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Objective: The objective of the study was to analyze the diagnostic process and the time until the start of treatment of patients with idiopathic pulmonary fibrosis in relation to the publication of successive clinical practice guide. Material and methods: Multicenter, observational, ambispective study, in which patients includes in the idiopathic pulmonary fibrosis registry of the Spanish Society of Pulmonologist and Thoracic Surgery were analyzed. An electronic data collection notebook was enabled on the society's website. Sociodemographic and clinical variables were collected at diagnosis and follow-up of the patients. Results: From January 2012 to december 2019, 1064 patients were included in the registry, with 929 finally analyzed. The diagnosis process varied depending on the year in which it was performed, and the radiological pattern observed in the high-resolution computed tomography. Up to 26.3% of the cases (244) were diagnosed with chest high-resolution computed tomography and clinical evaluation. Surgical biopsy was used up to 50.2% of cases diagnosed before 2011, while it has been used in 14.2% since 2018. The median time from the onset of symptoms to diagnosis was 360 days (IQR 120-720), taking more than 2 years in the 21.0% of patients. A percentage of 79.4 of patients received antifibrotic treatment. The average time from diagnosis to the antifibrotic treatment has been 309 ± 596.5 days, with a median of 49 (IQR 0-307). Conclusions: The diagnostic process, including the time until diagnosis and the type of test used, has changed from 2011 to 2019, probably due to advances in clinical research and the publication of diagnostic-therapeutic consensus guidelines.
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Background: Fibrotic hypersensitivity pneumonitis (fHP) is an immune-mediated interstitial lung disease caused by sensitisation to chronic allergen inhalation. This study aimed to determine prognostic indicators of progression and mortality in fHP. Methods: This was a retrospective, multicentre, observational, cross-sectional cohort study of consecutive patients diagnosed with fHP from 1 January 2012 to 31 December 2021. Multivariate Cox regression analyses were used to calculate hazard ratios (HRs) with 95% confidence intervals for predictors of progression and survival. Results: A total of 403 patients were diagnosed with fHP: median (interquartile range) age 66.5 (14.0)â years, 51.9% females and 55.1% never-smokers. The cause of fHP was mainly fungal (39.7%) or avian (41.4%). Lung biopsy was performed in 269 cases (66.7%). In the whole cohort the variables that were related to mortality or lung transplant were older age (HR 1.08; p<0.001), percentage predicted forced vital capacity (HR 0.96; p=0.001), lymphocytosis in bronchoalveolar lavage (BAL) (HR 0.93; p=0.001), presence of acute exacerbation during follow-up (HR 3.04; p=0.001) and GAP (gender, age and lung physiology) index (HR 1.96; p<0.01). In the group of biopsied patients, the presence of fibroblastic foci at biopsy (HR 8.39; p<0.001) stands out in multivariate Cox regression analyses as a highly significant predictor for increased mortality or lung transplant. GAP index (HR 1.26; p=0.009), lymphocytosis in BAL (HR 0.97; p=0.018) and age (HR 1.03; p=0.018) are also predictors of progression. Conclusions: The study identified several prognostic factors for progression and/or survival in fHP. The presence of fibroblastic foci at biopsy was a consistent predictor for increased mortality and the presence of lymphocytosis in BAL was inversely related to mortality.
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Introduction: The aim of the study was to know the management of patients with diffuse interstitial lung disease (ILD) associated with a systemic autoimmune diseases (SAD) in pulmonology outpatient clinics in Spain. Methodology: The ILD work area of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) designed a self-completed questionnaire of 25 questions, on aspects related to the diagnosis and treatment of ILD-SAD. This was distributed among the attendees of the winter meeting of the ILD Area and later via e-mail to all the members of the ILD area of SEPAR. Participation was anonymous, voluntary and without consideration. Results: 74 pulmonologists from 58 hospitals participated. 77% had a specialized ILD consultation. All Units with SEPAR accreditation had a committee made up of pulmonologists and radiologists and a majority participation of pathologists and rheumatologists. In 75% of the centers there was a close collaboration with Rheumatology for the management of ILD-SAD. 85% considered that the frequency of ILD-SAD consults is increasing, the most frequent being ILD associated with rheumatoid arthritis. The treatment of ILD-SAD is decided by consensus between pulmonologist and rheumatologist in 91.3% of the cases. 67% of pulmonologists consider that immunosuppressants and biological therapies can slow down the progression of ILD-SAD. 51% use antifibrotics therapies in these pathologies. Conclusions: Almost all of the accredited Spanish ILD Units by SEPAR have established collaborations with Rheumatology for the adequate management of patients with ILD-SAD, this practice having been extended to units not yet accredited.
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BACKGROUND: Before the advent of genome-wide association studies (GWAS), ADAM33, ADRB2, CD14, MS4A2 (alias FCER1B), IL13, IL4, IL4R, and TNF constituted the most replicated non-HLA candidate genes with asthma and related traits. However, except for the IL13-IL4 region, none of these genes have been found in close proximity of genome-wide significant hits among GWAS for asthma or related traits. Here we aimed to assess the reproducibility of these asthma associations and to test if associations were more evident considering the effect of age at diagnosis. METHODOLOGY/PRINCIPAL FINDINGS: We systematically evaluated 286 common single nucleotide polymorphisms (SNPs) of these 8 genes in a sample of 1,865 unrelated Spanish individuals (606 asthmatics and 1,259 controls). We found that variants at MS4A2, IL4R and ADAM33 genes demonstrated varying association effects with the age at diagnosis of asthma, with 10 SNPs showing study-wise significance after the multiple comparison adjustment. In addition, in silico replication with GWAS data supported the association of IL4R. CONCLUSIONS/SIGNIFICANCE: Our results support the important role of MS4A2, IL4R and ADAM33 genes in asthma and/or atopy susceptibility. However, additional studies in larger samples sets are needed to firmly implicate these genes in asthma susceptibility, and also to identify the causal variation underlying the associations found.
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Asma/diagnóstico , Asma/genética , Polimorfismo de Nucleotídeo Único , Proteínas ADAM/genética , Adolescente , Adulto , Alelos , Estudos de Casos e Controles , Criança , Mapeamento Cromossômico , Frequência do Gene , Estudos de Associação Genética , Predisposição Genética para Doença , Genótipo , Humanos , Subunidade alfa de Receptor de Interleucina-4/genética , Razão de Chances , Receptores de IgE/genética , Adulto JovemRESUMO
BACKGROUND: There are no previous population-based studies assessing the prevalence of Blomia tropicalis (BT). This investigation analyzes the prevalence of sensitization to BT and its contribution to asthma and related diseases among young adults in the Canary Islands. MATERIAL AND METHODS: From a random sample of 9506 adults, aged between 20 and 44, who had previously answered a short respiratory questionnaire, a further 20% random sample was drawn. All participants filled in an extensive questionnaire, and they underwent spirometry and bronchial hyperresponsiveness (BHR) test and skin testing to several allergens as well as the determination of total IgE and specific IgE. RESULTS: The prevalence of positive skin tests to BT was 13.8% (95% confidence interval [CI] 10.6-17.9%) and that of BT-specific IgE 17.9% (95% CI 14.2-24.5%). Sensitization to BT (positive skin tests or positive specific IgE) was 20.9% (95% CI 16.9-25.5%). Most of the subjects sensitized to BT were also sensitized to Dermatophagoides pteronissimus; however, only 7% displayed monosensitization to BT. Among subjects who exhibited BHR, sensitization to BT reached 46.7% (95% CI 32.7-60.9%), among those with asthma 50% (95% CI 26.8-73.2%), in subjects with rhinitis 37.2% (95% CI 28.1-47.6%), and in those suffering from dermatitis 25.9% (95% CI 18.7-34.5%). A strong association of BT sensitization with BHR and asthma before and after 15 years was found. CONCLUSION: The prevalence of sensitization to BT among young adults in the Canary Islands is high, and it displays a close relationship with allergic respiratory diseases and dermatitis.