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Rationale: Cough is a key symptom in patients with fibrotic interstitial lung disease (ILD). Objective: This study evaluated the prevalence, longitudinal change, associations, and prognostic significance of cough severity in patients with fibrotic ILD. Methods: We included consecutive patients with idiopathic pulmonary fibrosis (IPF) and non-IPF fibrotic ILD who completed the 100mm-Cough Severity Visual Analog Scale (VAS) from the prospective multi-center Canadian Registry for Pulmonary Fibrosis. Baseline cough severity and associations with patient demographics and clinical factors were determined. Relationships between baseline cough severity and health outcomes were evaluated. Measurements and Main Results: Patients with IPF (n=1061) had higher median baseline cough severity than those with non-IPF fibrotic ILD (n=2825) [24 vs 20mm, p<0.001], with worse cough associated with gastroesophageal reflux disease for both cohorts. Worse cough severity was independently associated with worse health-related quality of life at baseline, larger annualized decline in DLCO, development of disease progression, and reduced transplant-free survival in both IPF and non-IPF fibrotic ILD cohorts. The IPF cohort (2.2mm, 95% CI 1.6-2.9mm) had larger annualized increments in cough severity compared to the non-IPF fibrotic ILD cohort (1.1mm, 95% CI 0.8-1.4mm; p=0.004). There was no difference in worsening cough over time comparing those receiving and not receiving ILD-targeted therapy or with and without lung function decline. Conclusion: Cough is common in patients with IPF and non-IPF fibrotic ILD, with increasing cough severity over time irrespective of ILD-targeted therapy. Patient-reported cough severity has prognostic implications on health-related quality of life, disease progression, and survival in fibrotic ILD.
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OBJECTIVES: Interstitial lung disease (ILD) in connective tissue diseases (CTD) have highly variable morphology. We aimed to identify imaging features and their impact on ILD progression, mortality and immunosuppression response. METHODS: Patients with CTD-ILD had high-resolution chest computed tomography (HRCT) reviewed by expert radiologists blinded to clinical data for overall imaging pattern (usual interstitial pneumonia [UIP]; non-specific interstitial pneumonia [NSIP]; organizing pneumonia [OP]; fibrotic hypersensitivity pneumonitis [fHP]; and other). Transplant-free survival and change in percent-predicted forced vital capacity (FVC) were compared using Cox and linear mixed effects models adjusted for age, sex, smoking, and baseline FVC. FVC decline after immunosuppression was compared with pre-treatment. RESULTS: Of 645 CTD-ILD patients, the frequent CTDs were systemic sclerosis (n = 215), rheumatoid arthritis (n = 127), and inflammatory myopathies (n = 100). NSIP was the most common pattern (54%), followed by UIP (20%), fHP (9%), and OP (5%). Compared with UIP, FVC decline was slower for NSIP (1.1%/year, 95%CI 0.2, 1.9) and OP (3.5%/year, 95%CI 2.0, 4.9), and mortality was lower for NSIP (HR 0.65, 95%CI 0.45, 0.93) and OP (HR 0.18, 95%CI 0.05, 0.57), but higher in fHP (HR 1.58, 95%CI 1.01, 2.40). The extent of fibrosis also predicted FVC decline and mortality. After immunosuppression, FVC decline was slower compared with pre-treatment in NSIP (by 2.1%/year, 95%CI 1.4, 2.8), with no change for UIP or fHP. CONCLUSION: Multiple radiologic patterns are possible in CTD-ILD, including a fHP pattern. NSIP and OP were associated with better outcomes and response to immunosuppression, while fHP had worse survival compared with UIP.
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BACKGROUND AND OBJECTIVE: Establishing an accurate and timely diagnosis of idiopathic pulmonary fibrosis (IPF) is essential for appropriate management and prognostication. In some cases, surgical lung biopsy (SLB) is performed but carries non-negligible risk. The objective of this retrospective study was to determine if SLB is associated with accelerated lung function decline in patients with IPF using the Canadian Registry for Pulmonary Fibrosis. METHODS: Linear mixed models and Cox proportional hazards regression models were used to compare decline in forced vital capacity (FVC)%, diffusion capacity of the lung (DLCO%) and risk of death or lung transplantation between SLB and non-SLB patients. Adjustments were made for baseline age, sex, smoking history, antifibrotic use, and lung function. A similar analysis compared lung function changes 12 months pre- and post-SLB. RESULTS: A total of 81 SLB patients and 468 non-SLB patients were included. In the SLB group, the post-biopsy annual FVC% decline was 2.0% (±0.8) in unadjusted, and 2.1% (±0.8) in adjusted models. There was no difference in FVC% decline, DLCO% decline, or time to death or lung transplantation between the two groups, in adjusted or unadjusted models (all p-values >0.07). In the pre-post SLB group, no differences were identified in FVC% decline in unadjusted or adjusted models (p = 0.07 for both). CONCLUSION: No association between SLB and lung function decline or risk of death or lung transplantation was identified in this multi-centre study of patients with IPF.
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Fibrose Pulmonar Idiopática , Pulmão , Sistema de Registros , Humanos , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/cirurgia , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/patologia , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Biópsia , Pulmão/patologia , Pulmão/fisiopatologia , Pulmão/cirurgia , Idoso , Capacidade Vital/fisiologia , Transplante de Pulmão , Canadá/epidemiologia , Testes de Função Respiratória , Prognóstico , Modelos de Riscos Proporcionais , Estudos de Coortes , Taxa de SobrevidaRESUMO
BACKGROUND: Approximately 50% of patients with interstitial lung disease (ILD) experience frailty, which remains unexplored in acute exacerbations of ILD (AE-ILD). A better understanding may help with prognostication and resource planning. We evaluated the association of frailty with clinical characteristics, physical function, hospital outcomes, and post-AE-ILD recovery. METHODS: Retrospective cohort study of AE-ILD patients (01/2015-10/2019) with frailty (proportion ≥0.25) on a 30-item cumulative-deficits index. Frail and non-frail patients were compared for pre- and post-hospitalization clinical characteristics, adjusted for age, sex, and ILD diagnosis. One-year mortality, considering transplantation as a competing risk, was analysed adjusting for age, frailty, and Charlson Comorbidity Index (CCI). RESULTS: 89 AE-ILD patients were admitted (median: 67 years, 63% idiopathic pulmonary fibrosis). 31 were frail, which was associated with older age, greater CCI, lower 6-min walk distance, and decreased independence pre-hospitalization. Frail patients had more major complications (32% vs 10%, p = .01) and required more multidisciplinary support during hospitalization. Frailty was not associated with 1-year mortality (HR: 0.97, 95%CI: [0.45-2.10]) factoring transplantation as a competing risk. CONCLUSIONS: Frailty was associated with reduced exercise capacity, increased comorbidities and hospital complications. Identifying frailty may highlight those requiring additional multidisciplinary support, but further study is needed to explore whether frailty is modifiable with AE-ILD.
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Fragilidade , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Humanos , Fragilidade/complicações , Fragilidade/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Doenças Pulmonares Intersticiais/diagnóstico , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/terapia , Fibrose Pulmonar Idiopática/diagnóstico , PrognósticoRESUMO
BACKGROUND: Little is known about generalisability of randomised controlled trials (RCTs) for idiopathic pulmonary fibrosis (IPF). We evaluated eligibility criteria for phase III IPF RCTs to determine their representativeness in clinical registries, and calculated forced vital capacity (FVC) changes according to eligibility criteria. METHODS: Common eligibility criteria used in >60% of IPF RCTs were identified from a literature search and applied to patients with IPF from prospective Australian and Canadian registries. Additional pre-specified criteria of 6-min walk distance (6MWD) and different measures of preceding disease progression were also evaluated. Joint longitudinal-survival modelling was used to compare FVC decline according to eligibility for individual and composite criteria. RESULTS: Out of 990 patients with IPF, 527 (53%) met all common RCT eligibility criteria at the first clinic visit, including 343 with definite IPF and 184 with radiological probable usual interstitial pneumonia pattern without histological confirmation (i.e. provisional IPF). The percentages of eligible patients for landmark RCTs of nintedanib and pirfenidone were 19-50%. Adding 6MWD ≥150â m and different measures of preceding disease progression to the composite common criteria reduced the percentages of patients meeting eligibility to 52% (n=516) and 4-18% (n=12-61), respectively. Patients meeting the composite common criteria had less-rapid 1-year FVC decline than those who did not (-90 versus -103â mL, p=0.01). Definite IPF generally had more-rapid 1-year FVC decline compared to provisional IPF. CONCLUSIONS: Eligibility criteria of previous IPF RCTs have limited generalisability to clinical IPF populations, with FVC decline differing between eligible and ineligible populations.
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Fibrose Pulmonar Idiopática , Humanos , Austrália , Canadá , Fibrose Pulmonar Idiopática/tratamento farmacológico , Capacidade Vital , Progressão da Doença , Piridonas/uso terapêutico , Sistema de Registros , Preparações Farmacêuticas , Resultado do Tratamento , Ensaios Clínicos Controlados Aleatórios como AssuntoRESUMO
Rationale: Fibrotic interstitial lung disease (fILD) is a group of pathologic entities characterized by scarring of the lungs and high morbidity and mortality. Research investigating how socioeconomic and residential factors impact outcomes in patients with fILD is lacking. Objectives: To determine the association between neighborhood-level disadvantage and presentation severity, disease progression, lung transplantation, and mortality in patients with fILD from the United States and Canada. Methods: We performed a multicenter, international, prospective cohort study of 4,729 patients with fILD from one U.S. and eight Canadian ILD registry sites. Neighborhood-level disadvantage was measured by the area deprivation index in the United States and the Canadian Index of Multiple Deprivation in Canada. Measurements and Main Results: In the U.S. but not in the Canadian cohort, patients with fILD living in neighborhoods with the greatest disadvantage (top quartile) experience the highest risk of mortality (hazard ratio = 1.51, P = 0.002), and in subgroups of patients with idiopathic pulmonary fibrosis, the top quartile of disadvantage experienced the lowest odds of lung transplantation (odds ratio = 0.46, P = 0.04). Greater disadvantage was associated with reduced baseline DLCO in both cohorts, but it was not associated with baseline FVC or FVC or DLCO decline in either cohort. Conclusions: Patients with fILD who live in areas with greater neighborhood-level disadvantage in the United States experience higher mortality, and patients with idiopathic pulmonary fibrosis experience lower odds of lung transplantation. These disparities are not seen in Canadian patients, which may indicate differences in access to care between the United States and Canada.
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Disparidades nos Níveis de Saúde , Disparidades em Assistência à Saúde , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Características de Residência , Privação Social , Determinantes Sociais da Saúde , Idoso , Canadá/epidemiologia , Progressão da Doença , Feminino , Disparidades em Assistência à Saúde/economia , Disparidades em Assistência à Saúde/estatística & dados numéricos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/economia , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/cirurgia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/economia , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/cirurgia , Transplante de Pulmão/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Gravidade do Paciente , Prognóstico , Estudos Prospectivos , Fatores de Risco , Estados Unidos/epidemiologiaRESUMO
BACKGROUND: Individuals with lung disease commonly use the internet as a source of health information on pulmonary rehabilitation (PR). The objective of this study was to characterize internet resources on PR, and to assess the content, readability, and quality of patient-directed PR resources. METHODS: The first 200 websites for the search term 'pulmonary rehabilitation resources and exercise' were analyzed on Google, Yahoo, and Bing. Website content was assessed based on 30 key components of PR from the 2013 and 2021 international consensus statements. Website quality was determined using DISCERN, JAMA benchmarks, and Global Quality Scale (GQS). RESULTS: 66 unique PR websites were identified with the two most common categories being scientific resources (39%) and foundation/advocacy organizations (33%). The average reading level of websites was 11 ± 3. PR content varied significantly across websites (mean range 13.4-21.5). Median DISCERN total score and GQS score were 4 (IQR 3-4) and 3.5 (IQR 2-4), respectively, representing moderate-good quality. Foundation/advocacy websites had higher DISCERN and GQS scores compared to other websites. CONCLUSION: Foundation/advocacy websites had the highest quality and reliability metrics; however, the higher-than-recommended reading levels may compromise patient comprehension and utilization. This study provides critical insight on the current state of online PR health-related information.
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Compreensão , Pneumopatias , Humanos , Reprodutibilidade dos TestesRESUMO
BACKGROUND: Progressive fibrosing interstitial lung disease (PF-ILD) is characterised by progressive physiological, symptomatic and/or radiographic worsening. The real-world prevalence and characteristics of PF-ILD remain uncertain. METHODS: Patients were enrolled from the Canadian Registry for Pulmonary Fibrosis between 2015 and 2020. PF-ILD was defined as a relative forced vital capacity (FVC) decline ≥10%, death, lung transplantation or any two of: relative FVC decline ≥5% and <10%, worsening respiratory symptoms or worsening fibrosis on computed tomography of the chest, all within 24â months of diagnosis. Time-to-event analysis compared progression between key diagnostic subgroups. Characteristics associated with progression were determined by multivariable regression. RESULTS: Of 2746 patients with fibrotic ILD (mean±sd age 65±12â years; 51% female), 1376 (50%) met PF-ILD criteria in the first 24â months of follow-up. PF-ILD occurred in 427 (59%) patients with idiopathic pulmonary fibrosis (IPF), 125 (58%) with fibrotic hypersensitivity pneumonitis (HP), 281 (51%) with unclassifiable ILD (U-ILD) and 402 (45%) with connective tissue disease-associated ILD (CTD-ILD). Compared with IPF, time to progression was similar in patients with HP (hazard ratio (HR) 0.96, 95% CI 0.79-1.17), but was delayed in patients with U-ILD (HR 0.82, 95% CI 0.71-0.96) and CTD-ILD (HR 0.65, 95% CI 0.56-0.74). Background treatment varied across diagnostic subtypes, with 66% of IPF patients receiving antifibrotic therapy, while immunomodulatory therapy was utilised in 49%, 61% and 37% of patients with CHP, CTD-ILD and U-ILD, respectively. Increasing age, male sex, gastro-oesophageal reflux disease and lower baseline pulmonary function were independently associated with progression. CONCLUSIONS: Progression is common in patients with fibrotic ILD, and is similarly prevalent in HP and IPF. Routinely collected variables help identify patients at risk for progression and may guide therapeutic strategies.
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Alveolite Alérgica Extrínseca , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Idoso , Alveolite Alérgica Extrínseca/complicações , Alveolite Alérgica Extrínseca/epidemiologia , Canadá/epidemiologia , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Sistema de RegistrosRESUMO
PURPOSE OF REVIEW: This review provides an update on recent advances in the diagnosis, pathogenesis, clinical presentation, histopathological findings, and treatment approaches for antineutrophil cytoplasmic antibody (ANCA) vasculitis-associated interstitial lung disease (AAV-ILD) with a focus on literature published in the last 3 years. RECENT FINDINGS: Although there is no validated definition of AAV-ILD, which contributes to some of the heterogeneity seen in study results, there has been an increasing number of publications in recent years on this topic. Most patients with AAV-ILD have MPO-ANCA vasculitis, and this association appears to reduce their 5-year-survival to 60-66% (Sun et al. BMC Pulm Med 21(1), 2021, Maillet et al. J Autoimmun 106, 2020). Median age of diagnosis ranges from mid-60 s to mid-70 s (Ando et al. Respir Med 107(4), 2013), Kagiyama et al. BMJ Open Respir Res 2(1):1-9, 2015, Hozumi et al. Lung 194(2):235-42, 2016, Liu et al. Chest 156(4):715-23, 2019, Maillet et al. J Autoimmun 106, 2020, Wurmann et al. Sarcoidosis Vasc Diffuse Lung Dis 37(1):37-42, 2020, Watanabe et al. BMC Pulm Med 19(1), 2019). Computed tomography (CT) chest imaging for patients with AAV-ILD often shows a usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) pattern (12-58% and 13-61%, respectively) (Sun et al. BMC Pulm Med 21(1), 2021, Maillet et al. J Autoimmun 106, 2020, Wurmann et al. Sarcoidosis Vasc Diffuse Lung Dis 37(1):37-42, 2020, Watanabe et al. BMC Pulm Med 19(1), 2019, Baqir at al. Sarcoidosis Vasc Diffuse Lung Dis Off J WASOG 36(3):195-201, 2019). Additionally, lung biopsies typically do not demonstrate active inflammation, or capillaritis, questioning whether these patients should be treated with either immunotherapy or anti-fibrotic therapy, or both (Hozumi et al. Lung 194(2):235-42, 2016, Liu et al. Chest 156(4):715-23, 2019, Kitching at al. Nat Rev Dis Prim 6(1):71, 2020, Tanaka et al. Respir Med 106(12):1765-70, 2012). Besides immunosuppressive treatments, recent advances in anti-fibrotic therapy may offer patients with progressive AAV-ILD an alternative and/or more effective and individualized treatment option.
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Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Doenças Pulmonares Intersticiais , Sarcoidose , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Pulmão , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/terapia , PeroxidaseRESUMO
BACKGROUND AND OBJECTIVE: Rheumatoid arthritis (RA) is a frequent cause of interstitial lung disease (ILD); however, the impact of rheumatoid factor and anti-citrullinated peptide antibody seropositivity in ILD without connective tissue disease (CTD) is unclear. We examined the association of seropositivity with ILD progression, mortality and response to immunosuppression in non-CTD ILD. METHODS: A total of 1570 non-CTD patients (with idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, interstitial pneumonia with autoimmune features or unclassifiable ILD) and 181 RA-ILD patients were included from a prospective registry. Longitudinal forced vital capacity (FVC), transplant-free survival and incidence of progressive fibrosing-ILD (PF-ILD) were compared between seronegative non-CTD ILD (reference group), seropositive non-CTD ILD and RA-ILD using linear mixed-effect and Cox proportional hazards models adjusted for age, sex, smoking pack-years and baseline FVC. Interaction between seropositivity and immunosuppression on FVC decline was assessed in patients with ≥6 months of follow-up before and after the treatment. RESULTS: Two hundred and seventeen (13.8%) patients with seropositive non-CTD ILD had similar rates of FVC decline and transplant-free survival compared to seronegative non-CTD ILD, but more frequently met the criteria for PF-ILD (hazard ratio [HR] = 1.35, p = 0.004). RA-ILD had slower FVC decline (p = 0.03), less PF-ILD (HR = 0.75, p = 0.03) and lower likelihood of lung transplant or death (HR = 0.66, p = 0.01) compared to seronegative non-CTD ILD. No interaction was found between seropositivity and treatment on FVC decline in non-CTD ILD. CONCLUSION: Seropositivity in non-CTD ILD was not associated with improved outcomes or treatment response, highlighting the importance of other disease features in determining prognosis and predicting response to immunosuppression.
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Artrite Reumatoide , Doenças do Tecido Conjuntivo , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Artrite Reumatoide/complicações , Doenças do Tecido Conjuntivo/complicações , Humanos , Fibrose Pulmonar Idiopática/complicações , Pulmão , Doenças Pulmonares Intersticiais/etiologia , Peptídeos/uso terapêutico , Fator ReumatoideRESUMO
BACKGROUND AND OBJECTIVE: Inhalational exposures are a known cause of interstitial lung disease (ILD), but little is understood about their prevalence across ILD subtypes and their relationship with pulmonary function and survival. METHODS: Patients with fibrotic ILD were identified from the multicentre Canadian Registry for Pulmonary Fibrosis. Patients completed questionnaires regarding ILD-related occupational and environmental exposures. The relationship between exposures and the outcomes of baseline age, gender, family history, pulmonary function and survival was analysed using linear and logistic regression models, linear mixed-effect regression models and survival analysis using multivariable Cox proportional hazards along with the log-rank test. RESULTS: There were 3820 patients included in this study, with 2385 (62%) having ILD-related inhalational exposure. Exposed patients were younger, particularly in the idiopathic pulmonary fibrosis subgroup. Inhalational exposure was associated with male gender (adjusted OR 1.46, 95% CI 1.28-1.68, p < 0.001) and family history of pulmonary fibrosis (adjusted OR 1.73, 95% CI 1.40-2.15, p < 0.001). Patients with any inhalational exposure had improved transplant-free survival (hazard ratio 0.81, 95% CI 0.71-0.92, p = 0.001); this effect persisted across diagnostic subtypes. The relationship between exposures and annual change in forced vital capacity varied by ILD subtype. CONCLUSION: Patients with fibrotic ILD report high prevalence of inhalational exposures across ILD subtypes. These exposures were associated with younger age at diagnosis, male gender and family history of pulmonary fibrosis. Identification of an inhalational exposure was associated with a survival benefit. These findings suggest that inhaled exposures may impact clinical outcomes in patients with ILD, and future work should characterize the mechanisms underlying these relationships.
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Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Canadá/epidemiologia , Fibrose , Humanos , Fibrose Pulmonar Idiopática/complicações , Pulmão , Masculino , Sistema de RegistrosRESUMO
RATIONALE: The European Quality of Life 5-Dimensions 5-Levels questionnaire (EQ-5D-5L) is a multidimensional patient-reported questionnaire that supports calculation of quality-adjusted life-years. Our objectives were to demonstrate feasibility of use and to calculate the minimum important difference (MID) of the EQ-5D-5L and its associated visual analogue scale (EQ-VAS) in patients with fibrotic interstitial lung disease (ILD). METHODS: Patients who completed the EQ-5D-5L were identified from the prospective multicentre CAnadian REgistry for Pulmonary Fibrosis. Validity, internal consistency and responsiveness of the EQ-5D-5L were assessed, followed by calculation of the MID for the EQ-5D-5L and EQ-VAS. Anchor-based methods used an unadjusted linear regression against pulmonary function tests (PFTs) and dyspnoea and other quality of life questionnaires. Distribution-based method used one-half SD and SE measurement (SEM) calculations. RESULTS: 1816 patients were analysed, including 472 (26%) with idiopathic pulmonary fibrosis. EQ-5D-5L scores were strongly correlated with the dyspnoea and other quality of life questionnaires and weakly associated with PFTs. The estimated MID for EQ-5D-5L ranged from 0.0050 to 0.054 and from 0.078 to 0.095 for the anchor-based and distribution-based methods, respectively. The MID for EQ-VAS ranged from 0.5 to 5.0 and from 8.0 to 9.7 for the anchor-based and distribution-based methods. Findings were similar across ILD subtypes, sex and age. CONCLUSION: We used a large and diverse cohort of patients with a variety of fibrotic ILD subtypes to suggest validity and MID of both the EQ-5D-5L and EQ-VAS. These findings will assist in designing future clinical trials and supporting cost-effectiveness analyses of potential treatments for patients with fibrotic ILD.
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Psicometria/métodos , Fibrose Pulmonar/psicologia , Qualidade de Vida , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Reprodutibilidade dos Testes , Inquéritos e QuestionáriosRESUMO
RATIONALE: The University of California, San Diego Shortness of Breath Questionnaire (UCSDSOBQ) is a frequently used domain-specific dyspnea questionnaire; however, there is little information available regarding its use and minimum important difference (MID) in fibrotic interstitial lung disease (ILD). We aimed to describe the key performance characteristics of the UCSDSOBQ in this population. METHODS: UCSDSOBQ scores and selected anchors were measured in 1933 patients from the prospective multi-center Canadian Registry for Pulmonary Fibrosis. Anchors included the St. George's Respiratory Questionnaire (SGRQ), European Quality of Life 5 Dimensions 5 Levels questionnaire (EQ-5D-5L) and EQ visual analogue scale (EQ-VAS), percent-predicted forced vital capacity (FVC%), diffusing capacity of the lung for carbon monoxide (DLCO%), and 6-min walk distance (6MWD). Concurrent validity, internal consistency, ceiling and floor effects, and responsiveness were assessed, followed by estimation of the MID by anchor-based (linear regression) and distribution-based methods (standard error of measurement). RESULTS: The UCSDSOBQ had a high level of internal consistency (Cronbach's alpha = 0.97), no obvious floor or ceiling effect, strong correlations with SGRQ, EQ-5D-5L, and EQ-VAS (|r| > 0.5), and moderate correlations with FVC%, DLCO%, and 6MWD (0.3 < |r| < 0.5). The MID estimate for UCSDSOBQ was 5 points (1-8) for the anchor-based method, and 4.5 points for the distribution-based method. CONCLUSION: This study demonstrates the validity of UCSDSOBQ in a large and heterogeneous population of patients with fibrotic ILD, and provides a robust MID estimate of 5-8 points.
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Dispneia/diagnóstico , Dispneia/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Inquéritos e Questionários/normas , Idoso , Canadá/epidemiologia , Estudos de Coortes , Dispneia/fisiopatologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/epidemiologia , Fibrose Pulmonar/fisiopatologia , Sistema de Registros/normas , Reprodutibilidade dos Testes , Capacidade Vital/fisiologiaRESUMO
BACKGROUND AND OBJECTIVE: Despite the high mortality of acute exacerbations of interstitial lung disease (AE-ILD), there is minimal evidence to guide management decisions. We aimed to assess the feasibility and outcomes of a standardized management protocol for AE-ILD. METHODS: We performed a retrospective cohort study of patients with AE-ILD admitted to hospital between January 2015 and August 2019. Patients were managed with a standardized protocol including chest computed tomography (CT) at diagnosis, pulse corticosteroid treatment, and a follow-up CT 7 days after corticosteroid pulse. The association between idiopathic pulmonary fibrosis (IPF) versus non-IPF diagnosis and transplant-free survival within 1-year of AE-ILD was assessed using adjusted Cox proportional hazards regression survival analysis. Associations with CT chest improvement 7 days after corticosteroid pulse were secondarily assessed. RESULTS: 89 patients with AE-ILD were identified. 1-year transplant-free and overall survival were 20.2 and 51.7%, respectively. Protocol adherence to pulse corticosteroids was high (95.5%). A diagnosis of IPF was associated with higher risk of death or transplant at 1-year versus a non-IPF diagnosis [hazard ratio (HR) 2.23, 95% CI 1.19-4.17, p = 0.012]. There were no significant associations with 7-day CT improvement; however, CT improvement was associated with higher transplant-free survival (p = 0.02) and a lower risk of in-hospital mortality (χ2 = 7.06, p = 0.01) on unadjusted analysis. CONCLUSIONS: IPF is associated with a higher risk of death or transplant at 1-year as compared to a non-IPF diagnosis in patients with AE-ILD managed using a standardized protocol. Improvement on CT chest 7 days after corticosteroid pulse is associated with better survival.
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Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Progressão da Doença , Estudos de Viabilidade , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/tratamento farmacológico , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/terapia , Prognóstico , Estudos RetrospectivosRESUMO
BACKGROUND: Comorbidities are frequent and have been associated with poor quality of life, increased hospitalizations, and mortality in patients with interstitial lung disease (ILD). However, it is unclear how comorbidities lead to these negative outcomes and whether they could influence ILD disease progression. The goal of this study was to identify clusters of patients based on similar comorbidity profiles and to determine whether these clusters were associated with rate of lung function decline and/or mortality. METHODS: Patients with a major fibrotic ILD (idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis, connective tissue disease-associated ILD, and unclassifiable ILD) from the CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) were included. Hierarchical agglomerative clustering of comorbidities, age, sex, and smoking pack-years was conducted for each ILD subtype to identify combinations of these features that frequently occurred together in patients. The association between clusters and change in lung function over time was determined using linear mixed effects modeling, with adjustment for age, sex, and smoking pack-years. Kaplan Meier curves were used to assess differences in survival between the clusters. RESULTS: Discrete clusters were identified within each fibrotic ILD. In IPF, males with obstructive sleep apnea (OSA) had more rapid decline in FVC %-predicted (- 11.9% per year [95% CI - 15.3, - 8.5]) compared to females without any comorbidities (- 8.1% per year [95% CI - 13.6, - 2.7]; p = 0.03). Females without comorbidities also had significantly longer survival compared to all other IPF clusters. There were no significant differences in rate of lung function decline or survival between clusters in the other fibrotic ILD subtypes. CONCLUSIONS: The combination of male sex and OSA may portend worse outcomes in IPF. Further research is required to elucidate the interplay between sex and comorbidities in ILD, as well as the role of OSA in ILD disease progression.
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Alveolite Alérgica Extrínseca/epidemiologia , Fibrose Pulmonar Idiopática/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Adulto , Fatores Etários , Idoso , Alveolite Alérgica Extrínseca/diagnóstico , Canadá/epidemiologia , Análise por Conglomerados , Comorbidade , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Medição de Risco , Fatores de Risco , Fatores Sexuais , Apneia Obstrutiva do Sono/epidemiologia , Fumar/efeitos adversos , Fumar/epidemiologia , Fatores de TempoRESUMO
Sarcoidosis-related mortality appears to be rising in North America, with increasing rates in females and the elderly. We aimed to estimate trends in sarcoidosis incidence, prevalence and mortality in Ontario, Canada.We performed a cohort study using health administrative data from Ontario between 1996 and 2015. International Classification of Diseases and Ontario Health Insurance Plan codes were used for case detection. Three disease definitions were created: 1) sarcoidosis, two or more physician claims within 2â years; 2) chronic sarcoidosis, five or more physician claims within 3â years; and 3) sarcoidosis with histology, two or more physician claims with a tissue biopsy performed between claims.Overall, 18â550, 9199 and 3819 individuals with sarcoidosis, chronic sarcoidosis and sarcoidosis with histology, respectively, were identified. The prevalence of sarcoidosis was 143 per 100â000 in 2015, increasing by 116% (p<0.0001) from 1996. The increase in age-adjusted prevalence was higher in males than females (136% versus 99%; p<0.0001). The incidence of sarcoidosis declined from 7.9 to 6.8 per 100â000 between 1996 and 2014 (15% decrease; p=0.0009). A 30.3% decrease in incidence was seen among females (p<0.0001) compared with a 5.5% increase in males (p=0.47). Age- and sex-adjusted mortality rates of patients with sarcoidosis rose from 1.15% to 1.47% between 1996 and 2015 (28% increase; p=0.02), with the overall trend being nonsignificant (p=0.39). Mortality rates in patients with chronic sarcoidosis increased significantly over the study period (p=0.0008).The prevalence of sarcoidosis is rising in Ontario, with an apparent shifting trend in disease burden from females to males. Mortality is increasing in patients with chronic sarcoidosis.
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Mortalidade/tendências , Sarcoidose/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Ontário/epidemiologia , Prevalência , Sarcoidose/mortalidade , Distribuição por Sexo , Assistência de Saúde Universal , Adulto JovemRESUMO
Surgical volume-outcome relationships are well established but have not been studied in patients with interstitial lung disease (ILD) undergoing surgical lung biopsy (SLB). Our study objective was to determine if hospital SLB volume is associated with post-operative mortality in patients with ILD.A cohort study using administrative, population-based data from Ontario, Canada was performed in adults with ILD who underwent a SLB between 2001 and 2014. The association between yearly hospital SLB volume and 30-day post-operative mortality was assessed using multilevel logistic regression modelling.3057 surgical lung biopsies for ILD were performed during the study period with a median (interquartile range) yearly hospital volume of 73 (34-143) procedures. 30-day mortality was 7.1%, 20.2% and 1.9% in overall, nonelective and elective patients, respectively. Higher yearly hospital SLB volume was associated with lower odds of 30-day post-operative mortality after adjusting for patient characteristics (OR 0.84, 95% CI 0.73-0.97; p=0.02), with the association appearing stronger for nonelective versus elective procedures (OR 0.84, 95% CI 0.69-1.02; p=0.08 versus OR 0.94, 95% CI 0.74-1.18; p=0.57).Higher yearly hospital SLB volume was associated with lower post-operative mortality in patients with ILD, with the association appearing to be mainly driven by nonelective cases. SLB mortality was significantly higher for nonelective cases.
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Biópsia/efeitos adversos , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/cirurgia , Pulmão/patologia , Pulmão/cirurgia , Adulto , Biópsia/métodos , Canadá , Coleta de Dados , Procedimentos Cirúrgicos Eletivos , Feminino , Humanos , Seguro Saúde , Tempo de Internação , Doenças Pulmonares Intersticiais/patologia , Masculino , Ontário , Complicações Pós-Operatórias , Período Pós-Operatório , Resultado do TratamentoRESUMO
PURPOSE: International guidelines recommend screening for connective tissue disease (CTD) with autoantibodies when evaluating patients with idiopathic interstitial lung disease (ILD). Idiopathic inflammatory myositis comprises of a subgroup of CTD diagnosed with myositis antibodies (MA), often presenting with ILD. Our aim was to evaluate the utility of MA screening in patients with idiopathic ILD. METHODS: A retrospective analysis was conducted on patients referred with idiopathic ILD to a tertiary centre ILD clinic who were screened for MA. Patients with known or suspected CTD were excluded. Descriptive statistics, univariate analysis and multivariable logistic regression were used to detect associations between MA and patient characteristics. RESULTS: Of 360 patients, 165 met inclusion criteria and 44 (26.7%) were identified to have MA. Fourteen patients (8.5%) had a change in diagnosis as a result of MA screening. Multivariable logistic regression identified the presence of MA to be associated with current smoking [OR 6.87 (1.65-28.64), p = 0.008] and a diffusing capacity of < 70% predicted [OR 2.55 (1.09-5.97), p = 0.03]. In patients with a change in diagnosis due to MA screening, 3 (1.8%) underwent a surgical lung biopsy and 2 (1.2%) were previously treated with antifibrotic therapy. CONCLUSIONS: Screening for MA in patients with idiopathic ILD can contribute to a change in patient diagnosis, and may prevent invasive testing and unproven use of antifibrotic therapy. These results support the addition of MA to CTD screening panels during the initial evaluation of idiopathic ILD.
Assuntos
Autoanticorpos/imunologia , Doenças do Tecido Conjuntivo/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Miosite/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Doenças do Tecido Conjuntivo/imunologia , Feminino , Histidina-tRNA Ligase/imunologia , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/imunologia , Modelos Logísticos , Doenças Pulmonares Intersticiais/imunologia , Masculino , Programas de Rastreamento , Pessoa de Meia-Idade , Análise Multivariada , Miosite/imunologia , Estudos Retrospectivos , Ribonucleoproteínas/imunologiaRESUMO
RATIONALE: Patients commonly use the Internet as a resource for health information; however, no studies have evaluated the online information about idiopathic pulmonary fibrosis (IPF). OBJECTIVES: We sought to determine the readability, content (compared with established guidelines), bias, and quality of online IPF resources. METHODS: We analyzed the first 200 hits for "idiopathic pulmonary fibrosis" in Google, Yahoo, and Bing. Each website was evaluated for content related to IPF features and treatments that are discussed in clinical guidelines. Website quality was assessed using the validated DISCERN instrument. MEASUREMENTS AND MAIN RESULTS: Eligibility criteria were met in 181 websites. The median reading grade level was 12. More content was provided in scientific resources (academic institutions or governmental organizations) and foundation/advocacy organization sites than in personal commentary (blog) sites; however, most sites provided incomplete and/or inaccurate information. Nonindicated and/or harmful pharmacotherapies for IPF were described as potential IPF treatments in 48% of websites and were most often recommended in foundation/advocacy organization websites. Azathioprine and corticosteroids were discussed as potential chronic treatments of IPF in 13.3 and 30.6% of the 98 websites that had been updated after publication of data demonstrating harm from these medications. Website quality (DISCERN score) was poor in all site types but was worse in news/media reports and personal commentary (blog) sites than in sites from scientific and foundation/advocacy organizations. CONCLUSIONS: Patient-directed online information on IPF is frequently incomplete, inaccurate, and outdated. There is no reliable method for patients to identify sites that provide appropriate information on IPF.