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Background: The P2Y12 receptor inhibitors clopidogrel and prasugrel are widely used. Clopidogrel and prasugrel have different metabolic pathways, but whether their adverse event (AE) profiles differ significantly is unclear. Objective: This study aimed to compare the possible AEs induced by clopidogrel and prasugrel and to assess the rank-order of their AEs submitted to a spontaneous reporting database. Materials and Methods: Data were extracted from the Japanese Adverse Drug Event Report database (JADER). Reports of AEs associated with clopidogrel and prasugrel were analyzed to calculate the reporting odds ratios (RORs) and 95% confidence intervals (CIs). Results: Based on 5869 reports for clopidogrel (69.6%, men) and 513 reports for prasugrel (74.1%, men), 703 and 135 different AEs were identified, respectively. Bleeding complications including hemorrhage were commonly reported for both clopidogrel and prasugrel. As for AEs related to clopidogrel, unexpected AEs such as interstitial lung disease (227 reports; ROR, 1.77; 95% CI, 1.49-2.10), abnormal hepatic function (137 reports; ROR, 1.27; 95% CI, 1.07-1.51), and hepatocellular injury (96 reports; ROR, 120.0; 95% CI, 94.9-151.8) ranked at relatively high positions based on the number of occurrences, unlike prasugrel. Conclusion: This analysis of the national pharmacovigilance database highlights distinct AE profiles for clopidogrel and prasugrel. Unexpected AEs associated with clopidogrel were identified, providing valuable insights for clinical monitoring and patient safety.
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Sistemas de Registro de Reacción Adversa a Medicamentos , Clopidogrel , Enfermedades Pulmonares Intersticiales , Farmacovigilancia , Inhibidores de Agregación Plaquetaria , Clorhidrato de Prasugrel , Antagonistas del Receptor Purinérgico P2Y , Humanos , Clopidogrel/efectos adversos , Clorhidrato de Prasugrel/efectos adversos , Clorhidrato de Prasugrel/uso terapéutico , Japón/epidemiología , Enfermedades Pulmonares Intersticiales/inducido químicamente , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/epidemiología , Masculino , Inhibidores de Agregación Plaquetaria/efectos adversos , Femenino , Factores de Riesgo , Antagonistas del Receptor Purinérgico P2Y/efectos adversos , Persona de Mediana Edad , Anciano , Bases de Datos Factuales , Medición de Riesgo , Hemorragia/inducido químicamente , Hemorragia/epidemiología , Adulto , Pueblos del Este de AsiaRESUMEN
BACKGROUND: Cough remains a persistent symptom in patients with idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs). To inform future research, treatment and care models, we conducted the first systematic synthesis of evidence on its associated burden. METHODS: A literature search was performed for articles published between January 2010 and October 2023 using databases including Embase, MEDLINE and the Cochrane Library. Studies in patients with IPF and other ILDs reporting cough-related measures were eligible for inclusion. Included studies were categorised based on the types of ILD they examined and their design. Study details, patient characteristics and outcomes were extracted, and the risk of bias was assessed. A narrative synthesis approach was employed to interpret the findings. RESULTS: Sixty-one studies were included: 33 in IPF, 18 in mixed-ILDs, six in connective tissue disease-associated-ILDs and four in sarcoidosis. Across the studies, a range of tools to assess cough and its impact were used. The most frequently used measures of cough were cough severity visual analogue scale (VAS) and objective cough counts, whereas the most frequently used health-related quality of life (HRQoL)/impact measures were the St. George's Respiratory Questionnaire (SGRQ) and Leicester Cough Questionnaire (LCQ). In IPF, studies consistently reported correlations between various cough and HRQoL measures, including between cough VAS scores and objective cough counts, LCQ scores and SGRQ scores. Similar correlations were observed in studies in other ILDs, but data were more limited. Qualitative studies in both IPF and other ILDs consistently highlighted the significant cough-related burden experienced by patients, including disruption of daily activities, fatigue and social embarrassment. Although there were no studies specifically investigating the economic burden of cough, one study in patients with fibrotic ILD found cough severity was associated with workplace productivity loss. CONCLUSIONS: Our study underscores the heterogeneity in assessing cough and its impact in IPF and other ILDs. The findings confirm the negative impact of cough on HRQoL in IPF and suggest a comparable impact in other ILDs. Our synthesis highlights the need for standardised assessment tools, along with dedicated studies, particularly in non-IPF ILDs and on the economic burden of cough.
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Costo de Enfermedad , Tos , Fibrosis Pulmonar Idiopática , Enfermedades Pulmonares Intersticiales , Humanos , Tos/diagnóstico , Tos/fisiopatología , Tos/epidemiología , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/epidemiología , Fibrosis Pulmonar Idiopática/fisiopatología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Calidad de VidaRESUMEN
BACKGROUND: Not all chronic diseases have clear pathways and time targets for diagnosis. We explored pathways and timings for four major chronic respiratory diseases in England. METHODS: Using deidentified electronic healthcare records from Clinical Practice Research Datalink Aurum linked to Hospital Episode Statistics, we derived cohorts of patients diagnosed with asthma, chronic obstructive pulmonary disease (COPD), ILD or bronchiectasis at three time periods (2008/2009, 2018/2019 and 2020/2021). We followed people 2 years before and 2 years after diagnosis, calculating the proportion of people who presented with symptoms, underwent diagnostic tests, were treated and consulted healthcare (primary or secondary) and calculated time intervals between events. We repeated analyses by socioeconomic status and geographical region. RESULTS: We descriptively studied patient pathways for 429 619 individuals across all time frames and diseases. Most people (>87%) had first evidence of diagnosis in primary care. The proportion of people reporting symptoms prior to diagnosis was similar for asthma, COPD and ILD (41.0%-57.9%) and higher in bronchiectasis (67.9%-71.8%). The proportion undergoing diagnostic tests was high for COPD and bronchiectasis (77.6%-89.2%) and lower for asthma (14%-32.7%) and ILD (2.6%-3.3%). The proportion of people undergoing diagnostic tests decreased in 2020/2021 for all diseases, mostly COPD. Time (months) (median (IQR)) between symptoms and diagnosis, averaged over three time periods, was lowest in asthma (~7.5 (1.3-16.0)), followed by COPD (~8.6 (1.8-17.2)), ILD (~10.1 (3.6-18.0)) and bronchiectasis (~13.5 (5.9-19.8)). Time from symptoms to diagnosis increased by ~2 months in asthma and COPD over the three time periods. Although most patients were symptomatically treated prior to diagnosis, time between diagnosis and postdiagnostic treatment was around 4 months for ILD, 3 months for bronchiectasis and instantaneous for asthma and COPD. Socioeconomic status and regional trends showed little disparity. CONCLUSION: Current pathways demonstrate missed opportunities to diagnose and manage disease and to improve disease coding.
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Asma , Bronquiectasia , Enfermedad Pulmonar Obstructiva Crónica , Humanos , Inglaterra/epidemiología , Masculino , Femenino , Asma/epidemiología , Asma/diagnóstico , Asma/terapia , Bronquiectasia/epidemiología , Bronquiectasia/diagnóstico , Bronquiectasia/terapia , Anciano , Persona de Mediana Edad , Enfermedad Pulmonar Obstructiva Crónica/epidemiología , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Enfermedad Pulmonar Obstructiva Crónica/terapia , Adulto , Enfermedad Crónica , Atención Primaria de Salud/estadística & datos numéricos , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/terapia , Adulto Joven , Vías Clínicas , Adolescente , Anciano de 80 o más Años , Registros Electrónicos de Salud/estadística & datos numéricos , Factores de TiempoRESUMEN
BACKGROUND: Progressive pulmonary fibrosis is the symptomatic, physiological, and radiological progression of interstitial lung diseases. The aim of this study was to examine the relationship between progressive pulmonary fibrosis and demographic characteristics and to evaluate the effect on clinical outcomes and mortality. METHODS: This cross-sectional study included 221 patients diagnosed with non-idiopathic pulmonary fibrosis interstitial lung diseases who were followed in the last 5 years. Patient symptoms, clinical, radiological, and demographic data were examined. Risk factors for the development of progressive pulmonary fibrosis and the relationship with clinical outcomes and mortality were examined. RESULTS: Of the patients, 33.0% (n = 73) had fibrotic idiopathic nonspecific interstitial pneumonia (iNSIP), 35.7% (n = 79) had fibrotic hypersensitivity pneumonia (HP), 18.1% (n = 40) had fibrotic connective tissue disease (CTD) interstitial lung diseases (ILD), and 13.1% (n = 29) had postinfectious fibrotic ILD. The progressive pulmonary fibrosis development rates of the subtypes were 46.5% iNSIP (n = 34), 86.0% fibrotic HP (n = 68), 42.5% fibrotic CTD-ILD (n = 17), and 20.7% postinfectious ILD (n = 6). The presence of progressive pulmonary fibrosis was associated with the development of respiratory failure and mortality (odds ratio [OR]: 2.70, 95% CI: 1.04-7.05 and OR: 2.13, 95% CI: 1.23-3.69). Progressive pulmonary fibrosis development was higher in hypersensitivity pneumonia patients with farmer's lung (OR: 5.06, 95% CI: 1.02-25.18). CONCLUSION: Progressive pulmonary fibrosis was more prevalent in older patients. Farming was an important risk factor in the development of hypersensitivity pneumonia-progressive pulmonary fibrosis. Respiratory failure and mortality were higher in those who developed progressive pulmonary fibrosis.
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Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales , Fibrosis Pulmonar , Humanos , Estudios Transversales , Masculino , Femenino , Persona de Mediana Edad , Anciano , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/epidemiología , Fibrosis Pulmonar/epidemiología , Fibrosis Pulmonar/complicaciones , Fibrosis Pulmonar/mortalidad , Factores de Riesgo , Alveolitis Alérgica Extrínseca/complicaciones , Alveolitis Alérgica Extrínseca/patología , Alveolitis Alérgica Extrínseca/epidemiología , Fibrosis Pulmonar Idiopática/complicaciones , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/epidemiología , Fibrosis Pulmonar Idiopática/patología , Adulto , Enfermedades del Tejido Conjuntivo/complicacionesRESUMEN
PURPOSE OF REVIEW: To provide timely and relevant insights into the complex relationship between pulmonary vascular disease (PVD) and chronic lung disease (CLD), focusing on the causative and consequential dynamics between these conditions. RECENT FINDINGS: There are shared pathogenic mechanisms between pulmonary arterial hypertension (PAH) and group 3 pulmonary hypertension, including altered expression of mediators and growth factors implicated in both conditions. Factors such as hypoxia, hypoxemia, and hypercapnia also contribute to pulmonary vascular remodelling and endothelial dysfunction. However, the role of hypoxia as the sole driver of pulmonary hypertension in CLD is being reconsidered, particularly in chronic obstructive pulmonary disease (COPD), with evidence suggesting a potential role for cigarette smoke products in initiating pulmonary vascular impairment. On the other hand, interstitial lung disease (ILD) encompasses a group of heterogeneous lung disorders characterized by inflammation and fibrosis of the interstitium, leading to impaired gas exchange and progressive respiratory decline, which could also play a role as a cause of pulmonary hypertension. SUMMARY: Understanding the intricate interplay between the pulmonary vascular compartment and the parenchymal and airway compartments in respiratory disease is crucial for developing effective diagnostic and therapeutic strategies for patients with PVD and CLD, with implications for both clinical practice and research.
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Hipertensión Pulmonar , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/fisiopatología , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/epidemiología , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/epidemiología , Hipertensión Pulmonar/fisiopatología , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Enfermedad Pulmonar Obstructiva Crónica/epidemiología , Enfermedad Pulmonar Obstructiva Crónica/complicaciones , Comorbilidad , Enfermedad Crónica , Hipoxia/fisiopatología , Enfermedades Pulmonares/fisiopatología , Enfermedades Pulmonares/epidemiología , Enfermedades Pulmonares/etiología , Remodelación Vascular/fisiología , Enfermedades Vasculares/fisiopatología , Enfermedades Vasculares/epidemiología , Enfermedades Vasculares/etiología , Enfermedades Vasculares/complicacionesRESUMEN
INTRODUCTION: Interstitial lung disease in children (chILD) are rare and mostly severe lung diseases. Very few epidemiological data are available in limited series of patients. The aim of this study was to assess the prevalence and incidence of chILD in France. METHODS: We performed within the RespiRare network a multicentre retrospective observational study in patients with chILD from 2000 to 2022 and a prospective evaluation of chILD's incidence between February 2022 and 2023. RESULTS: chILD was reported in 790 patients in 42 centres. The estimated 2022 prevalence in France was 44 /million children (95% CI 40.76 to 47.46) and the computed incidence was 4.4 /million children (95% CI 3.44 to 5.56). The median age at diagnosis was 3 months with 16.9% of familial forms. Lung biopsy and genetic analyses were performed in 23.4% and 76.9%, respectively. The most frequent chILD aetiologies in the <2 years group were surfactant metabolism disorders (16.3%) and neuroendocrine cell hyperplasia of infancy (11.8%), and in the 2-18 years group diffuse alveolar haemorrhage (12.2%), connective tissue diseases (11.4%), hypersensitivity pneumonitis (8.8%) and sarcoidosis (8.8%). The management included mainly oxygen therapy (52%), corticosteroid pulses (56%), oral corticosteroids (44%), azithromycin (27.2%), enteral nutrition (26.9%), immunosuppressants (20.3%) and hydroxychloroquine (15.9%). The 5-year survival rate was 57.3% for the patients diagnosed before 2 years and 86% between 2 and 18 years. CONCLUSION: This large and systematic epidemiological study confirms a higher incidence and prevalence of chILD than previously described. In order to develop international studies, efforts are still needed to optimise the case collection and to harmonise diagnostic and management practices.
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Enfermedades Pulmonares Intersticiales , Humanos , Francia/epidemiología , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/terapia , Femenino , Masculino , Niño , Preescolar , Adolescente , Incidencia , Estudios Retrospectivos , Lactante , Prevalencia , Estudios ProspectivosRESUMEN
A variety of workplace exposures (organic or inorganic dusts as well as gases, fumes, or vapors) can cause diffuse interstitial lung disease. The latency period until onset of the disease can exceed 30 years. The disease course varies greatly and depends on the quantity of the inhaled substance and its fibrogenic effect. Pulmonary high-resolution computed tomography (HRCT) patterns do not differ significantly from those of interstitial lung diseases (ILD) of other etiologies. Therefore, without knowledge of the occupational history, work-related ILDs are often classified as idiopathic. In addition, there is increasing evidence in the recent literature that high exposure to silica dust can trigger autoimmune diseases (also involving the lungs). For this reason, a qualified occupational history is now an indispensable part of the interdisciplinary diagnosis of ILDs.
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Enfermedades Pulmonares Intersticiales , Enfermedades Profesionales , Humanos , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Profesionales/etiología , Enfermedades Profesionales/diagnóstico , Tomografía Computarizada por Rayos X , Exposición Profesional/efectos adversosRESUMEN
INTRODUCTION: Interstitial lung disease (ILD) is a prevalent complication in patients with common variable immunodeficiency (CVID) and is often related to other characteristics such as bronchiectasis and autoimmunity. Because the term ILD encompasses a variety of acute and chronic pulmonary conditions, diagnosis is usually based on imaging features. Histopathology is less available. This study was conducted with the aim of investigating the ILD in patients with CVID. MATERIALS AND METHODS: In this retrospective cross-sectional study, sixty CVID patients who referred to the pulmonology and immunodeficiency clinics of Mofid Children's Hospital between 2013 and 2022 were included. The diagnosis of ILD were based on transbronchial lung biopsy (TBB) or clinical and radiological symptoms. The prevalence of ILD in CVID patients was determined. Also, the CVID patients with and without ILD were compared in terms of demographic characteristics, clinical, laboratory and radiologic findings. RESULTS: Among all patients, ten patients had ILD (16.6%). In terms of laboratory parameters, there was a significant difference between platelets in the two groups of CVID patients with and without ILD, and the level of platelets was higher in the group of patients with ILD. Moreover, in terms of clinical symptoms, pneumonia, diarrhea and hepatomegaly were significantly different between the two groups and were statistically higher in the group of patients with ILD (P < 0.05). Autoimmunity and malignancy were not significantly different in two groups. There was a significant difference in, hyperinflation between the two groups of CVID patients with and without ILD, and the frequency of, hyperinflation was higher in the patients without ILD (P = 0.040). CONCLUSION: Understanding the pathogenesis of ILD plays an essential role in revealing non-infectious pulmonary complications that occur in CVID patients. Increasing efforts to understand ILD not only shed light on its hidden pathogenesis and clinical features, but also enhance our understanding of CVID in a broader sense.
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Inmunodeficiencia Variable Común , Enfermedades Pulmonares Intersticiales , Humanos , Inmunodeficiencia Variable Común/complicaciones , Inmunodeficiencia Variable Común/epidemiología , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/epidemiología , Femenino , Masculino , Estudios Transversales , Estudios Retrospectivos , Irán/epidemiología , Adulto , Adolescente , Niño , Adulto Joven , Prevalencia , Pulmón/patología , Pulmón/diagnóstico por imagen , Persona de Mediana EdadRESUMEN
BACKGROUND: Organic dust is associated with hypersensitivity pneumonitis, and associations with other types of interstitial lung disease (ILD) have been suggested. We examined the association between occupational organic dust exposure and hypersensitivity pneumonitis and other ILDs in a cohort study. METHODS: The study population included all residents of Denmark born in 1956 or later with at least 1 year of gainful employment since 1976. Incident cases of hypersensitivity pneumonitis and other ILDs were identified in the Danish National Patient Register 1994-2015. Job exposure matrices were used to assign individual annual levels of exposure to organic dust, endotoxin and wood dust from 1976 to 2015. We analysed exposure-response relations by different exposure metrics using a discrete-time hazard model. RESULTS: For organic dust, we observed increasing risk with increasing cumulative exposure with incidence rate ratios (IRR) per 10 unit-years of 1.19 (95% CI 1.12 to 1.27) for hypersensitivity pneumonitis and 1.04 (95% CI 1.02 to 1.06) for other ILDs. We found increasing risk with increasing cumulative endotoxin exposure for hypersensitivity pneumonitis and other ILDs with IRRs per 5000 endotoxin units/m3-years of 1.55 (95% CI 1.38 to 1.73) and 1.09 (95% CI 1.00 to 1.19), respectively. For both exposures, risk also increased with increasing duration of exposure and recent exposure. No increased risks were observed for wood dust exposure. CONCLUSION: Exposure-response relations were observed between organic dust and endotoxin exposure and hypersensitivity pneumonitis and other ILDs, with lower risk estimates for the latter. The findings indicate that organic dust should be considered a possible cause of any ILD. TRIAL REGISTRATION NUMBER: j.no.: 1-16-02-196-17.
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Alveolitis Alérgica Extrínseca , Polvo , Enfermedades Pulmonares Intersticiales , Enfermedades Profesionales , Exposición Profesional , Humanos , Alveolitis Alérgica Extrínseca/epidemiología , Alveolitis Alérgica Extrínseca/etiología , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/etiología , Exposición Profesional/efectos adversos , Dinamarca/epidemiología , Masculino , Femenino , Persona de Mediana Edad , Enfermedades Profesionales/epidemiología , Enfermedades Profesionales/etiología , Incidencia , Adulto , Endotoxinas/efectos adversos , Endotoxinas/análisis , Factores de RiesgoRESUMEN
OBJECTIVE: The objective of this study is to determine the associations of protein-specific anti-malondialdehyde-acetaldehyde (MAA) antibodies with prevalent and incident rheumatoid arthritis-interstitial lung disease (RA-ILD). METHODS: Within a multicenter, prospective cohort of US veterans with RA, RA-ILD was validated by medical record review of clinical diagnoses, chest imaging, and pathology. Serum antibodies to MAA-albumin, MAA-collagen, MAA-fibrinogen, and MAA-vimentin (IgA, IgM, and IgG) were measured by a standardized enzyme-linked immunosorbent assay. Associations of anti-MAA antibodies with prevalent and incident RA-ILD were assessed using multivariable regression models adjusting for established RA-ILD risk factors. RESULTS: Among 2,739 participants with RA (88% male, mean age of 64 years), there were 114 with prevalent and 136 with incident RA-ILD (average time to diagnosis: 6.6 years). Higher IgM anti-MAA-collagen (per 1 SD: adjusted odds ratio [aOR] 1.28, 95% confidence interval [CI] 1.02-1.61), IgA anti-MAA-fibrinogen (aOR 1.48, 95% CI 1.14-1.92), and IgA (aOR 1.78, 95% CI 1.34-2.37) and IgG (aOR 1.48, 95% CI 1.14-1.92) anti-MAA-vimentin antibodies were associated with prevalent RA-ILD. In incident analyses, higher IgA (per one SD: adjusted hazards ratio [aHR] 1.40, 95% CI 1.11-1.76) and IgM (aHR 1.29, 95% CI 1.04-1.60) anti-MAA-albumin antibody concentrations were associated with increased ILD risk. Participants with IgA (aHR 2.13, 95% CI 1.16-3.90) or IgM (aHR 1.98, 95% CI 1.08-3.64) anti-MAA-albumin antibody concentrations in the highest quartile had an approximately two-fold increased risk of incident RA-ILD. Across all isotypes, anti-MAA-fibrinogen, anti-MAA-collagen, and anti-MAA-vimentin antibodies were not significantly associated with incident RA-ILD. CONCLUSION: Protein-specific anti-MAA antibodies to collagen, fibrinogen, and vimentin were associated with prevalent RA-ILD. IgA and IgM anti-MAA-albumin antibodies were associated with a higher risk of incident RA-ILD. These findings suggest that MAA modifications and resultant immune responses may contribute to RA-ILD pathogenesis.
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Acetaldehído , Artritis Reumatoide , Autoanticuerpos , Enfermedades Pulmonares Intersticiales , Veteranos , Humanos , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/epidemiología , Masculino , Persona de Mediana Edad , Femenino , Artritis Reumatoide/inmunología , Artritis Reumatoide/epidemiología , Anciano , Estados Unidos/epidemiología , Estudios Prospectivos , Acetaldehído/inmunología , Autoanticuerpos/inmunología , Autoanticuerpos/sangre , Incidencia , Prevalencia , Fibrinógeno/inmunología , Inmunoglobulina M/inmunología , Vimentina/inmunología , Inmunoglobulina A/inmunología , Inmunoglobulina A/sangre , Inmunoglobulina G/inmunología , Inmunoglobulina G/sangre , Colágeno/inmunología , Albúmina Sérica/inmunologíaRESUMEN
BACKGROUND: The purpose of this study is to analyze the distribution of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) in patients with idiopathic inflammatory myopathies (IIMs) in southwest China and to explore the relevance between each subtype, each clinical feature, and to explore the relevance between the laboratory indexes. METHODS: For this study, 200 patients with IIMs were tested for myositis autoantibodies. Clinical manifestations and laboratory metrics were collected and the correlations between autoantibodies and clinical phenotypes were analyzed. RESULTS: MSAs were found in 73.5% of the patients. The most frequently MSAs were anti-MDA5 (26.8%), followed by anti-ARS (18.5%). Anti-Ro52 was the most prevalent in MAAs (46.2%). Interstitial lung disease (ILD) and arthralgia were more frequent in anti-MDA5 and anti-Jo-1 positive groups (each p < 0.05). Anti-TIF1-γ and anti-NXP2 were associated with dysphagia (each p < 0.05). Different antibody subtypes were associated with laboratory indicators of response to muscle damage and immune status. Logistic regression showed that anti-MDA5 and anti-Jo-1 were independent risk factors for ILD (OR = 4.542, p = 0.004; OR = 4.290, p = 0.018, respectively) and arthralgia (OR = 7.856, p = 0.000; OR = 5.731, p = 0.004, respectively), whereas anti-TIF1-γ and anti-NXP2 were independent risk factors for dysphagia (OR = 4.521, p = 0.009; OR = 6.889, p = 0.017, respectively). CONCLUSIONS: Different antibody subtypes were associated with specific clinical features. Anti-MDA5 and anti-Jo-1 were independent risk factors for ILD and arthralgia. Anti-TIF1-γ and anti-NXP2 were independent risk factors for dysphagia.
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Autoanticuerpos , Miositis , Humanos , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Miositis/inmunología , Miositis/sangre , Miositis/epidemiología , Miositis/diagnóstico , Femenino , Masculino , China/epidemiología , Persona de Mediana Edad , Adulto , Helicasa Inducida por Interferón IFIH1/inmunología , Anciano , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/sangre , Relevancia ClínicaRESUMEN
Objective: To analyze the clinical characteristics of primary Sjögren's syndrome (pSS) combined with interstitial lung disease (ILD), so as to provide a theoretical basis for the early diagnosis, treatment and prevention of PSS-ILD. Methods: From October 2017 to January 2022, patients with pSS who were admitted to the Department of Rheumatology at Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine were included in this retrospective study. Patients were divided into the pSS-ILD (102 cases) and pSS-non-ILD groups (154 cases) based on the presence or absence of ILD on high-resolution computed tomography (HRCT). Demographics information, clinical symptoms, laboratory indicators and HRCT features were compared, and the logistic regression analysis was utilized to identify the risk factors. Results: A total of 256 patients were included. Patients with pSS-ILD were more often female, and their age and disease duration were significantly higher than those in the pSS-non-ILD group (p < 0.05). The HRCT imaging classification included ground glass-like shadow (78.4%) and patchy solid shadow (17.6%), and Non-specific interstitial pneumonitis (NSIP) (72.5%) was the predominant typology. Regarding the laboratory indexes, the positive rates of erythrocyte sedimentation rate, C-reactive protein, white blood cell count, neutrophil/lymphocyte ratio, triglycerides, total cholesterol, and anti-SS-A52 antibodies were significantly higher in the pSS-ILD patients than in the pSS-non-ILD group, while the positive rates of anti-synaptic antibodies were lower than in the pSS-non-ILD group, and the differences between two groups were statistically significant (p < 0.05). Logistic regression showed that age >60 years, longer duration of disease, higher triglycerides, and cholesterol were risk factors for pSS-ILD patients. Conclusion: The clinical features of pSS-ILD patients were xerophthalmia, cough and shortness of breath, and HRCT can help to diagnose the disease at an early stage. Age over 60 years, chronic course of disease, and elevated lipid levels are risk factors for ILD in pSS patients, and the relationship between autoimmune antibody levels and the occurrence of ILD needs to be further confirmed in follow-up studies with large sample sizes. These findings have the potential to provide useful information for early diagnosis, treatment, and prevention of the development of pSS-ILD.
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Enfermedades Pulmonares Intersticiales , Síndrome de Sjögren , Tomografía Computarizada por Rayos X , Humanos , Síndrome de Sjögren/complicaciones , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/complicaciones , Femenino , Estudios Retrospectivos , Masculino , Persona de Mediana Edad , Factores de Riesgo , Adulto , Anciano , China/epidemiologíaRESUMEN
PURPOSE: World Trade Center (WTC) exposure is associated with obstructive airway diseases and sarcoidosis. There is limited research regarding the incidence and progression of non-sarcoidosis interstitial lung diseases (ILD) after WTC-exposure. ILD encompasses parenchymal diseases which may lead to progressive pulmonary fibrosis (PPF). We used the Fire Department of the City of New York's (FDNY's) WTC Health Program cohort to estimate ILD incidence and progression. METHODS: This longitudinal study included 14,525 responders without ILD prior to 9/11/2001. ILD incidence and prevalence were estimated and standardized to the US 2014 population. Poisson regression modeled risk factors, including WTC-exposure and forced vital capacity (FVC), associated with ILD. Follow-up time ended at the earliest of incident diagnosis, end of study period/case ascertainment, transplant or death. RESULTS: ILD developed in 80/14,525 FDNY WTC responders. Age, smoking, and gastroesophageal reflux disease (GERD) prior to diagnosis were associated with incident ILD, though FVC was not. PPF developed in 40/80 ILD cases. Among the 80 cases, the average follow-up time after ILD diagnosis was 8.5 years with the majority of deaths occurring among those with PPF (PPF: n = 13; ILD without PPF: n = 6). CONCLUSIONS: The prevalence of post-9/11 ILD was more than two-fold greater than the general population. An exposure-response gradient could not be demonstrated. Half the ILD cases developed PPF, higher than previously reported. Age, smoking, and GERD were risk factors for ILD and PPF, while lung function was not. This may indicate that lung function measured after respirable exposures would not identify those at risk for ILD or PPF.
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Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales , Fibrosis Pulmonar , Ataques Terroristas del 11 de Septiembre , Humanos , Estudios Longitudinales , Masculino , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Persona de Mediana Edad , Femenino , Incidencia , Capacidad Vital , Adulto , Prevalencia , Factores de Riesgo , Fibrosis Pulmonar/epidemiología , Fibrosis Pulmonar/fisiopatología , Ciudad de Nueva York/epidemiología , Reflujo Gastroesofágico/epidemiología , Exposición Profesional/efectos adversos , Fumar/efectos adversos , Fumar/epidemiología , Anciano , Factores de Tiempo , Socorristas/estadística & datos numéricosRESUMEN
BACKGROUND: Interstitial lung diseases (ILDs) are a heterogeneous group of disorders, a subset of which develop progressive pulmonary fibrosis (PPF). There is little information on the epidemiology and treatment of PPFs in Japan. This retrospective cohort study estimated the incidence probability of progression to PPFs in patients with fibrosing ILDs other than idiopathic pulmonary fibrosis in a real-world Japanese setting. Management procedures and treatment patterns were also quantified. METHODS: Data were extracted from the Medical Data Vision database from 01-Jan-2012 to 28-May-2020, comprising a 6.91-year patient identification period, 1-year pre-index period, and post-index period. The primary outcome was the cumulative incidence probability of progression to PPF up to 24 months. Subgroup analyses were performed by the presence/absence of connective tissue disease-ILD and by pre-specified ILD clinical diagnosis. RESULTS: Of the 34,960 eligible patients (mean age: 71.1 years, males: 52.5%), 14,580 (41.7%) progressed to PPF. The 24-month incidence probability of progression to PPF was 39.5%. A relatively comparable percentage of patients progressed across all ILD subtypes. Oral corticosteroids and tacrolimus were the most common therapies during the pre- and post-index periods. Treatment rates were very low in the post-index period. CONCLUSIONS: This is the first claims database study to estimate the incidence probability of progression to PPF in Japan. Progression appeared common in patients with chronic fibrosing ILDs, with comparable percentages of patients across all subtypes developing PPF at 2 years. Future studies should assess the impact of regular monitoring and early intervention on treating fibrotic ILDs and preventing progression.
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Bases de Datos Factuales , Progresión de la Enfermedad , Fibrosis Pulmonar , Humanos , Japón/epidemiología , Anciano , Masculino , Incidencia , Femenino , Fibrosis Pulmonar/epidemiología , Fibrosis Pulmonar/terapia , Estudios Retrospectivos , Persona de Mediana Edad , Tacrolimus/administración & dosificación , Corticoesteroides/administración & dosificación , Corticoesteroides/uso terapéutico , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/terapia , Estudios de Cohortes , Anciano de 80 o más AñosRESUMEN
BACKGROUND: Interstitial lung diseases (ILD) are a heterogenous group of over 200 disorders affecting the pulmonary interstitium. Although there have been advances in knowledge on ILDs in Australia, the characterisation of the health and economic burden of disease remained largely undetermined until recently. OBJECTIVE: The main objective of this review is to provide a synopsis of health and economic burden of ILDs in Australia, based on recently completed research. DISCUSSION: Recent research has demonstrated that idiopathic pulmonary fibrosis (IPF) is the most frequent ILD in Australia. Incidence and prevalence of IPF have demonstrated an increasing trend over the past decades. Mortality has also increased over the past decades, but has shown a slight decreasing trend recently, since the introduction of antifibrotic medication. Health-related quality of life is poor in patients with IPF, and care is estimated to cost approximately AU$299 million per year in Australia. Early diagnosis and referral to tertiary care is crucial for favourable outcomes, and general practitioners are considerably important to this as the first interface to identify patients at risk and detect early symptoms of ILDs.
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Costo de Enfermedad , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/economía , Enfermedades Pulmonares Intersticiales/terapia , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Australia/epidemiología , Calidad de Vida/psicología , Prevalencia , Adulto , Fibrosis Pulmonar Idiopática/economía , Fibrosis Pulmonar Idiopática/epidemiología , Fibrosis Pulmonar Idiopática/terapia , IncidenciaRESUMEN
Interstitial lung disease (ILD) encompasses a heterogeneous group of more than 200 diffuse parenchymal lung diseases with various clinical courses. Disease progression is one of the most important prognostic factors, and, the definition of progressive pulmonary fibrosis (PPF) has recently been established. This study aimed to estimate the prevalence, risk factors, and prognosis of PPF among patients with non-idiopathic pulmonary fibrosis (IPF) in real-world practice. A total of 215 patients were retrospectively analyzed between January 2010 and June 2023 at the Haeundae Paik Hospital in the Republic of Korea. According to the criteria proposed in 2022 by Raghu et al, PPF defined as a condition that satisfies 2 or more of the following in the past year: worsening of respiratory symptoms, physiological evidence of disease progression, and radiological evidence of disease progression. The median age of the subjects was 67 years and 63.7% were female. A total of 40% was diagnosed with PPF and connective tissue disease-associated ILD (52.3%) was the most common type, followed by nonspecific interstitial pneumonitis (NSIP) (25.6%) and cryptogenic organizing pneumonitis (16.3%). In multivariate logistic regression for predicting PPF, both the use of steroids and immunosuppressants (OR: 2.57, 95% CI: 1.41-4.67, Pâ =â .002) and home oxygen use (OR: 25.17, 95% CI: 3.21-197.24, Pâ =â .002) were independent risk factors. During the follow-up period, the mortality rate was significantly higher in the PPF group than in the non-PPF group (24.4% vs 2.3%, Pâ <â .001). In the survival analysis using the Cox proportional hazard regression model, disease progression, older age and lower forced vital capacity (FVC) were independent risk factors for mortality. Our study demonstrated that the prevalence of PPF was 40%. Concomitant therapy of steroids with an immunosuppressants and home oxygen use are risk factors for PPF. PPF itself was significantly associated with high mortality rates. Risk factors for mortality were disease progression, older age, and lower FVC.
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Progresión de la Enfermedad , Humanos , Femenino , Masculino , Estudios Retrospectivos , Anciano , Prevalencia , República de Corea/epidemiología , Pronóstico , Persona de Mediana Edad , Factores de Riesgo , Fibrosis Pulmonar/epidemiología , Fibrosis Pulmonar/mortalidad , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Inmunosupresores/uso terapéuticoRESUMEN
RATIONALE: The proportion of patients who develop progressive pulmonary fibrosis (PPF), along with risk factors for progression remain poorly understood. OBJECTIVES: To examine factors associated with an increased risk of developing PPF among patients at a referral center. METHODS: We identified patients with a diagnosis of interstitial lung disease (ILD) seen within the Cleveland Clinic Health System. Utilizing a retrospective observational approach we estimated the risk of developing progression by diagnosis group and identified key clinical predictors using the FVC component of both the original progressive fibrotic interstitial lung disease (PFILD) and the proposed PPF (ATS) criteria. RESULTS: We identified 5934 patients with a diagnosis of ILD. The cumulative incidence of progression over the 24 months was similar when assessed with the PFILD and PPF criteria (33.1 % and 37.9 % respectively). Of those who met the ATS criteria, 9.5 % did not meet the PFILD criteria. Conversely, 4.3 % of patients who met PFILD thresholds did not achieve the 5 % absolute FVC decline criteria. Significant differences in the rate of progression were seen based on underlying diagnosis. Steroid therapy (HR 1.46, CI 1.31-1.62) was associated with an increased risk of progressive fibrosis by both PFILD and PPF criteria. CONCLUSION: Regardless of the definition used, the cumulative incidence of progressive disease is high in patients with ILD in the 24 months following diagnosis. Some differences are seen in the risk of progression when assessed by PFILD and PPF criteria. Further work is needed to identify modifiable risk factors for the development of progressive fibrosis.
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Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/fisiopatología , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/complicaciones , Masculino , Femenino , Estudios Retrospectivos , Capacidad Vital/fisiología , Persona de Mediana Edad , Anciano , Factores de Riesgo , Fibrosis Pulmonar/fisiopatología , Fibrosis Pulmonar/complicaciones , Fibrosis Pulmonar/epidemiología , IncidenciaRESUMEN
Background/aim: The objective of this study is to evaluate the clinical presentations and adverse outcomes of Coronavirus Disease 2019 (COVID-19) in patients with systemic sclerosis (SSc) and assess the impact of SSc features on the clinical course of COVID-19. Materials and methods: In this multicenter, retrospective study, SSc patients with COVID-19 were included. Clinical features of SSc, along with detailed COVID-19 data, were extracted from medical records and patient interviews. Results: The study included 112 patients (mean age 51.4 ± 12.8 years; 90.2% female). SSc-associated interstitial lung disease (ILD) was evident in 57.1% of the patients. The findings revealed hospitalization in 25.5%, respiratory support in 16.3%, intensive care unit admission in 3.6%, and a mortality rate of 2.7% among SSc patients with COVID-19. Risk factors for respiratory failure, identified through univariate analysis, included ILD (OR: 7.49, 95% CI: 1.63-34.46), ≥1 comorbidity (OR: 4.55, 95% CI: 1.39-14.88), a higher physician global assessment score at the last outpatient visit (OR 2.73, 95% CI: 1.22-6.10), and the use of mycophenolate at the time of infection (OR: 5.16, 95 %CI: 1.79-14.99). Notably, ≥1 comorbidity emerged as the sole significant predictor of the need for respiratory support in COVID-19 (OR: 5.78, 95% CI: 1.14-29.23). In the early post-COVID-19 period, 17% of patients reported the progression of the Raynaud phenomenon, and 10.6% developed new digital ulcers. Furthermore, progression or new onset of dyspnea and cough were detected in 28.3% and 11.4% of patients, respectively. Conclusion: This study suggests a potential association between adverse outcomes of COVID-19 and SSc-related ILD, severe disease activity, and the use of mycophenolate. Additionally, it highlights that having comorbidities is an independent risk factor for the need for respiratory support in COVID-19 cases.
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COVID-19 , SARS-CoV-2 , Esclerodermia Sistémica , Humanos , COVID-19/complicaciones , COVID-19/epidemiología , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/epidemiología , Femenino , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto , Factores de Riesgo , Enfermedades Pulmonares Intersticiales/epidemiología , Hospitalización/estadística & datos numéricos , Comorbilidad , Anciano , Insuficiencia Respiratoria/epidemiología , Insuficiencia Respiratoria/etiología , Progresión de la EnfermedadRESUMEN
Introducción: Goh et al. propusieron en 2008 un algoritmo clasificatorio de SSc-EPID limitada o extensa. La prevalencia de ambos en el momento del diagnóstico de SSc-EPID no se conoce con exactitud. Métodos: La revisión se realizó mediante MEDLINE y SCOPUS desde 2008 hasta 2023 y utilizando los términos: «sistémica», «esclerodermia» o «enfermedad pulmonar intersticial» [MesH]. Se utilizó la escala de Newcastle-Ottawa para la evaluación de la calificación de los estudios observacionales y la escala de Jadad para los ensayos clínicos. Se realizó el método inverso ponderado por la varianza. Resultados: Se incluyeron inicialmente 27 estudios en la revisión sistemática y metaanálisis (SRMA). De ellos, 17 estudios no tenían datos coincidentes. Comunicaron datos de 2.149 pacientes, 1.369 (81,2%) eran mujeres. La edad media era de 52,4 (DE 6,6) años. El 45,2% de los pacientes presentaban el subtipo difuso y el 54,8% el subtipo limitado o esclerodermia sinusal. El 38,7% de los pacientes presentaban anticuerpos antitopoisomerasa positivos y el 14,2% anticuerpos anticentrómero positivos. El porcentaje medio de capacidad vital forzada al inicio del estudio fue del 80,5% (DE 6,9) y de capacidad de difusión pulmonar para el monóxido de carbono fue del 59,1% (DE 9,6). Doce estudios presentaron datos de extensión de SSc-EPID ajustados por PFR y se incluyeron en el metaanálisis. Los 10 estudios observacionales de cohortes se analizaron por separado. El porcentaje global de afectación limitada se estimó en un 63,5% (IC del 95%: 55,3-73; p<0,001) utilizando el modelo de efectos aleatorios. La heterogeneidad entre estudios (I2) fue del 9,8% (IC del 95%: 0-68,2%). La afectación pulmonar extensa se estimó en 34,3% (IC del 95%: 26-45,4; p<0,001). La heterogeneidad entre estudios (I2) fue del 0% (IC del 95%: 0-61,6%) con el modelo de efectos aleatorios.(AU)
Introduction: Goh et al. proposed in 2008 a classificatory algorithm of limited or extensive SSc-ILD. The prevalence of both at the time of diagnosis of SSc-ILD is not known with exactitude. Methods: The review was undertaken by means of MEDLINE and SCOPUS from 2008 to 2023 and using the terms: systemic, scleroderma or interstitial lung disease [MesH]. The Newcastle-Ottawa Scale was used for the qualifying assessment for observational studies and the Jadad scale for clinical trials. The inverse variance-weighted method was performed. Results: Twenty-seven studies were initially included in the systematic review and meta-analysis (SRMA). Of these, 17 studies had no overlapping data. They reported data from 2,149 patients, 1,369 (81.2%) were female. The mean age was 52.4 (SD 6.6) years. 45.2% of the patients had the diffuse subtype and 54.8% had the limited or sine scleroderma subtype. A total of 38.7% of the patients showed positive antitopoisomerase antibodies and 14.2% positive anticentromere antibodies. The mean percentage of forced vital capacity at baseline was 80.5% (SD 6.9) and of diffusing capacity of the lungs for carbon monoxide was 59.1% (SD 9.6). Twelve studies presented SSc-ILD extension data adjusted for PFTs and were included in the meta-analysis. The 10 observational cohort studies were analyzed separately. The overall percentage of limited extension was estimated at 63.5% (95%CI 55.373; p<0.001) using the random-effects model. Heterogeneity between studies (I2) was 9.8% (95%CI 068.2%) with the random-effects model. Extensive pulmonary involvement was estimated at 34.3% (95%CI 2645.4; p<0.001). Heterogeneity between studies (I2) was 0% (95%CI 061.6%) with the random-effects model. Conclusion: The overall percentage of limited SSc-ILD at the time of diagnosis of SSc-ILD was estimated at 63.5% and extensive at 34.3%.(AU)
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Humanos , Masculino , Femenino , Enfermedades Pulmonares Intersticiales/diagnóstico , Prevalencia , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/etiología , Capacidad VitalRESUMEN
Rationale: It is unknown whether air pollution is associated with radiographic features of interstitial lung disease in individuals with chronic obstructive pulmonary disease (COPD). Objectives: To determine whether air pollution increases the prevalence of interstitial lung abnormalities (ILA) or percent high-attenuation areas (HAA) on computed tomography (CT) in individuals with a heavy smoking history and COPD. Methods: We performed a cross-sectional study of SPIROMICS (Subpopulations and Intermediate Outcome Measures in COPD Study), focused on current or former smokers with COPD. Ten-year exposure to particulate matter ⩽2.5 µm in aerodynamic diameter (PM2.5), nitrogen oxides (NOx), nitrogen dioxide (NO2), and ozone before enrollment CT (completed between 2010 and 2015) were estimated with validated spatiotemporal models at residential addresses. We applied adjusted multivariable modified Poisson regression and linear regression to investigate associations between pollution exposure and relative risk (RR) of ILA or increased percent HAA (between -600 and -250 Hounsfield units), respectively. We assessed for effect modification by MUC5B-promoter polymorphism (variant allele carriers GT or TT vs. GG at rs3705950), smoking status, sex, and percent emphysema. Results: Among 1,272 participants with COPD assessed for HAA, 424 were current smokers, and 249 were carriers of the variant MUC5B allele. A total of 519 participants were assessed for ILA. We found no association between pollution exposure and ILA or HAA. Associations between pollutant exposures and risk of ILA were modified by the presence of MUC5B polymorphism (P value interaction term for NOx = 0.04 and PM2.5 = 0.05) and smoking status (P value interaction term for NOx = 0.05; NO2 = 0.01; and ozone = 0.05). With higher exposure to NOx and PM2.5, MUC5B variant carriers had an increased risk of ILA (RR per 26 ppb NOx, 2.41; 95% confidence interval [CI], 0.97-6.0; and RR per 4 µg â m-3 PM2.5, 1.43; 95% CI, 0.93-2.2, respectively). With higher exposure to NO2, former smokers had an increased risk of ILA (RR per 10 ppb, 1.64; 95% CI, 1.0-2.7). Conclusions: Exposure to ambient air pollution was not associated with interstitial features on CT in this population of heavy smokers with COPD. MUC5B modified the association between pollution and ILA, suggesting that gene-environment interactions may influence prevalence of interstitial lung features in COPD.