Mortality In Interstitial Lung Disease Research Articles

Machine learning classifier is associated with mortality in interstitial lung disease: a retrospective validation study leveraging registry data

BackgroundMortality prediction in interstitial lung disease (ILD) poses a significant challenge to clinicians due to heterogeneity across disease subtypes. Currently, forced vital capacity (FVC) and Gender, Age, and Physiology (GAP) score are the two most utilized metrics in prognostication. Recently, a machine learning classifier system, Fibresolve, designed to identify a variety of computed tomography (CT) patterns associated with idiopathic pulmonary fibrosis (IPF), was demonstrated to have a significant association with mortality across multiple subtypes of ILD. The purpose of this follow-up study was to retrospectively validate these findings in a large, external cohort of patients with ILD.MethodsIn this multi-center validation study, Fibresolve was applied to chest CT scans of patients with confirmed ILD that had available follow-up data. Fibresolve scores categorized by tertile were analyzed using Cox regression analysis adjusted for tobacco use and modified GAP (mGAP) score.ResultsOf 643 patients included, 446 (69.3%) died over a median follow-up time of 144 [1-821] weeks. The median [range] mGAP score was 5 [3–7]. In multivariable analysis, Fibresolve score categorized by tertile was significantly associated with mortality (Tertile 2 HR 1.47, 95% CI 0.82–2.37, p = 0.11; Tertile 3 HR 3.12, 95% CI 1.98–4.90, p < 0.001). Subgroup analyses revealed significant associations amongst those with non-IPF ILDs (Tertile 2 HR 1.95, 95% CI 1.28–2.97, Tertile 3 HR 4.66, 95% CI 2.94–7.38) and severe disease, defined by a FVC ≤ 75% (Tertile 2 HR 2.29, 95% CI 1.43–3.67, Tertile 3 HR 4.80, 95% CI 2.93–7.86).ConclusionsFibresolve is independently associated with mortality in ILD, particularly amongst patients with non-IPF ILDs and in those with severe disease.

Computed tomography machine learning classifier correlates with mortality in interstitial lung disease

BackgroundA machine learning classifier system, Fibresolve, was designed and validated as an adjunct to non-invasive diagnosis in idiopathic pulmonary fibrosis (IPF). The system uses a deep learning algorithm to analyze chest computed tomography (CT) imaging. We hypothesized that Fibresolve is a useful predictor of mortality in interstitial lung diseases (ILD). MethodsFibresolve was previously validated in a multi-site >500-patient dataset. In this analysis, we assessed the usefulness of Fibresolve to predict mortality in a subset of 228 patients with IPF and other ILDs in whom follow up data was available. We applied Cox regression analysis adjusting for the Gender, Age, and Physiology (GAP) score and for other known predictors of mortality in IPF. We also analyzed the role of Fibresolve as tertiles adjusting for GAP stages. ResultsDuring a median follow-up of 2.8 years (range 5 to 3434 days), 89 patients died. After adjusting for GAP score and other mortality risk factors, the Fibresolve score significantly predicted the risk of death (HR: 7.14; 95% CI: 1.31–38.85; p = 0.02) during the follow-up period, as did forced vital capacity and history of lung cancer. After adjusting for GAP stages and other variables, Fibresolve score split into tertiles significantly predicted the risk of death (p = 0.027 for the model; HR 1.37 for 2nd tertile; 95% CI: 0.77–2.42. HR 2.19 for 3rd tertile; 95% CI: 1.22–3.93). ConclusionsThe machine learning classifier Fibresolve demonstrated to be an independent predictor of mortality in ILDs, with prognostic performance equivalent to GAP based solely on CT images.

Clinical utility of the Borg dyspnoea score in 6-minute walk tests in interstitial lung disease: A systematic review

BackgroundExertional dyspnoea, a cardinal symptom in interstitial lung disease (ILD), can be objectively measured during a 6-min walk test (6MWT) using the Borg Dyspnoea Score (BDS). However, the clinical utility of this measurement is unclear. The purpose of this systematic review was to determine the association between 6MWT BDS and prognosis (mortality and lung transplantation), other 6MWT variables and measures of pulmonary function. MethodsMEDLINE, EMBASE, Cochrane and SCOPUS databases were used to identify studies reporting an association between post-6MWT BDS and the relevant outcomes in adults with ILD. Language was limited to English. Study quality was assessed using the Quality in Prognosis Study risk of bias tool. A narrative synthesis for each outcome was performed. ResultsTen full-text studies (n = 518) were included. Four studies had high overall risk of bias. Two studies (n = 127) reported prognosis and both found that higher 6MWT BDS was associated with increased all-cause mortality. However, the certainty of evidence was very low due to study design and likely publication bias. Higher post-6MWT BDS may be associated with shorter, or no effect on 6MWD; and lower pulmonary function. There was insufficient evidence that BDS correlated with 6MWT oxygen saturation. ConclusionsPost-6MWT BDS has a potential role as a predictor of all-cause mortality in ILD, 6MWD and lower pulmonary function. Larger studies designed to confirm these relationships and assess the independent association between the 6MWT BDS and clinical outcomes are required.

Trends in Incidence, Prevalence, and Mortality of Interstitial Lung Disease in Latin America, 1990 to 2019

Trends in Incidence, Prevalence, and Mortality of Interstitial Lung Disease in Latin America, 1990 to 2019

Severe pulmonary hypertension-interstitial lung disease presenting as right ventricular failure: stabilisation with intravenous prostacyclin and maintenance with inhaled prostacyclin.

Pulmonary hypertension (PH) leads to increased morbidity and mortality in interstitial lung disease (ILD). While the INCREASE trial highlighted the use of inhaled prostacyclin in PH-ILD patients, such therapy may be inadequate when right ventricular failure (RVF) is also present. In this study, we report the use of intravenous prostacyclin in three PH-ILD patients to stabilise right ventricular (RV) function, with a subsequent transition to maintenance therapy with inhaled prostacyclin. We evaluated three consecutive PH-ILD patients with RVF. RV afterload and pulmonary vascular resistance (PVR) were treated with intravenous prostacyclin during the induction phase of the therapy. Patients transitioned from intravenous prostacyclin to the maintenance phase of the treatment with inhaled prostacyclin once three transition criteria were met: cardiac index (CI) >2 L·min-1·m-2, PVR <7 Wood units (WU) and tricuspid annular plane systolic excursion (TAPSE) change >1 mm or TAPSE >1.6 cm. Pre-treatment parameters for the three patients were a mean PVR of 14.3 WU, a mean Fick CI of 1.8 L·min-1·m-2 and a mean TAPSE of 1.4 cm. The average intravenous prostacyclin dose at the time of transition to maintenance therapy was 20.7 ng·kg-1·m-2 of treprostinil. At 3-months follow-up, the mean PVR was 6.3 WU, Fick CI 2.2 L·min-1·m-2 and TAPSE 1.7 cm. This case series of three PH-ILD patients with RVF introduces the concept of an initial intravenous prostacyclin induction phase, followed by a transition to maintenance therapy with inhaled prostacyclin. Further development of this treatment algorithm with a refinement of the transition criteria, potential testing in a clinical trial and a longer-term follow-up period is warranted to improve the outcomes of advanced PH-ILD patients with concomitant RVF.

Serum KL-6 as a Biomarker of Progression at Any Time in Fibrotic Interstitial Lung Disease.

The development of a progressive phenotype of interstitial lung disease (ILD) is still unpredictable. Whereas tools to predict mortality in ILD exist, scores to predict disease progression are missing. The aim of this study was to investigate whether baseline serum KL-6 as an established marker to assess disease activity in ILD, alone or in combination with clinical variables, could improve stratification of ILD patients according to progression risk at any time. Consecutive patients with fibrotic ILD, followed at our institution between 2008 and 2015, were investigated. Disease progression was defined as relative decline of ≥10% in forced vital capacity (FVC) or ≥15% in diffusing capacity of the lung for carbon monoxide (DLco)% from baseline at any time. Serum KL-6 was measured using an automated immunoassay (Fujirebio Europe, Gent, Belgium). A stepwise logistic regression was performed to select variables to be included in the score. A total of 205 patients (49% idiopathic pulmonary fibrosis (IPF), 51% fibrotic nonspecific interstitial pneumonia (NSIP)) were included, of them 113 (55%) developed disease progression during follow up. Male gender (G) and serum KL-6 strata (K) were significant predictors of progression at regression analysis and were included in the GK score. A threshold of 2 GK score points was best for discriminating patients at high risk versus low risk to develop disease progression at any time. Serum KL-6 concentration, alone or combined in a simple score with gender, allows an effective stratification of ILD patients for risk of disease progression at any time.

1148P The effects of statin use on lung cancer development in patients with idiopathic pulmonary fibrosis using clinical data warehouse

Statin use was associated with reduced all-cause mortality in interstitial lung disease and idiopathic pulmonary fibrosis (IPF) patients. To further investigate the potential preventive effects of statins on lung cancer development in IPF patients, we used clinical data warehouse (CDW) for electronic medical record (EMR)-based clinical research.

Pectoralis muscle area and its association with indices of disease severity in interstitial lung disease

RationaleThe pathophysiology of interstitial lung disease (ILD) impacts body composition, whereby ILD severity is linked to lower lean mass. ObjectivesTo determine i) if pectoralis muscle area (PMA) is a surrogate for whole-body lean mass in ILD, ii) whether PMA is associated with ILD severity, and iii) if the longitudinal change in PMA is associated with pulmonary function and mortality in ILD. MethodsPatients with ILD (n = 164) were analyzed retrospectively. PMA was quantified from a chest computed tomography scan. Peripheral oxygen saturation (SpO2), 6-min walk distance (6MWD), and pulmonary function were obtained as part of routine clinical care. Dyspnea and quality of life were assessed using the UCSD Shortness of Breath Questionnaire and European Quality of Life 5 Dimensions questionnaire, respectively. ResultsPMA was associated with whole-body lean mass (p < 0.001). After adjusting for age, sex, height, body mass, and prednisone status, PMA was associated with %-predicted forced vital capacity (FVC), %-predicted diffusion capacity (DLCO), resting and exertional SpO2, and dyspnea (all p < 0.05), but not forced expiratory volume in 1 s (FEV1), FEV1/FVC, 6MWD, or quality of life (all p > 0.05). The annual negative PMA slope was associated with annual negative slopes in FVC, FEV1, and DLCO (all p < 0.05), but not FEV1/FVC (p = 0.46). Annual slope in PMA was associated with all-cause mortality (hazard ratio = −0.80, 95% CI:0.889–0.959; p < 0.001). ConclusionIn patients with ILD, PMA is a suitable surrogate for whole-body lean mass. A lower PMA is associated with indices of ILD severity, which supports the notion that ILD progression may involve sarcopenia.

Better Together: A Mixed-Methods Study of Palliative Care Co-Management for Patients with Interstitial Lung Disease.

Background: The morbidity and mortality of interstitial lung disease (ILD) is high, despite novel therapeutics. Recognizing unmet needs for symptom management, advance care planning (ACP), and support for people with ILD and their families, we developed a palliative care-ILD collaborative care pilot program to improve access to palliative care. Methods: In the quantitative arm of this mixed-methods study, we evaluated which patients were cared for through the palliative care co-management program and the impact of the program on rates of ACP and opioid prescribing. In the qualitative arm, we interviewed patients and family caregivers, as well as pulmonary and palliative care clinicians, to understand perceptions about palliative care. Results: Thirty-one patients were co-managed by the palliative care and ILD teams during the study period. Half (48.4%) had idiopathic pulmonary fibrosis. Mean forced vital capacity (FVC) was 61.7%. Nearly half (48.4%) received all of their palliative care via telehealth. With palliative care, the rate of ACP notes increased from 3.2% to 100% (p < 0.001), rate of advance directive completion increased from 22.6% to 35.5% (p = 0.046), and rate of physician orders for life-sustaining treatments (POLST) form completion increased from 0% to 35.5% (p = 0.001). Half (51.6%) were prescribed opiates, overwhelmingly short-acting opiates to use as needed for severe episodic dyspnea. Themes from the qualitative analyses included that the palliative care team was supportive and patient-centered, improved symptoms and medication side effects, and enhanced illness understanding. Clinicians reported how palliative care co-management improved patient care and clinician experience, but barriers to referral remain including misperceptions about palliative care on the part of providers and patients. Conclusions: Palliative care co-management for patients with moderately severe ILD holds promise, and our experience can inform groups at other centers who are interested in developing such care models. Ongoing challenges include systematically reaching all patients who are likely to benefit.

Impact of post-capillary pulmonary hypertension on mortality in interstitial lung disease.

Pulmonary hypertension (PH) influences mortality in patients with interstitial lung disease (ILD). Almost all studies on patients with ILD, have focused on the clinical impact of pre-capillary PH on survival. Therefore, little is known about the influence of post-capillary PH. We aimed to assess the prevalence of post-capillary PH and its clinical impact on survival in patients with ILD, followed by comparison with pre-capillary PH. This retrospective study enrolled 1152 patients with ILD who were diagnosed with PH using right heart catheterization between May 2007 and December 2015. We analyzed the demographics and composite outcomes (defined as death from any cause or lung transplantation) of patients with post-capillary PH and compared them with patients with pre-capillary PH. Thirty-two (20%) of the 157 patients with ILD-PH were diagnosed with post-capillary PH. Patients with post-capillary PH had significantly lower modified Medical Research Council scores, higher diffusion capacity for carbon monoxide, higher resting PaO2, lower pulmonary vascular resistance (PVR), and higher lowest oxygen saturation during the 6-min walk test compared to those with pre-capillary PH. Cardiovascular diseases were associated with a higher risk of mortality in patients with post-capillary PH. Multivariate Cox proportional hazards analysis demonstrated no significant difference between the composite outcomes in pre-capillary and post-capillary PH, while PVR and the ILD Gender-Age-Physiology Index were significantly associated with the composite outcome. We found that approximately one-fifth of patients with ILD-PH were diagnosed with post-capillary PH, and that PVR and not post-capillary PH was associated with mortality.

Trends and seasonal variation of hospitalization and mortality of interstitial lung disease in the United States from 2006 to 2016

BackgroundIn the recent years, the overall trends in hospital admission and mortality of interstitial lung disease (ILD) are unknown. In addition, there was some evidence that interstitial lung disease death rate highest in the winter but this finding was only available in one study. This study will investigate the trend and seasonal variations in hospital admission and mortality rates of ILD from 2006 to 2016.MethodFrom the Nationwide Inpatient Sample database, we collected all cases with the International Classification of Diseases (ICD)-9 or ICD-10 codes of ILD excluding identifiable external causes (drug, organic or inorganic dusts) from 2006 to 2016. Hospitalization rates of each year were calculated based on U.S Census population data. Monthly hospitalization and in-hospital mortality rates were analyzed by seasonal and trend decomposition. Subgroups of idiopathic interstitial fibrosis (IPF), acute respiratory failure (ARF), pneumonia were analyzed.ResultsFrom 2006 to 2016, all-cause hospital admission rate of patients with interstitial lung disease (ILD) and IPF-only subgroup declined but their overall mortality remained unchanged (except IPF subgroup and acute respiratory failure subgroup). Acute respiratory failure related admission account for 23% of all causes and pneumonia 17.6%. Mortality of ILD in general and subgroup of ILD with ARF was highest in winter, up to 8.13% ± 0.60 and 26.3% ± 10.2% respectively. The seasonal variations of hospital admission and mortality of ILD in general was not changed when infectious pneumonia cases were ruled out. All cause admission rates were highest in months from January to April. Subgroup analysis also showed seasonal variations with highest hospitalization rates for all subgroups (IPF, ARF, pneumonia) in the months from December to April (winter to early Spring).ConclusionFrom 2006 to 2016, admission rates of ILD of all causes and IPF subgroup declined but in-hospital mortality of ILD of all causes remained unchanged. Mortality of IPF subgroup and acute respiratory failure subgroup trended down. All-cause hospital admissions and mortality of ILD have a strong seasonal variation. Hospitalization rates for all subgroups (IPF, ARF, pneumonia) were highest in the months from December to April.

Diagnostic and prognostic predictive values of circulating KL-6 for interstitial lung disease: A PRISMA-compliant systematic review and meta-analysis.

Past investigations showed inconsistent results for diagnostic and prognostic predictive values of Krebs von den Lungen-6 (KL-6) for interstitial lung disease (ILD). Web of Science and PubMed were systematically searched on for articles exploring the association of KL-6 and ILDs published between September 1993 and March 2019. For comparisons between-groups, the standard mean difference and 95% confidence intervals (CIs) were computed as the effect sizes. For diagnostic studies, a summary of sensitivity, specificity, positive likelihood ratios, negative likelihood ratios, and diagnostic odds ratio, which indicated the accuracy of KL-6 in the differentiation of ILDs and no ILDs, were calculated from the true positive, true negative, false positive, and false negative of each study. In addition, the summary receive-operating characteristics curve was constructed to summarize the TP and FP rates. For follow-up study, we computed hazard ratios (HRs) and 95% CIs for mortality. ILD patients showed elevated concentrations of KL-6, compared to healthy controls and patients without ILD. The meta-analysis showed a sensitivity (0.85 [95% CI: 0.77-0.91]) and specificity (0.97 [95% CI: 0.90-0.99]) of KL-6 for ILDs. In addition, it showed elevated baseline circulating levels of KL-6 in subsequent active ILD, compared to subsequent inactive ILD. Moreover, there was a significant association between baseline levels of circulating KL-6 and mortality of ILD (HR 2.95, 95% CI 2.45-3.55, I = 65.9%, P = .032). In conclusion, the study suggested that circulating KL-6 showed diagnostic and prognostic predictive values for ILDs.

Evaluation of Acute Physiology and Chronic Health Evaluation (APACHE) IV Score Warrick Scoring High Resolution CT Chest and Six-Minute Walk Test in Predicting Mortality in Patients with Interstitial Lung Disease

Abstract Background: Some clinical parameter and APACHE IV scoring systems allow assessment of severity of the disease in patients with interstitial lung diseases (ILD) and provide an estimate of in-hospital morbidity and mortality. Aim of Study: This study aimed to detect the ability of clinical parameters and APACHE IV score in predicting the mortality of interstitial lung disease patients in chest depart-ment. Patients and Methods: A prospective observational cohort study was performed at the Chest Department of Bab El-Sha'eria and Al-Hussein Al-Azhar University Hospitals. The study included patients with interstitial lung disease admitted between January 2015 and December 2019. Data were collected from 50 patients (29 female and 21 male) with age range from 24-61 years old (43.6±9.43). During the 1st day of admission, full history taking, HRCT Chest, Warrick score, pulmonary function test, arterial blood gases, APACH IV score, six minute walking test were recorded. Results: The study revealed that there; 38 (76%) patients with Interstitial Lung Disease survived and 12 (24%) non survived. There was a significant difference between PaO2, O2 sat, PaCO2, Systolic Pulmonary Artery pressure, and 6- minute walking distance, 4-meter walk time/seconds, APACH IV, Warrick score on admission between survived and non-survived subjects. Conclusion: Six-minute walk test, 4 meter walking time, systolic pulmonary artery pressure APACHE IV score and Warrick scoring high resolution C-T chest can be used as useful test in predicting mortality in interstitial lung disease.

IBS14.02 Best Management of Early Stage NSCLC in ILD Patients

IBS14.02 Best Management of Early Stage NSCLC in ILD Patients

1299P - Radiographic characteristics and poor prognostic factors of interstitial lung disease (ILD) in nivolumab-treated patients with non-small cell lung cancer (NSCLC)

1299P - Radiographic characteristics and poor prognostic factors of interstitial lung disease (ILD) in nivolumab-treated patients with non-small cell lung cancer (NSCLC)

Lung Cancer development and mortality in interstitial lung disease with and without connective tissue diseases: a five-year Nationwide population-based study

BackgroundConnective tissue disease associated with interstitial lung disease (CTD-ILD) and interstitial lung disease (ILD) alone have same pathological and imaging backgrounds. However, the differences between lung cancer development and the mortality risk between these two conditions are unclear. Incidence of primary lung cancer and all-cause mortality were studied between interstitial lung disease patients with and without connective tissue disease.MethodsData were extracted from the Korean National Health Insurance Research Database in 2009. A total of 12,787 cases of ILD without idiopathic pulmonary fibrosis and 2491 cases of CTD-ILD were diagnosed in 2009. The cohort was followed up until June 30, 2014. Incident lung cancers and all-cause mortality were ascertained.ResultsThe overall incidence of lung cancer was 165.7 and 161.8 per 10,000 person-years in the CTD-ILD and ILD-only, respectively (rate ratio, 1.08; 95% confidence interval, 0.89–1.30). CTD-ILD patients in the 40–49 and 50–59 years old age groups had lung cancer incidence rates of 92.5 and 139.2, which were 2.0 and 1.7 times higher than those in the ILD-only, respectively. All-cause mortality was significantly higher in the CTD-ILD group compared to ILD-only group in patients aged 50–79 years. All-cause mortality of women in the 50–59, 60–69 and 70–79 age groups was 2.0, 1.8, and 1.4 times higher in the CTD-ILD group than in the ILD-only group, respectively.ConclusionsCTD-ILD patients aged < 60 years had a higher lung cancer incidence than ILD-only patients in the same age group. Furthermore, CTD-ILD patients aged 50–79 years had higher all-cause mortality than ILD-only patients in the same age group.

Machine learning assisted evaluation of interstitial lung diseases

Interstitial lung disease (ILD) is an inflammatory condition encompassing greater than 200 different chronic disorders that often lead to pulmonary fibrosis and the attendant morbidity and mortality. Distinguishing one ILD from another can be challenging even for experts given similar clinical manifestations. However, firm diagnosis is important for management, counseling, and surveillance. Computer-Aided Lung Informatics for Pathology Evaluation and Ratings (CALIPER, Mayo Clinic, Rochester, MN, USA), a machine learned image analysis tool for characterizing and quantifying diffused lung diseases on CT, has been shown to correlate with ILD mortality such as for idiopathic pulmonary fibrosis. The CALIPER assessment of ILD is not influenced by confounding conditions such as emphysema or pulmonary hypertension that may affect pulmonary function measurements. Moreover, CALIPER can provide more reproducible results with less inter- and intraobserver variability compared to the conventional subjective visual assessment of CT scans by radiologists. Herein, we aim to provide an overview of ILD and discuss CALIPER evaluation of ILD.

Risk factors for mortality and mortality rates in interstitial lung disease patients in the intensive care unit.

Data on interstitial lung disease (ILD) outcomes in the intensive care unit (ICU) is of limited value due to population heterogeneity. The aim of this study was to examine risk factors for mortality and ILD mortality rates in the ICU.We performed a systematic review using five databases. 50 studies were identified and 34 were included: 17 studies on various aetiologies of ILD (mixed-ILD) and 17 on idiopathic pulmonary fibrosis (IPF). In mixed-ILD, elevated APACHE score, hypoxaemia and mechanical ventilation are risk factors for mortality. No increased mortality was found with steroid use. Evidence is inconclusive on advanced age. In IPF, evidence is inconclusive for all factors except mechanical ventilation and hypoxaemia. The overall in-hospital mortality was available in 15 studies on mixed-ILD (62% in 2001-2009 and 48% in 2010-2017) and 15 studies on IPF (79% in 1993-2004 and 65% in 2005-2017). Follow-up mortality rate at 1 year ranged between 53% and 100%.Irrespective of ILD aetiology, mechanical ventilation is associated with increased mortality. For mixed-ILD, hypoxaemia and APACHE scores are also associated with increased mortality. IPF has the highest mortality rate among ILDs, but since 1993 the rate appears to be declining. Despite improving in-hospital survival, overall mortality remains high.

Prognostic factors and outcome of mechanically ventilated interstitial lung disease patients

BackgroundThe prognosis of mechanically ventilated interstitial lung disease (ILD) patients was controversial in previous studies. Identifying the factors associated with mortality could guide therapy and allow good use of ICU resources.AimThe aim was to study the outcome of ILD mechanically ventilated patients admitted to the respiratory ICU and to demonstrate the possible factors associated with mortality in these patients.Patients and methodsThe observational prospective study was carried out on ILD patients undergoing mechanical ventilation, either invasive mechanical ventilation (IMV) or noninvasive ventilation (NIV). Clinical, radiological, and outcome assessments were done for all enrolled patients. For outcome assessment, patients were classified into either survivors or nonsurvivors.ResultsTwenty-one (70%) of the patients were subjected to NIV, whereas nine (30%) of them were subjected to IMV. The overall mortality rate was 53.3%. However, the mortality rate was 35% in patients with NIV, but 100% in patients with IMV. Severity assessment scores were significantly higher in nonsurvivors compared with survivors. Nonsurvivors also presented significantly with lower pH and higher PaCO2 compared with survivors. Acute Physiology and Chronic Health Evaluation-II score greater than or equal to 18.5, Simplified Acute Physiology Score greater than or equal to 27.5, Glasgow coma scale score less than 12.5 and PaO2/ FiO2 less than 161.5 were associated with increased risk of mortality of ILD patients.ConclusionMechanically ventilated ILD patients had a poor outcome. However, the survival rate of ILD patients was better on NIV than IMV. Severity assessment scores and PaO2/FiO2 could predict the risk of mortality in ILD patients.

African-American race and mortality in interstitial lung disease: a multicentre propensity-matched analysis

We studied whether African-American race is associated with younger age and decreased survival time at diagnosis of interstitial lung disease (ILD).We performed a multicentre, propensity score-matched analysis of patients with an ILD diagnosis followed at five US hospitals between 2006 and 2016. African-Americans were matched with patients of other races based on a time-dependent propensity score calculated from multiple patient, physiological, diagnostic and hospital characteristics. Multivariable logistic regression models were used. All-cause mortality and hospitalisations were compared between race-stratified patient cohorts with ILD, and sensitivity analyses were performed.The study included 1640 patients with ILD, 13% of whom were African-American, followed over 5041 person-years. When compared with patients of other races, African-Americans with ILD were younger at diagnosis (56 years versus 67 years), but in the propensity-matched analyses had greater survival (hazard ratio 0.46, 95% CI 0.28-0.77; p=0.003) despite similar risk of respiratory hospitalisations (relative risk 1.04, 95% CI 0.83-1.31; p=0.709), and similar GAP-ILD (gender-age-physiology-ILD) scores at study entry. Sensitivity analyses in a separate cohort of 9503 patients with code-based ILD diagnosis demonstrated a similar association of baseline demographic characteristics with all-cause mortality.We conclude that African-Americans demonstrate a unique phenotype associated with younger age at ILD diagnosis and perhaps longer survival time.