include both VIs and the option of an in-person visit or interview. This study can assist programs in designing their future interview formats in a trainee-centric fashion.

A Novel Ambulatory Curriculum for Pulmonary and Critical Care Fellowship Training.

Background: Dedicated ambulatory training during pulmonary and critical care medicine (PCCM) fellowships is often limited. A novel 2-year longitudinal outpatient pulmonary fellowship curriculum was previously developed, piloted, and studied. The exportability and potential impact of this ambulatory curriculum on PCCM fellowship training nationally is not known. Objective: We aim to understand the current state of ambulatory training in PCCM fellowships and the impact of a standardized outpatient curriculum on fellows' ambulatory knowledge and competency. Methods: Nineteen programs participated in the study from 2017 to 2019. Six programs received the first year of content, seven programs received the entire 2-year curriculum, and seven programs served as a control. Fellows, faculty, and program directors (PDs) completed a series of surveys assessing satisfaction with ambulatory education and the curriculum. Fellows completed a series of medical knowledge inventories, and programs submitted in-training exam scores. Results: A total of 221 fellows (39%) and 17 PDs (89%) completed the precurriculum surveys, and 38 (12%) fellows and 10 (53%) PDs completed postcurriculum surveys. Before curriculum implementation, only 34.4% of fellows rated the quality of their ambulatory education as good or outstanding compared with 57.9% at the end of the study. Eighty-five percent of faculty and 89% of PDs rated the curriculum as good or excellent. Faculty believed that the teaching scripts were easy to use (78.4%), were factually accurate (86.3%), and provided high-yield information (82.1%). The majority of PDs indicated that the curriculum positively impacted patient care (78%) and fulfilled an unmet educational need (100%), and most planned to continue the curriculum after the study (78%). Feedback surrounded the need for updated content based on recently published guidelines and studies. Conclusion: The curriculum is a standardized and feasible way to address a previously unmet need in PCCM fellowship education. PDs rated the curriculum highly and most plan to continue it in the future. Our limited data set suggests that the curriculum was well received by fellows and faculty and positively impacted perceptions of ambulatory education and preparedness for independent practice. Future study with a larger sample of fellows is needed to better understand the generalizability of these findings.

The Role of Surgical Lung Biopsy in the Diagnosis of Fibrotic Interstitial Lung Disease: Perspective from the Pulmonary Fibrosis Foundation.

Diagnosis of interstitial lung disease (ILD) requires a multidisciplinary discussion approach that includes clinicians, radiologists, and pathologists. Surgical lung biopsy (SLB) is currently the recommended standard in obtaining pathologic specimens for patients with ILD requiring a tissue diagnosis. The increased diagnostic confidence and accuracy provided by microscopic pathology assessment of SLB specimens must be balanced with the associated risks in patients with ILD. This document was developed by the SLB Working Group of the Pulmonary Fibrosis Foundation, composed of a multidisciplinary group of ILD physicians, including pulmonologists, radiologists, pathologists, and thoracic surgeons. In this document, we present an up-to-date literature review of the indications, contraindications, risks, and alternatives to SLB in the diagnosis of fibrotic ILD; outline an integrated approach to the decision-making around SLB in the diagnosis of fibrotic ILD; and provide practical information to maximize the yield and safety of SLB.

A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex.

INRODUCTION: The mechanistic target of rapamycin inhibitors (mTORi) sirolimus and everolimus stabilize lung function in patients with pulmonary lymphangioleiomyomatosis (LAM) but do not induce remission. Pre-clinical studies suggest that simvastatin in combination with sirolimus induces LAM cell death. The objective of this study was to assess the safety of simvastatin with either sirolimus or everolimus in LAM patients. METHODS: This was a phase II single arm trial evaluating the safety of escalating daily simvastatin (20-40 mg) in LAM patients already treated with sirolimus or everolimus. Adverse events and changes in lipid panel profile, pulmonary function tests, and VEGF-D were assessed. RESULTS: Ten LAM patients on a stable dose of mTORi for >3 months were treated with 20 mg simvastatin for two months followed by 40 mg for two months. The most common adverse events were peripheral edema (30%), cough (30%), and diarrhea (30%). No patients withdrew or had a reduction in simvastatin dose because of adverse events. Two patients required sirolumus dose reduction for supratherapeutic trough levels following simvastatin initiation. Total cholesterol and low density lipoproteins declined over the study period (-46.0 mg/dL±20.8, p = 0.008; -41.9 mg/dL±22.0, p = 0.01, respectively). There was also a decline in FEV1 (-82.0 mL±86.4, p = 0.02) but no significant change in FVC, DLCO, or VEGF-D. CONCLUSIONS: The combination of simvastatin with mTORi in LAM patients is safe and well-tolerated from an adverse events perspective. The addition of simvastatin, however, was associated with decline in FEV1 and the efficacy of this combination should be explored in larger trials.

Virtually Hosting a National Medical Society Conference. Lessons Learned from the 2020 Association of Pulmonary and Critical Care Medicine Program Directors Conference.

The coronavirus pandemic forced the Association of Pulmonary and Critical Care Medicine Program Directors to change the 2020 annual conference to a virtual format with relatively short notice. Using the experience of the planning committee and survey feedback from attendees, we describe the steps taken to implement a virtual conference and lessons learned in the process. The lessons described include frequent and concise communication, establishment of roles within a discrete production team, preparing speakers with a protocolized training session, active moderation of the chat box, using interactive polling and online documents to improve interactivity, a shorter agenda with more frequent breaks, encouraging "virtual happy hours" to connect with colleagues, and establishing facilitators for breakout rooms.

Fellowship Education in Interstitial Lung Disease. A National Survey of Program Directors and Trainees.

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.

Pulmonary Involvement in Sjögren Syndrome.

Sjögren syndrome (SS) is a progressive autoimmune disease characterized by dryness, predominantly of the eyes and mouth, caused by chronic lymphocytic infiltration of the lacrimal and salivary glands. Extraglandular inflammation can lead to systemic manifestations, many of which involve the lungs. Studies in which lung involvement is defined as requiring the presence of respiratory symptoms and either radiograph or pulmonary function test abnormalities quote prevalence estimates of 9% to 22%. The most common lung diseases that occur in relation to SS are airways disease and interstitial lung disease. Evidence-based guidelines to inform treatment recommendations for lung involvement are largely lacking.

Exposure to Ambient Particulate Matter Is Associated With Accelerated Functional Decline in Idiopathic Pulmonary Fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF), a progressive disease with an unknown pathogenesis, may be due in part to an abnormal response to injurious stimuli by alveolar epithelial cells. Air pollution and particulate inhalation of matter evoke a wide variety of pulmonary and systemic inflammatory diseases. We therefore hypothesized that increased average ambient particulate matter (PM) concentrations would be associated with an accelerated rate of decline in FVC in IPF. METHODS: We identified a cohort of subjects seen at a single university referral center from 2007 to 2013. Average concentrations of particulate matter < 10 and < 2.5 µg/m3 (PM10 and PM2.5, respectively) were assigned to each patient based on geocoded residential addresses. A linear multivariable mixed-effects model determined the association between the rate of decline in FVC and average PM concentration, controlling for baseline FVC at first measurement and other covariates. RESULTS: One hundred thirty-five subjects were included in the final analysis after exclusion of subjects missing repeated spirometry measurements and those for whom exposure data were not available. There was a significant association between PM10 levels and the rate of decline in FVC during the study period, with each µg/m3 increase in PM10 corresponding with an additional 46 cc/y decline in FVC (P = .008). CONCLUSIONS: Ambient air pollution, as measured by average PM10 concentration, is associated with an increase in the rate of decline of FVC in IPF, suggesting a potential mechanistic role for air pollution in the progression of disease.

Interstitial Lung Disease in the Elderly.

BACKGROUND: Despite the relationship between idiopathic pulmonary fibrosis (IPF) and advancing age, little is known about the epidemiology of interstitial lung disease (ILD) in the elderly. We describe the diagnoses, clinical characteristics, and outcomes of patients who were elderly at the time of ILD diagnosis. METHODS: Among subjects from a prospective cohort study of ILD, elderly was defined as age ≥ 70 years. Diagnoses were derived from a multidisciplinary review. Differences between elderly and nonelderly groups were determined using the χ2 test and analysis of variance. RESULTS: Of the 327 subjects enrolled, 80 (24%) were elderly. The majority of elderly subjects were white men. The most common diagnoses were unclassifiable ILD (45%), IPF (34%), connective tissue disease (CTD)-ILD (11%), and hypersensitivity pneumonitis (8%). Most elderly subjects (74%) with unclassifiable ILD had an imaging pattern inconsistent with usual interstitial pneumonia (UIP). There were no significant differences in pulmonary function or 3-year mortality between nonelderly and elderly subjects combined or in a subgroup analysis of those with IPF. CONCLUSIONS: Although IPF was the single most common diagnosis, the majority of elderly subjects had non-IPF ILD. Our findings highlight the need for every patient with new-onset ILD, regardless of age, to be surveyed for exposures and findings of CTD. Unclassifiable ILD was common among the elderly, but for most, the radiographic pattern was inconsistent with UIP. Although the effect of ILD may be more pronounced in the elderly due to reduced global functionality, ILD was not more severe or aggressive in this group.

Determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis undergoing lung transplant evaluation.

Little is known about the physiologic determinants of 6-minute walk distance in idiopathic pulmonary fibrosis. We investigated the demographic, pulmonary function, echocardiographic, and hemodynamic determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis evaluated for lung transplantation. We performed a cross-sectional analysis of 130 patients with idiopathic pulmonary fibrosis who completed a lung transplantation evaluation at the Hospital of the University of Pennsylvania between 2005 and 2010. Multivariable linear regression analysis was used to generate an explanatory model for 6-minute walk distance. After adjustment for age, sex, race, height, and weight, the presence of right ventricular dilation was associated with a decrease of 50.9 m (95% confidence interval [CI], 8.4-93.3) in 6-minute walk distance ([Formula: see text]). For each 200-mL reduction in forced vital capacity, the walk distance decreased by 15.0 m (95% CI, 9.0-21.1; [Formula: see text]). For every increase of 1 Wood unit in pulmonary vascular resistance, the walk distance decreased by 17.3 m (95% CI, 5.1-29.5; [Formula: see text]). Six-minute walk distance in idiopathic pulmonary fibrosis depends in part on circulatory impairment and the degree of restrictive lung disease. Future trials that target right ventricular morphology, pulmonary vascular resistance, and forced vital capacity may potentially improve exercise capacity in patients with idiopathic pulmonary fibrosis.

Changing the Ambulatory Training Paradigm. Design and Implementation of an Outpatient Pulmonology Fellowship Curriculum.

RATIONALE: The Accreditation Council for Graduate Medical Education has mandated that pulmonary fellows practice evidence-based medicine "across multiple care settings." Currently, most clinical fellowship training is inpatient based, suggesting that more robust fellowship training in outpatient pulmonology is needed. No standardized ambulatory pulmonary curriculum is currently available. OBJECTIVES: To design, implement and test the feasibility of a standardized, case-based outpatient curriculum implemented for pulmonary fellows at the Perelman School of Medicine at the University of Pennsylvania. METHODS: A list of 20 topics in ambulatory pulmonology was generated and was used to create a series of literature-based teaching scripts, which served as the foundation for twice-monthly small group teaching sessions. Before implementation, the fellows were asked to complete a survey regarding their impressions of their existing outpatient training and their competency in ambulatory patient care. Participants were surveyed again at 6 and 12 months thereafter. MEASUREMENTS AND MAIN RESULTS: Fellow survey respondents reported that the curriculum improved the overall ambulatory educational experience. Before implementation, only 6 of 12 fellows (50%) agreed that their current instruction on relevant outpatient pulmonary topics was adequate, compared with 100% after the curriculum (P = 0.01, n = 10). In addition, only five fellows (42%) initially agreed or strongly agreed that their current outpatient educational experience had prepared them well for independent pulmonary practice, compared with 90% on the 12-month survey (P = 0.02). CONCLUSIONS: We created a standardized outpatient pulmonary curriculum and demonstrated its feasibility in positively influencing fellows' perceived competency in ambulatory pulmonology. Additional assessment of knowledge, performance outcomes, and applicability at other institutions is needed.

Pulmonary involvement in Sjögren syndrome.

Sjögren syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic inflammation of exocrine glands and a variety of extraglandular sites. Lung involvement as defined by symptoms and either pulmonary function testing or radiographic abnormalities occurs in approximately 10 to 20% of patients. Subclinical lung disease is even more frequent and often includes evidence of small airways disease and airway inflammation. In general, patients will have evidence of both airway and interstitial lung disease by radiographs and pathology. Bronchiolitis and bronchiectasis are the most common airway manifestations while the interstitial pathologies associated with SS include nonspecific interstitial pneumonitis, usual interstitial pneumonitis, and lymphocytic interstitial pneumonitis. Patients with SS are also at an increased risk of lymphoma. A protean of other lung abnormalities including amyloidosis, granulomatous lung disease, pseudolymphoma, pulmonary hypertension, and pleural disease have been described.

Echocardiographic and hemodynamic predictors of mortality in idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) can lead to the development of pulmonary hypertension, which is associated with an increased risk of death. In pulmonary arterial hypertension, survival is directly related to the capacity of the right ventricle to adapt to elevated pulmonary vascular load. The relative importance of right ventricular function in IPF is not well understood. Our objective was to evaluate right ventricular echocardiographic and hemodynamic predictors of mortality in a cohort of patients with IPF referred for lung transplant evaluation. METHODS: We performed a retrospective cohort study of 135 patients who met 2011 American Thoracic Society/European Respiratory Society criteria for IPF and who were evaluated for lung transplantation at the Hospital of the University of Pennsylvania. RESULTS: Right ventricle:left ventricle diameter ratio (hazard ratio [HR], 4.5; 95% CI, 1.7-11.9), moderate to severe right atrial and right ventricular dilation (HR, 2.9; 95% CI, 1.4-5.9; and HR, 2.7; 95% CI, 1.4-5.4, respectively) and right ventricular dysfunction (HR, 5.5; 95% CI, 2.6-11.5) were associated with an increased risk of death. Higher pulmonary vascular resistance was also associated with increased mortality (HR per 1 Wood unit, 1.3; 95% CI, 1.1-1.5). These risk factors were independent of age, sex, race, height, weight, FVC, and lung transplantation status. Other hemodynamic indices, such as mean pulmonary artery pressure and cardiac index, were not associated with outcome. CONCLUSIONS: Right-sided heart size and right ventricular dysfunction measured by echocardiography and higher pulmonary vascular resistance by invasive hemodynamic assessment predict mortality in patients with IPF evaluated for lung transplantation.

A comparative study of HRCT image metrics and PFT values for characterization of ILD and COPD.

RATIONALE AND OBJECTIVES: The aim of this study was to compare the performance of various image-based metrics computed from thoracic high-resolution computed tomography (HRCT) with data from pulmonary function testing (PFT) in characterizing interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD). MATERIALS AND METHODS: Fourteen patients with ILD and 11 with COPD had undergone both PFT and HRCT within 3 days. For each patient, 93 image-based metrics were computed, and their relationships with the 21 clinically used PFT parameters were analyzed using a minimal-redundancy-maximal-relevance statistical framework. The first 20 features were selected among the total of 114 mixed image metrics and PFT values in the characterization of ILD and COPD. RESULTS: Among the best-performing 20 features, 14 were image metrics, derived from attenuation histograms and texture descriptions. The highest relevance value computed from PFT parameters was 0.47, and the highest from image metrics was 0.52, given the theoretical bound as [0, 0.69]. The ILD or COPD classifier using the first four features achieved a 1.92% error rate. CONCLUSIONS: Some image metrics are not only as good discriminators as PFT for the characterization of ILD and COPD but are also not redundant when PFT values are provided. Image metrics of attenuation histogram statistics and texture descriptions may be valuable for further investigation in computer-assisted diagnosis.

Computational analysis of thoracic multidetector row HRCT for segmentation and quantification of small airway air trapping and emphysema in obstructive pulmonary disease.

RATIONALE AND OBJECTIVES: Obstructive pulmonary disease phenotypes are related to variable combinations of emphysema and small-airway disease, the latter manifested as air trapping (AT) on imaging. The investigators propose a method to extract AT information quantitatively from thoracic multi-detector row high-resolution computed tomography (HRCT), validated by pulmonary function testing (PFT) correlation. MATERIALS AND METHODS: Seventeen patients with obstructive pulmonary disease who underwent HRCT and PFT within a 3-day interval were retrospectively identified. Thin-section volumetric HRCT in inspiration and expiration was registered and analyzed using custom-made software. Nonaerated regions of lung were segmented through exclusion of voxels > -50 Hounsfield units (HU); emphysematous areas were segmented as voxels < -950 HU on inspiratory images. Small-airway AT volume (ATV) was segmented as regions of lung voxels whose attenuation values increased by less than a specified change threshold (set from 5 to 300 HU in 25-HU increments) between inspiration and expiration. Inspiratory and expiratory total segmented lung volumes, emphysema volume (EV), and ATV for each threshold were subsequently calculated and correlated with PFT parameters. RESULTS: A strong positive correlation was obtained between total segmented lung volume in inspiration and total lung capacity (r = 0.83). A strong negative correlation (r = -0.80) was obtained between EV and the ratio between forced expiratory volume in 1 second and forced vital capacity. Stronger negative correlation with forced expiratory volume in 1 second/forced vital capacity (r = -0.85) was demonstrated when ATV (threshold, 50 HU) was added to EV, indicating improved quantification of total AT to predict obstructive disease severity. A moderately strong positive correlation between ATV and residual volume was observed, with a maximum r value of 0.72 (threshold, 25 HU), greater than that between EV and residual volume (r = 0.58). The benefit of ATV quantification was greater in a subgroup of patients with negligible emphysema compared to patients with moderate to severe emphysema. CONCLUSIONS: Small-airway AT segmentation in conjunction with emphysema segmentation through computer-assisted methodologies may provide better correlations with key PFT parameters, suggesting that the quantification of emphysema-related and small airway-related components of AT from thoracic HRCT has great potential to elucidate phenotypic differences in patients with chronic obstructive pulmonary disease.

Candidate selection, timing of listing, and choice of procedure for lung transplantation.

Decisions about patient selection, timing of listing, and choice of procedure are important steps in optimizing the outcome of lung transplantation. Selection of candidates for lung transplantation requires an appreciation of the effect of pretransplant patient characteristics on posttransplant outcomes. Familiarity with the natural history of the underlying disease and of disease-specific prognostic factors is essential in making decisions about when to list candidates. Decisions about transplanting 1 or 2 lungs are principally determined by the underlying disease, but in cases in which both procedures are acceptable, factors such as survival benefit, patient's age, and center-specific preferences come into play.

Effect of treatment guidelines on the initial management of idiopathic pulmonary fibrosis.

WHAT IS ALREADY KNOWN ABOUT THIS SUBJECT: Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disease with no known aetiology and no proven treatment. Despite the absence of efficacy data, many physicians treat IPF with corticosteroids either as monotherapy or in combination with a cytotoxic agent. Specialty society guidelines published in 1999 and 2000 recognize that treatment may not be appropriate for all patients with IPF, but recommend that if treatment is to be initiated, a combination of corticosteroids with a cytotoxic agent is preferred over corticosteroids alone. It is not known how the use of corticosteroids and cytotoxic agents in the treatment of IPF has changed over time and whether published guidelines have altered prescribing practices. WHAT THIS STUDY ADDS: The results of this study demonstrate a modest but statistically significant reduction in the overall use of corticosteroids since the publication of treatment guidelines. At the same time, there was a more pronounced increase in the combined use of corticosteroids and cytotoxic agents consistent with recommendations. Even with the increase in the use of combination therapy, corticosteroid monotherapy remained the most commonly prescribed regimen among treated patients. Given the lack of established benefit and the risks associated with corticosteroid therapy, the reasons for the continued use of corticosteroid monotherapy in the majority of treated patients warrant further investigation. AIMS: To assess the impact of specialty society guidelines on the use of corticosteroids and cytotoxic agents in the initial management of patients with idiopathic pulmonary fibrosis. METHODS: A retrospective cohort study of 941 patients with an incident diagnosis of IPF was conducted using a large medical records database. The primary outcome was a new prescription for corticosteroids with or without a cytotoxic agent within 30 days of diagnosis. The primary exposure was whether diagnosis occurred before or after the publication of treatment guidelines. Logistic regression was used to control for changes in population demographics and disease characteristics across time. RESULTS: In total, 187 patients (19.9%) received a new corticosteroid prescription within 30 days of diagnosis. Fewer patients received corticosteroids after the publication of guidelines (22.2% vs. 17.7%; adjusted OR for steroid use after the publication of guidelines 0.65, 95% confidence interval 0.46, 0.92, P = 0.014). Among the 187 patients treated with corticosteroids, 22 (11.8%) also received a cytotoxic agent. The use of cytotoxic agents among users of corticosteroids increased significantly after the publication of guidelines (5.1% vs. 19.3%) with a fully adjusted OR = 4.71 (95% CI 1.56, 14.21, P = 0.006). CONCLUSIONS: Since the publication of treatment guidelines, there has been a small reduction in the overall use of corticosteroids. Consistent with these guidelines, the use of cytotoxic agents among those prescribed corticosteroids has increased significantly; however, the use of these agents remains uncommon.