Burden of Disease and Productivity Loss in the European Economic Area in Patients Affected by Fibrosing Interstitial Lung Disease.

INTRODUCTION: Progression of fibrosis in interstitial lung diseases (ILD) has been associated with poor prognosis, lower quality of life for patients and caregivers, and higher healthcare costs. This study estimated the burden of disease and productivity loss of progressively fibrosing ILD, focusing on progressive pulmonary fibrosis other than idiopathic pulmonary fibrosis (non-IPF PPF) and systemic sclerosis-associated ILD (SSc-ILD) in the European Economic Area (EEA). METHODS: An economic model was built to estimate the clinical burden of SSc-ILD and non-IPF PPF. The model was based on published data on disease prevalence and disease burden (in terms of comorbidities, exacerbations, and deaths) as well as on productivity loss (in terms of sick days, early retirement, permanent disability, and job loss). Aggregate income loss was obtained by multiplying productivity loss by the median daily income in each country/area of investigation. A sensitivity analysis was performed to test the impact of the variability of the model assumptions. RESULTS: In the whole EEA, a total of 86,794 and 13,221 individuals were estimated to be affected by non-IPF PPF and SSc-ILD, respectively. Estimated annual sick days associated with the diseases were 3,952,604 and 672,172, early retirements were 23,174 and 5341, permanently disabled patients were 41,748 and 4037, and job losses were 19,789 and 2617 for non-IPF PPF and SSc-ILD, respectively. Annual exacerbations were estimated to be 22,401-31,181 and 1259-1753, while deaths were 5791-6171 and 572-638 in non-IPF PPF and SSc-ILD, respectively. The estimated annual aggregate income loss in EEA, accounting for losses due to annual sick days, early retirements, and permanently disabled patients, was €1433 million and €220 million in non-IPF PPF and SSc-ILD, respectively. The productivity loss due to job losses was €194 million and €26 million in non-IPF PPF and SSc-ILD, respectively. The main driver of aggregate income loss variability was the prevalence. CONCLUSION: The impact of non-IPF PPF and SSc-ILD on society is definitely non-negligible. Actions to reduce the burden on our societies are highly needed.

Deep Learning Assessment of Progression of Emphysema and Fibrotic Interstitial Lung Abnormality.

Rationale: Although studies have evaluated emphysema and fibrotic interstitial lung abnormality individually, less is known about their combined progression. Objectives: To define clinically meaningful progression of fibrotic interstitial lung abnormality in smokers without interstitial lung disease and evaluate the effects of fibrosis and emphysema progression on mortality. Methods: Emphysema and pulmonary fibrosis were assessed on the basis of baseline and 5-year follow-up computed tomography scans of 4,450 smokers in the COPDGene Study using deep learning algorithms. Emphysema was classified as absent, trace, mild, moderate, confluent, or advanced destructive. Fibrosis was expressed as a percentage of lung volume. Emphysema progression was defined as an increase by at least one grade. A hybrid distribution and anchor-based method was used to determine the minimal clinically important difference in fibrosis. The relationship between progression and mortality was evaluated using multivariable shared frailty models using an age timescale. Measurements and Main Results: The minimal clinically important difference for fibrosis was 0.58%. On the basis of this threshold, 2,822 (63%) had progression of neither emphysema nor fibrosis, 841 (19%) had emphysema progression alone, 512 (12%) had fibrosis progression alone, and 275 (6.2%) had progression of both. Compared with nonprogressors, hazard ratios for mortality were 1.42 (95% confidence interval, 1.11-1.82) in emphysema progressors, 1.49 (1.14-1.94) in fibrosis progressors, and 2.18 (1.58-3.02) in those with progression of both emphysema and fibrosis. Conclusions: In smokers without known interstitial lung disease, small changes in fibrosis may be clinically significant, and combined progression of emphysema and fibrosis is associated with increased mortality.

Clinical assessment for pulmonary hypertension in interstitial lung disease.

BACKGROUND: Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD), as its development confers a poor prognosis. There are no specific recommendations for methods of assessment for PH in ILD populations. AIMS: To determine current assessment practices for PH in an Australian ILD centre. METHODS: In the Austin Health ILD database, 162 consecutive patients with idiopathic pulmonary fibrosis or connective tissue disease-associated ILD were identified and retrospectively evaluated for methods of PH assessment with transthoracic echocardiography (TTE), serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and right heart catheterisation (RHC) in relation to patient demographic and physiological parameters. RESULTS: The median follow-up was 30 (14.4-56.4) months. At baseline, vital capacity was 80.0 ± 18.4% predicted, and diffusing capacity for carbon monoxide was 59.6 ± 15.2% predicted. Evaluation for PH was performed in 147 (90.7%) patients, among whom 105 (64.8%) had TTE performed at least once. At the initial TTE, 33.7% patients had high probability of PH, defined as RVSP >40 mmHg + RAp and/or right ventricular dysfunction. At the time of the most recent TTE, these criteria were met in 45 (52.3%) patients. Elevated serum NT-proBNP levels during the first year were observed in 47 (38.8%) patients. Only 14 (8.6%) patients had RHC. CONCLUSION: Our institutional PH assessment practice in ILD demonstrates a substantial prevalence of probable PH at baseline. As new therapies emerge for the treatment of PH in ILD, well-defined screening practices are important in this population for early identification and optimal management.

Viewpoint: a multidisciplinary approach to the assessment of patients with systemic sclerosis-associated interstitial lung disease.

Systemic sclerosis (SSc) is a rare and heterogeneous disease affecting the skin and internal organs. SSc-associated ILD (SSc-ILD) is a common and often early manifestation of SSc. This article discusses the rationale for a multidisciplinary approach (MDA) to the early identification and assessment of patients with SSc-ILD. Diagnosis of SSc-ILD is often challenging as patients with early disease can be asymptomatic, and SSc-ILD symptoms, such as exertional dyspnea and cough, are non-specific. High-resolution computed tomography (HRCT) of the lungs is the gold standard for diagnosis of SSc-ILD since pulmonary function tests lack sensitivity and specificity, especially in early disease. On HRCT, most patients with SSc-ILD have a non-specific interstitial pneumonia (NSIP) pattern. In addition, findings of pulmonary hypertension and esophageal dysmotility may be present. The multi-organ involvement of SSc and the diverse spectrum of symptoms support an MDA for the diagnosis and assessment of patients with SSc-ILD, with input from rheumatologists, pulmonologists, gastroenterologists, radiologists, and other specialists. Key Points • Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc). • Early diagnosis is key to reducing the morbidity and mortality associated with SSc-ILD and other manifestations of SSc. • The multi-organ involvement of SSc supports a multidisciplinary approach to the diagnosis and assessment of patients with SSc-ILD, with input from rheumatologists, pulmonologists, gastroenterologists, radiologists, and other specialists.

Positron Emission Tomography to Improve Assessment of Interstitial Lung Disease in Patients With Systemic Sclerosis Eligible for Autologous Stem Cell Transplantation.

Positron emission tomography (PET) is a promising technique to improve the assessment of systemic sclerosis associated interstitial lung disease (SSc-ILD). This technique could be of particular value in patients with severe diffuse cutaneous SSc (dcSSc) that are possibly eligible for autologous hematopoietic stem cell transplantation (aHSCT). aHSCT is a potentially effective therapy for patients with severe dcSSc and ILD, leading to stabilization or improvement of lung function. However, there is a high need to improve patient selection, which includes (1) the selection of patients with rapidly progressive ILD for early rather than last-resort aHSCT (2) the prediction of treatment response on ILD and (3) the understanding of the mechanism(s) of action of aHSCT in the lungs. As previous studies with 18F-FDG PET in SSc-ILD and other forms of ILD have demonstrated its potential value in predicting disease progression and reactivity to anti-inflammatory treatment, we discuss the potential benefit of using this technique in patients with early severe dcSSc and ILD in the context of aHSCT. In addition, we discuss the potential value of other PET tracers in the assessment of ILD and understanding the mechanisms of action of aHSCT in the lung. Finally, we provide several suggestions for future research.

Direct and indirect costs of systemic sclerosis and associated interstitial lung disease: A nationwide population-based cohort study.

BACKGROUND AND OBJECTIVE: The study aimed to evaluate the direct and indirect costs of systemic sclerosis (SSc) in cases with and without interstitial lung disease (ILD). METHODS: Cases diagnosed with SSc (2002-2015) were identified in the Danish National Patient Registry. Cases were matched 1:4 with non-SSc controls from the general population. Data on costs were obtained from national databases. Excess cost was estimated as the annual cost per case subtracting the costs of the control. RESULTS: We identified 1869 cases and 7463 controls. Total excess cost (direct healthcare, elderly care and indirect costs) in the SSc-ILD cohort was €29,725, and €17,905 in the non-ILD SSc cohort. In- and out-patient contacts and forgone earnings were the key drivers of costs in both cohorts. Healthcare costs were higher before and after the diagnosis compared with the controls. Men incurred higher excess healthcare costs than women. Hospitalization and outpatient services were the key drivers of the gender-associated differences. Income from employment decreased more rapidly after diagnosis in the SSc-ILD cohort than in the non-ILD SSc cohort. Public transfer income increased after diagnosis, with the most pronounced difference in the SSc-ILD cohort. Disability pension was the key driver of public transfer income. CONCLUSION: SSc is associated with a significant individual and societal burden that is evident several years before and after the diagnosis. Total excess costs are higher in SSc-ILD than in the non-ILD SSc underlining the severity of pulmonary involvement. Initiatives to maintain work ability and to reduce hospital admissions may reduce the economic burden of SSc.

Healthcare utilization and economic burden in systemic sclerosis: a systematic review.

OBJECTIVE: Systemic sclerosis (SSc) is characterized by vasculopathy, fibrosis of skin and internal organs, and autoimmunity with complications including interstitial lung disease, pulmonary hypertension, and digital ulcers with substantial morbidity and disability. Patients with SSc may require considerable healthcare resources with economic impact. The purpose of this systematic review was to provide a narrative synthesis of the economic impact and healthcare resource utilization associated with SSc. METHODS: MEDLINE and EMBASE were searched from inception to 20 January 2021. Studies were included if they provided information regarding the total, direct and indirect cost of SSc. The cost of SSc subtypes and associated complications was determined. Risk of bias assessments through the Joanna Briggs Institute cross-sectional and case series checklists, and the Newcastle-Ottawa Cohort and Case-Control study scales were performed. A narrative synthesis of included studies was planned. RESULTS: The number of publications retrieved was 1778, of which 34 were included representing 20 cross-sectional, 11 cohort, and three case-control studies. Studies used various methods of calculating cost including prevalence-based cost-of-illness approach and health resource units cost analysis. Overall SSc total annual cost ranged from USD $14 959 to $23 268 in USA, CAD $10 673 to $18 453 in Canada, €4607 to €30 797 in Europe, and AUD $7060 to $11 607 in Oceania. Annual cost for SSc-associated interstitial lung disease and pulmonary hypertension was USD $31 285-55 446 and $44 454-63 320, respectively. CONCLUSION: Cost-calculation methodology varied greatly between included studies. SSc represents a significant patient and health resource economic burden. SSc-associated complications increase economic burden and are variable depending on geographical location and access.

Risk assessment in severe pulmonary hypertension due to interstitial lung disease.

BACKGROUND: The updated hemodynamic definition of pulmonary hypertension (PH) due to interstitial lung disease (ILD) differentiates severe and non-severe phenotypes, but no further risk stratification strategy has been established or validated for severe PH due to ILD. We aimed to assess the prognostic value of a truncated version of the European Society of Cardiology/European Respiratory Society (ESC/ERS) PH risk stratification scheme in severe PH due to ILD. METHODS: We retrospectively analyzed 185 patients with severe PH (mean pulmonary artery pressure of ≥35 mm Hg or ≥25 mm Hg with cardiac index <2.0 liter/min/m2) due to ILD who were enrolled in the Giessen PH Registry after being referred for invasive diagnostic work-up of suspected PH during 1995‒2018. A truncated ESC/ERS risk stratification scheme (based on 8 parameters from the full scheme) was applied. Kaplan-Meier and univariate Cox regression analyses were used to evaluate transplant-free survival and hazard ratios, respectively. RESULTS: During follow-up (median [interquartile range]: 19 [7-40] months), 146 events occurred. Using baseline data for risk stratification, 5-year transplant-free survival of low-, intermediate-, and high-risk groups was 43%, 15%, and 4%, respectively (log-rank p = 0.010; hazard ratio of high- vs low-risk group: 3.116 [95% CI: 1.428-6.800]). Using follow-up data (at 11 [6.0-32.5] months) for risk stratification, 5-year survival of low-, intermediate-, and high-risk groups was 22%, 3%, and 0%, respectively (log-rank p = 0.005). CONCLUSIONS: The truncated ESC/ERS scheme was clinically useful and demonstrated prognostic relevance in severe PH due to ILD.

Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis.

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a group of systemic vasculitides that predominantly affect small vessels, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pulmonary involvement is frequently observed in AAV patients, with various possible phenotypes in the different diseases. In the last years, among the possible types of lung involvement, a growing interest has been addressed to the interstitial lung disease (ILD). Prevalence of ILD is higher in MPA than in GPA; in fact, ILD has been reported in up to 45% of MPA patients and in 23% of GPA. Anti-MPO antibodies are the main ANCA subtype associated to ILD, in about 46-71% of cases, while anti-PR3 antibodies are reported in 0-29% of patients. High resolution computed tomography (HRCT) frequently detects interstitial lung abnormalities in AAV, up to 66% of patients with MPA, even if with an unclear clinical relevance, specifically in asymptomatic patients. Ground glass opacities, mainly consistent with diffuse alveolar hemorrhage (DAH), are the most frequent finding in MPA patients, but reticulations, interlobular septal thickening and honeycombing are also reported. ILD significantly affects quality of life and survival, with mortality increased 2 to 4 times, particularly higher in MPA patients with pulmonary fibrosis. Currently, immunosuppressive therapy is considered also as a possible treatment of ILD. However, a careful evaluation of progression and severity of lung involvement, should guide the treatment decision in the single patient. In this review, we discuss the available evidence on clinical features, diagnostic work-up, prognosis and management of AAV-ILD.

Quantitative computed tomography assessment for systemic sclerosis-related interstitial lung disease: comparison of different methods.

OBJECTIVES: To compare the previously defined six different histogram-based quantitative lung assessment (QLA) methods on high-resolution CT (HRCT) in patients with systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS: The HRCT images of SSc patients with ILD were reviewed, and the visual ILD score (semiquantitative) and the severity of ILD (limited or extensive) were calculated. The QLA score of ILD was evaluated using the previously defined six different methods and parameters (different lung attenuation ranges, skewness, kurtosis, mean lung attenuation, and standard deviation [SD]). Pulmonary function tests (PFTs) were also performed on all patients. Relationships among variables were evaluated using Spearman's correlation coefficient (r). Diagnostic performance of quantitative methods for the ability to differentiate the limited from extensive ILD was calculated using ROC analysis. RESULTS: Fifty-five patients were included in the study. There was a significant correlation between all quantitative and semiquantitative measurement results (p < 0.0001). The QLA scores revealed a significant correlation with PFT results. The kurtosis value of the voxels between - 200 and - 1024 Hounsfield unit (HU) (Method-5) showed the best correlation with semiquantitative evaluation (r = - 0.740, p < 0.0001). The ROC analysis demonstrated the best performance of SD of the voxels between - 400 and - 950 HU (Method-6) for histogram analysis method and Method-3 (voxels between - 260 and - 600 HU were calculated as ILD) for CT density cutoff methods. CONCLUSIONS: All the QLA methods are applicable in assessing the ILD score in SSc patients and have potential importance to differentiate limited from extensive ILD. KEY POINTS: • Quantitative interstitial lung disease assessment helps clinicians to assess systemic sclerosis patients with interstitial lung disease. • Quantitative lung assessment methods are applicable in assessing the interstitial lung disease score in systemic sclerosis patients. • Quantitative lung assessment methods have potential importance in the management of patients.

Lung cancer in rheumatoid arthritis. Is there a need for better risk assessment and screening?

Extra-articular manifestations are common in rheumatoid arthritis (RA), with lung involvement being one of the commonest. Apart from interstitial lung disease which is a well-recognized manifestation, it seems that lung cancer has also increased frequency in RA. In fact, recent meta-analyses have suggested that in RA compared with the general population, lymphomas and lung malignancy are more frequent. For the latter, male gender, seropositivity for rheumatoid factor and/or anti-citrullinated protein antibody (ACPA), as well as older age, has been suggested, among others, as risk factors. Several hypotheses have been formulated to explain the increased frequency of lung cancer in RA. These include smoking and/or interstitial lung as common risk factors for both RA and lung cancer and chronic inflammation predisposing to malignant diseases. Numerous questions remain to be answered. For example, are there any risk factors (e.g., positivity for rheumatoid factor or anti-citrullinated peptide antibodies) that would predict the development of lung cancer in these patients? Are there any screening procedures appropriate for early diagnosis and therefore better outcome? Data from large registries are needed to better define the profile of these patients.

Quantitative assessment of interstitial lung disease in Sjögren's syndrome.

BACKGROUND: Interstitial lung disease (ILD) is a frequent manifestation of Sjögren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard for diagnosis. Semiquantitative CT (SQCT) based on visual assessment (Goh and Taouli scoring) can estimate ILD extent, although it is burdened by relevant intra- and interobserver variability. Quantitative chest CT (QCT) is a promising alternative modality to assess ILD severity. AIM: To determine whether QCT assessment can identify extensive or limited lung disease in patients with SS and ILD. METHODS: This multi-center, cross-sectional and retrospective study enrolled patients with SS and a chest CT scan. SQCT assessment was carried out in a blinded and centralized manner to calculate both Goh and Taouli scores. An operator-independent analysis of all CT scans with the open-source software platform Horos was used to evaluate the QCT indices. Patients were classified according to the extent of ILD and differences in QCT index distribution were investigated with non-parametric tests. RESULTS: From a total of 102 consecutive patients with SS, the prevalence of ILD was 35.3% (36/102). There was a statistically significant difference in QCT index distribution between the SS with ILD and SS without ILD groups (p<0.001). Moreover, SS-ILD patients with ILD >20% (by Goh score) had a QCT index statistically different from those with limited ILD extent (p<0.001). Finally, QCT indices showed a moderate-to-good correlation with the Goh and Taouli scores (from 0.44 to 0.65; p<0.001). CONCLUSIONS: QCT indices can identify patients with SS and ILD and discriminate those with lesser or greater lung disease.

Data-Driven Identification of Adverse Event Reporting Patterns for Japan in VigiBase, the WHO Global Database of Individual Case Safety Reports.

INTRODUCTION: Adverse event reporting patterns vary between countries, reflecting differences in reporting culture, clinical practice and underlying patient populations. Japan collects about 60,000 domestic adverse event reports yearly and shares serious reports with the World Health Organization (WHO) Programme for International Drug Monitoring in VigiBase, the WHO global database of individual case safety reports. Understanding these reports in the global context can be helpful for regulators worldwide and can aid hypothesis-generation for Japanese-specific vulnerabilities to adverse drug reactions. OBJECTIVE: The objective of this study was to explore differences in the reporting of adverse events between Japan and other countries. METHODS: vigiPoint is a method for data-driven exploration in pharmacovigilance. It outlines data subsets, pinpoints key features and facilitates expert review, using odds ratios subjected to statistical shrinkage to distinguish one data subset from another. Here, we compared 260,000 Japanese reports in E2B format classified as serious and received in VigiBase between 2013 and 2018 with 2.5 million reports from the rest of the world (of which 51% are from the USA). Reporting patterns for which the 99% credibility interval of the shrunk log-odds ratios were above 0.5 or below - 0.5 were flagged as key features. The shrinkage was set to the vigiPoint default corresponding to 1% of the size of the Japanese data subset. As a sensitivity analysis, additional vigiPoint comparisons were performed between Japan and, in turn, Africa, the Americas, the Americas except the USA and Canada, Asia and Europe. RESULTS: There were higher reporting rates in Japan from physicians (83% vs. 39%) and pharmacists (17% vs. 10%). It was also more common to see reports with more than five drugs per report (22% vs. 14%) and with a single adverse event (72% vs. 45%). More than half of the Japanese reports had a vigiGrade completeness score above 0.8 compared with about one in five from the rest of the world. There were more reports than expected for patients aged 70-89 years and fewer reports for adults aged 20-59 years. Adverse events reported more often in Japan included interstitial lung disease, abnormal hepatic function, decreased platelet count, decreased neutrophil count and drug eruption. Adverse events reported less often included death, fatigue, dyspnoea, pain and headache. Drugs reported more often in Japan included prednisolone, methotrexate and peginterferon alfa-2b. Drugs reported less often included rosiglitazone and adalimumab as well as blood substitutes and perfusion solutions. The findings were generally robust to the sensitivity analysis except for the less often reported drugs, many of which were rarely reported in most countries, except in the USA. CONCLUSION: Analysis of Japanese adverse event reporting patterns in a global context has revealed key features that may reflect possible pharmaco-ethnic vulnerabilities in the Japanese, as well as differences in adverse event reporting and clinical practice. This knowledge is essential in the global collaboration of signal detection afforded by the WHO Programme for International Drug Monitoring.

Performance of the COPD Assessment Test in patients with connective tissue disease-associated interstitial lung disease.

BACKGROUND: Patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) often experience impaired health status. In daily clinical practice, a short and easy instrument for assessing health status would be useful to help better understand the patient's condition. The COPD Assessment Test (CAT) is a simple questionnaire about respiratory symptoms and their impact. We aimed to examine the CAT's performance characteristics and to generate data to support its reliability and validity in patients with CTD-ILD. METHODS: We used data from 132 CTD-ILD patients evaluated at Tosei General Hospital from July 2011 to July 2016 to assess the cross-sectional and longitudinal validity of the CAT. RESULTS: The mean age of the patients was 64.5 years and 87 (66%) were women. There were no significant differences in CAT score between any of the CTD subgroups. Internal consistency (Cronbach's α = 0.881) and repeatability (intraclass correlation coefficient [ICC] = 0.803) for the CAT score were acceptable. At baseline, CAT score was significantly associated with clinically meaningful measures of physiologic function, exercise capacity, and dyspnea. Change in CAT score over 6-12 months was also associated with change in other measures. In the distribution- and anchor-based analyses, the estimated minimal clinically important difference of CAT score was 1-4 points. CONCLUSION: These data support the validity and reliability of CAT as a sensitive measure for assessing health status in patients with CTD-ILD.

Computed Tomography Honeycombing Identifies a Progressive Fibrotic Phenotype with Increased Mortality across Diverse Interstitial Lung Diseases.

Rationale: Honeycombing on chest computed tomography (CT) has been described in diverse forms of interstitial lung disease (ILD); however, its prevalence and association with mortality across the spectrum of ILD remains unclear. Objective: To determine the prevalence and prognostic value of CT honeycombing and characterize associated mortality patterns across diverse ILD subtypes in a multicenter cohort. Methods: This was an observational cohort study of adult participants with multidisciplinary or adjudicated ILD diagnosis and documentation of chest CT imaging at index diagnosis across five U.S. hospitals (one tertiary and four nontertiary medical centers). Participants were stratified based on presence or absence of CT honeycombing. Vital status was determined from review of medical records and social security death index. Transplant-free survival was analyzed using univariate and multivariable Cox regression. Results: The sample comprised 1,330 participants (mean age, 66.8 yr; 50% men) with 4,831 person-years of follow-up. The prevalences of CT honeycombing were 42.0%, 41.9%, 37.6%, and 28.6% in chronic hypersensitivity pneumonitis, connective tissue disease-related ILD (CTD-ILD), idiopathic pulmonary fibrosis (IPF), and unclassifiable/other ILDs, respectively. Among those with CT honeycombing, cumulative mortality hazards were similar across ILD subtypes, except for CTD-ILD, which had a lower mortality hazard. Overall, the mean survival time was shorter among those with CT honeycombing (107 mo; 95% confidence interval [CI], 92-122 mo) than those without CT honeycombing (161 mo; 95% CI, 147-174 mo). CT honeycombing was associated with an increased mortality rate (hazard ratio, 1.72; 95% CI, 1.38-2.14) even after adjustment for center, sex, age, forced vital capacity, diffusing capacity, ILD subtype, and use of immunosuppressive therapy (hazard ratio, 1.62; 95% CI, 1.29-2.02). CT honeycombing was associated with an increased mortality rate within non-IPF ILD subgroups (chronic hypersensitivity pneumonitis, CTD-ILD, and unclassifiable/other ILD). In IPF, however, mortality rates were similar between those with and without CT honeycombing. Conclusions: CT honeycombing is prevalent in diverse forms of ILD and uniquely identifies a progressive fibrotic ILD phenotype with a high mortality rate similar to IPF. CT honeycombing did not confer additional risk in IPF, which is already known to be a progressive fibrotic ILD phenotype regardless of the presence of CT honeycombing.

Are idiopathic pulmonary fibrosis patients more anxious and depressive than patient's with other interstitial lung disease?

BACKGROUND AND AIM: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease (ILD) with unknown etiology that occurs primarily in older adults with a median survival time of 2.5±3.5 years. Since there is no curative treatment for IPF, patients with IPF may have symptoms of depression and anxiety more than those of other interstitial lung diseases. There is a few studies about comparison of anxiety depression with other interstitial lung disease and IPF. In this study, we investigated whether anxiety depression in IPF was more frequent than other ILDs and its effect on quality of life. METHODS: The study was designed as a prospective study. Age, sex, smoking status, respiratory symptoms, comorbidities, pulmonary function tests, diffusion capacity of the lungs for carbon monoxide (TLCO), SF-36, and depression/anxiety levels, radiological findings, erythrocyte sedimentation rate (ESR), CRP level, blood gas analysis, complete blood count parameters were recorded. RESULTS: The mean age of 50 IPF and 42 non-IPF interstitial lung disease patients were 67.4±7.1 and 64.9±7.2, respectively. Compared with the non-IPF group, SF-36 total, SF-36 physical function and SF-36 physical role severity were significantly lower in the IPF group, while the GAP score was significantly higher. There was no significant difference between the two groups in HAM-Anxiety and HAM-depression for total scores. But mild anxiety was present in most of non-IPF group. No severe anxiety was observed in this group. Forty-nine of 50 patients with IPF patients had moderate-severe anxiety and the difference was statistically significant compared to non-IPF patients. CONCLUSIONS: This is one of the first studies of anxiety and depression symptoms are also important in non IPF ILD like IPF. Patients with non-IPF ILD have similar anxiety depression with IPF patients in this study. This study led to the conclusion that anxiety depression should also be evaluated in non-IPF ILD patients.

Stereotactic ablative radiation therapy in lung cancer: an emerging standard.

PURPOSE OF REVIEW: Significant advances have been made in the field of stereotactic ablative radiotherapy (SABR) for the treatment of pulmonary neoplasms in recent years. This review aims to summarize recent salient evidence on SABR for early-stage nonsmall cell lung cancer (ES-NSCLC). RECENT FINDINGS: In medically inoperable patients, SABR remains the standard of care. The optimal SABR dosing regimen is being studied. Comparisons with non-SABR radiotherapy regimens with lower doses per fraction revealed benefit of SABR. In operable patients, no prospective clinical trial comparing SABR and surgery has been completed, although multiple trials are currently underway to address this question. SABR is generally cost-effective and safe in most patients, with preserved patient-reported quality of life. However, increased toxicity with SABR is noted in patients with disease close to, or invading the proximal tracheobronchial tree. Significant SABR-related toxicity and mortality is also reported in patients with coexisting interstitial lung disease. Considerations on pathologic confirmation, surveillance and multiple primaries are also addressed. SUMMARY: SABR is an effective and safe treatment for inoperable ES-NSCLC. Ongoing trials and comparative effectiveness research will help to clarify SABR's role in various lung cancer indications going forward.

Multiparametric Magnetic Resonance Imaging in the Assessment of Pulmonary Hypertension: Initial Experience of a One-Stop Study.

INTRODUCTION: Our goal was to assess the diagnostic performance of magnetic resonance imaging (MRI) as a single method to diagnose pulmonary hypertension (PH) compared to right heart catheterization (RHC), computed tomography (CT), and ventilation/perfusion (V/Q) scintigraphy. METHODS: We identified 35 patients diagnosed with PH by RHC in our institution who have also undergone a CT, a scintigraphy, and an MRI within a month. All cases were discussed in multidisciplinary meetings. We performed correlations between the MRI-derived hemodynamic parameters and those from RHC. The sensitivity and specificity of MRI were determined to identify its diagnostic performance to identify chronic thromboembolic pulmonary hypertension (CTEPH) and interstitial lung disease PH. The gold standard reference for the diagnosis of CTEPH and ILD was based on a review of multimodality imaging (V/Q scintigraphy and CT scan) and clinical findings. RESULTS: Our results showed a good correlation between the hemodynamic parameters of cardiac MRI and RHC. Pulmonary vascular resistance had the best correlation between both methods (r = 0.923). The sensitivity and specificity of MRI to diagnose CTEPH was 100 and 96.8%, respectively. For the ILD-related PH, the MRI yielded a sensitivity of 60.0% and a specificity of 100%. Additionally, cardiac MRI was able to confirm all cases of PAH due to congenital heart disease initially detected by echocardiography. CONCLUSIONS: MRI represents a promising imaging modality as an initial, single-shot study, for patients with suspected PH with the advantages of being non-invasive and having no radiation exposure.

Psychometric validation of the needs assessment tool: progressive disease in interstitial lung disease.

The inter-rater/test-retest reliability and construct validity of a palliative care needs assessment tool in interstitial lung disease (NAT:PD-ILD) were tested using NAT:PD-ILD-guided video-recorded consultations, and NAT:PD-ILD-guided consultations, and patient and carer-report outcomes (St George's Respiratory Questionnaire (SGRQ)-ILD, Carer Strain Index (CSI)/Carer Support Needs Assessment Tool (CSNAT)). 11/16 items reached at least fair inter-rater agreement; 5 items reached at least moderate test-retest agreement. 4/6 patient constructs demonstrated agreement with SGRQ-I scores (Kendall's tau-b, 0.24-20.36; P<0.05). 4/7 carer constructs agreed with the CSI/CSNAT items (kappa, 0.23-20.53). The NAT:PD-ILD is reliable and valid. Clinical effectiveness and implementation are to be evaluated.