Prognostic factors of progressive fibrotic hypersensitivity pneumonitis: a large, retrospective, multicentre, observational cohort study.

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) 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.

Economic Burden of Idiopathic Pulmonary Fibrosis in Spain: A Prospective Real-World Data Study (OASIS Study).

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease associated with dyspnoea, cough and impaired quality of life affecting around 7500 patients in Spain. OBJECTIVE: Our aim was to estimate the economic impact of IPF according to forced vital capacity (FVC) % predicted level in adult patients. METHODS: We conducted a prospective, observational, multicentric study of patients with confirmed IPF in Spain. Total annual IPF-related costs were estimated per patient, and categorised according to the FVC% predicted value (FVC < 50%, FVC 50-80%, FVC > 80%) and total sample. Incurred direct health- and non-health-related costs and indirect costs were calculated considering the IPF-related healthcare resource use and the corresponding unitarian costs. Results were updated to 2023 euros. RESULTS: Two hundred and four consecutive patients with IPF were included: 77% male, average age (standard deviation) 70.8 (7.6) years. At baseline, FVC% was < 50%, 50-80% and > 80% of predicted value in 10.8%, 74.5% and 14.7% of patients, respectively. The final cost-evaluable population included 180 subjects. The mean (standard deviation) total annual IPF-related cost was €26,997 (17,555), with statistically significant differences (p = 0.0002) between groups: €44,412 (33,389) for the FVC < 50%, €25,803 (14,688) for the FVC 50-80% and €23,242 (13,642) for the FVC > 80%. Annual direct health costs had the greatest weight and included pharmacological treatments [€22,324 (13,773)] and hospitalisation days [€1659 (7362)]. 14 patients had ≥ 1 acute exacerbation of IPF during the study; mean total cost of an acute exacerbation of IPF was €10,372. According to the multivariate analysis, an impaired lung function (FVC < 50%) and use of antifibrotic treatment were determinants of cost (p < 0.0001 both). CONCLUSIONS: We observed a significantly higher annual IPF-related cost at a lower level of predicted FVC%, the direct cost having the greatest weight to the total costs. Maintaining patients at early disease stages by slowing IPF progression is relevant to reduce the economic impact of IPF. CLINICAL TRIAL REGISTRATION: EU PAS register number EUPAS19387 (1 June, 2017).

Criterios de cribado de la enfermedad pulmonar intersticial difusa asociada a la artritis reumatoide: propuesta de expertos basada en metodología Delphi / Screening criteria for interstitial lung disease associated to rheumatoid arthritis: Expert proposal based on Delphi methodology

Objetivo: Elaborar una propuesta multidisciplinar de criterios de cribado de enfermedad pulmonar intersticial difusa (EPID) en pacientes con artritis reumatoide (AR) y, a la inversa, que sirvan de referencia en la derivación entre los servicios de Reumatología y Neumología para la detección precoz de estos pacientes. Métodos: Se revisó de forma sistemática la literatura sobre factores de riesgo para el desarrollo de EPID en la AR, la utilidad de los distintos métodos diagnósticos para su identificación en pacientes con AR y las diferentes propuestas de criterios de derivación a Reumatología por sospecha de AR precoz. Basándose en la evidencia disponible y en su experiencia clínica, un comité científico formado por dos reumatólogos y dos neumólogos propuso unos criterios de cribado que fueron evaluados mediante el método Delphi por un panel de siete neumólogos y siete reumatólogos. Todos los participantes eran expertos en esta patología. Resultados: Se han elaborado unos criterios para el cribado de EPID en pacientes diagnosticados de AR, y unos criterios para la detección precoz de AR en casos de EPID de causa no filiada. Se incluyen también propuestas sobre las pruebas complementarias a realizar en los diferentes escenarios clínicos considerados y sobre la periodicidad con la que debe repetirse el cribado. Conclusiones: Se propone por primera vez una estrategia de cribado selectivo para el diagnóstico precoz de los pacientes con EPID-AR. Esta propuesta pretende resolver algunos interrogantes clínicos habituales y facilitar la toma de decisiones. Los criterios propuestos deben ser evaluados en futuros estudios de validación.(AU)

Objective: To develop a joint proposal for screening criteria of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) and vice versa, which serves as a guidelines in patient referral between the Rheumatology and Pneumology departments to early detection of these patients. Methods: A systematic literature review was carried out on the risk factors for the development of ILD in RA patients, and for the referral criteria to Rheumatology for suspected early RA. Based on the available evidence, screening criteria were agreed using the Delphi method by a panel of pneumologists and rheumatologists with expertise in these pathologies. Results: Screening criteria for ILD in patients with RA and for the early detection of RA in cases with ILD of unknown etiology have been developed. In both cases, a detection strategy was based on clinical risk factors. Recommendations also included the complementary tests to be carried out in the different clinical scenarios and on the periodicity that screening should be repeated. Conclusion: A selective screening strategy is recommended for the first time in the early diagnosis of patients with ILD-RA. This multidisciplinary proposal aims to solve some common clinical questions and help decision-making, although its usefulness to identify these patients with good sensitivity must be confirmed in a validation study.(AU)

Screening criteria for interstitial lung disease associated to rheumatoid arthritis: Expert proposal based on Delphi methodology.

OBJECTIVE: To develop a joint proposal for screening criteria of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) and vice versa, which serves as a guidelines in patient referral between the Rheumatology and Pneumology departments to early detection of these patients. METHODS: A systematic literature review was carried out on the risk factors for the development of ILD in RA patients, and for the referral criteria to Rheumatology for suspected early RA. Based on the available evidence, screening criteria were agreed using the Delphi method by a panel of pneumologists and rheumatologists with expertise in these pathologies. RESULTS: Screening criteria for ILD in patients with RA and for the early detection of RA in cases with ILD of unknown etiology have been developed. In both cases, a detection strategy was based on clinical risk factors. Recommendations also included the complementary tests to be carried out in the different clinical scenarios and on the periodicity that screening should be repeated. CONCLUSION: A selective screening strategy is recommended for the first time in the early diagnosis of patients with ILD-RA. This multidisciplinary proposal aims to solve some common clinical questions and help decision-making, although its usefulness to identify these patients with good sensitivity must be confirmed in a validation study.

Recomendaciones SER-SEPAR para el manejo de la enfermedad pulmonar intersticial difusa asociada a la artritis reumatoide. Parte 2: tratamiento / SER-SEPAR recommendations for the the management of rheumatoid arthritis-related interstitial lung disease. Part 2: Treatment

Objetivo: Elaborar unas recomendaciones multidisciplinares para mejorar el manejo de la enfermedad pulmonar intersticial difusa asociada a la artritis reumatoide (EPID-AR). Métodos: Un panel de reumatólogos y neumólogos expertos identificó preguntas clínicas de investigación relevantes para el objetivo del documento. Se realizaron revisiones sistemáticas de la evidencia, que se graduó de acuerdo con los criterios del SIGN. Tras ello, se formularon las recomendaciones. Resultados: Se seleccionaron seis preguntas PICO, tres de las cuales específicamente evaluaron la seguridad y la eficacia de los glucocorticoides, fármacos de acción lenta moduladores de la enfermedad (FAME) sintéticos clásicos e inmunosupresores, FAME biológicos, FAME sintéticos dirigidos y antifibróticos en el tratamiento de los pacientes con EPID-AR. Se formularon un total de 12 recomendaciones específicas sobre este tema con base en la evidencia encontrada y/o consenso de expertos. Conclusiones: Se presenta el primer documento oficial SER-SEPAR de recomendaciones específicas para el abordaje terapéutico de la EPID-AR con el fin de ayudar en la toma de decisiones a los clínicos directamente implicados en su manejo.(AU)

Objective: To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD). Methods: Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria. Specific recommendations were made. Results: Six PICO questions were selected, three of which analysed the safety and effectiveness of glucocorticoids, classical synthetic disease-modifying anti-rheumatic drugs (DMARDs) and other immunosuppressants, biological agents, targeted synthetic DMARDs, and antifibrotic therapies in the treatment of this complication. A total specific of 12 recommendations on this topic were formulated based on the evidence found and/or expert consensus. Conclusions: We present the first official SER-SEPAR document with specific recommendations for RA-ILD management developed to resolve some common clinical questions, reduce clinical healthcare variability, and facilitate decision-making for patients.(AU)

Recomendaciones SER-SEPAR para el manejo de la enfermedad pulmonar intersticial difusa asociada a la artritis reumatoide. Parte 1: epidemiología, factores de riesgo y pronóstico / SER-SEPAR recommendations for the management of rheumatoid arthritis-related interstitial lung disease. Part 1: Epidemiology, risk factors and prognosis

Objetivo: Elaborar unas recomendaciones multidisciplinares para mejorar el manejo de la enfermedad pulmonar intersticial difusa asociada a la artritis reumatoide (EPID-AR). Métodos: Un panel de reumatólogos y neumólogos expertos identificó preguntas clínicas de investigación relevantes para el objetivo del documento. Se realizaron revisiones sistemáticas de la evidencia, que se graduó de acuerdo con los criterios del Scottish Intercollegiate Guidelines Network (SIGN). Tras ello, se formularon las recomendaciones. Resultados: Se seleccionaron seis preguntas PICO, tres de las cuales específicamente evaluaron la incidencia y la prevalencia de esta complicación, los factores de riesgo para su desarrollo, y los factores pronósticos de mortalidad y de progresión de la EPID-AR. Se formularon un total de 6 recomendaciones específicas sobre estos aspectos, estructuradas por pregunta, con base en la evidencia encontrada y/o consenso de expertos. Conclusiones: Se presenta el primer documento oficial SER-SEPAR de recomendaciones específicas para el abordaje la EPID-AR, con el fin de ayudar en la toma de decisiones a los clínicos directamente implicados en su manejo y aproximar la práctica asistencial a la mejor evidencia posible.(AU)

Objective: To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD). Methods: Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria. Specific recommendations were made. Results: Six PICO questions were selected, three of which analysed the incidence and prevalence of RA-ILD, associated risk factors, and predictors of progression and mortality. A total of 6 specific recommendations on these topics, structured by question, were formulated based on the evidence found and/or expert consensus. Conclusions: We present the first official SER-SEPAR document with specific recommendations for RA-ILD management developed to resolve some common clinical questions and facilitate decision-making for patients.(AU)

Clinical management and acute exacerbations in patients with idiopathic pulmonary fibrosis in Spain: results from the OASIS study.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with decline in lung function and poor prognosis entailing significant impairment in quality of life and high socioeconomic burden. The aim of this study was to characterize clinical management and resources utilization of patients with IPF in Spain, according to predicted forced vital capacity (FVC) % at baseline. METHODS: Prospective, non-interventional, multicentric real-world data study in patients with IPF in Spain with 12-months follow-up. Clinical management and resources utilization during study period were recorded and compared between groups. FVC decline and acute exacerbations occurrence and associated healthcare resource use were also analysed. FVC decline after 12 months was estimated as relative change. RESULTS: 204 consecutive patients with IPF were included and divided according to baseline FVC % predicted value. At baseline, patients with FVC < 50% received significantly more pharmacological and non-pharmacological treatments, and more help from caregiver. During the 12-months follow-up, patients with FVC < 50% required more specialized care visits, emergency visits, hospitalizations, pulmonary functions tests, non-health resource use (special transportation), and pharmacological treatments (p < 0.05 for all comparisons). Moreover, patients with FVC < 50% at baseline experienced more AE-IPF (p < 0.05), requiring more health-related resources use (primary care visits, p < 0.05). FVC decline was observed in all groups over the 12 months. FVC decreased on average by 2.50% (95% CI: - 5.98 to 0.98) along the year. More patients experienced an FVC decline > 10% in the more preserved lung function groups than in the FVC < 50% group, because of their already deteriorated condition. CONCLUSIONS: We observed a significantly higher annual IPF-related resource use in patients with more impaired lung function at baseline. Since FVC decreases irrespective of FVC% predicted at baseline, slowing IPF progression to maintain patients at early disease stages is relevant to improve IPF management and to optimize resource use. TRIAL REGISTRATION: EU PAS register number EUPAS19387 [June 01, 2017].

SER-SEPAR recommendations for the management of rheumatoid arthritis-related interstitial lung disease. Part 2: Treatment.

OBJECTIVE: To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD). METHODS: Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria. Specific recommendations were made. RESULTS: Six PICO questions were selected, three of which analysed the safety and effectiveness of glucocorticoids, classical synthetic disease-modifying anti-rheumatic drugs (DMARDs) and other immunosuppressants, biological agents, targeted synthetic DMARDs, and antifibrotic therapies in the treatment of this complication. A total of 12 recommendations were formulated based on the evidence found and/or expert consensus. CONCLUSIONS: We present the first official SER-SEPAR document with specific recommendations for RA-ILD management developed to resolve some common clinical questions, reduce clinical healthcare variability, and facilitate decision-making for patients.

SER-SEPAR recommendations for the management of rheumatoid arthritis-related interstitial lung disease. Part 1: Epidemiology, risk factors and prognosis.

OBJECTIVE: To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD). METHODS: Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria. Specific recommendations were made. RESULTS: Six PICO questions were selected, three of which analysed the incidence and prevalence of RA-ILD, associated risk factors, and predictors of progression and mortality. A total of 6 specific recommendations on these topics, structured by question, were formulated based on the evidence found and/or expert consensus. CONCLUSIONS: We present the first official SER-SEPAR document with specific recommendations for RA-ILD management developed to resolve some common clinical questions and facilitate decision-making for patients.

Diagnostic delay of associated interstitial lung disease increases mortality in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main extra-articular organ affected is the lung, sometimes in the form of diffuse interstitial lung disease (ILD) and conditions the prognosis. A multicenter, observational, descriptive and cross-sectional study of consecutive patients diagnosed with RA-ILD. Demographic, analytical, respiratory functional and evolution characteristics were analyzed to evaluate the predictors of progression and mortality. 106 patients were included. The multivariate analysis showed that the diagnostic delay was an independent predictor of mortality (HR 1.11, CI 1.01-1.23, p = 0.035). Also, age (HR 1.33, 95% CI 1.09-1.62, p = 0.0045), DLCO (%) (HR 0.85, 95% CI 0.73-0.98, p = 0.0246), and final SatO2 (%) in the 6MWT (HR 0.62, 95% CI 0.39-0.99, p = 0.0465) were independent predictor variables of mortality, as well as GAP index (HR 4.65, 95% CI 1.59-13.54, p = 0.0051) and CPI index (HR 1.12, 95% CI 1.03-1.22, p = 0.0092). The withdrawal of MTX or LFN after ILD diagnosis was associated with disease progression in the COX analysis (HR 2.18, 95% CI 1.14-4.18, p = 0.019). This is the first study that highlights the diagnostic delay in RA-ILD is associated with an increased mortality just like happens in IPF.

Comorbidities and Complications in Idiopathic Pulmonary Fibrosis.

Though idiopathic pulmonary fibrosis (IPF) is characterized by single-organ involvement, many comorbid conditions occur within other organ systems. Patients with IPF may present during evolution different complications and comorbidities that influence the prognosis and modify the natural course of their disease. In this chapter, we highlight common comorbid conditions encountered in IPF, discuss disease-specific diagnostic modalities, and review the current treatment data for several key comorbidities. The diagnosis and treatment of these comorbidities is a challenge for the pulmonologist specialized in interstitial lung diseases (ILDs). We will focus on pulmonary emphysema, lung cancer, gastroesophageal reflux, pulmonary hypertension, obstructive sleep apnea (sleep disorders), and acute exacerbation of IPF.

Pacientes octogenarios con enfermedad pulmonar obstructiva crónica: características y utilidad de los índices pronósticos / Octogenarian patients with chronic obstructive pulmonary disease: Characteristics and usefulness of prognostic indexes

Antecedentes y objetivo: La mayoría de los estudios sobre enfermedad pulmonar obstructiva crónica (EPOC) excluyen a pacientes octogenarios, por lo que la enfermedad no está bien caracterizada en esta franja etaria. El objetivo de este trabajo es estudiar las características clínicas de los pacientes octogenarios con EPOC y la utilidad de los índices pronósticos más empleados en la enfermedad en esta edad. Pacientes y método: Estudio retrospectivo de pacientes consecutivos atendidos en una consulta entre 2009 y 2017. Se estudiaron parámetros de función pulmonar, distribución de fenotipos clínicos, historial de ingresos, mortalidad, comorbilidades y la utilidad de los índices de Charlson, BODEX, COTE y CODEX para predecir mortalidad. Resultados: La muestra constó de 698 sujetos, 82 (11,7%)≥80 años. El seguimiento medio fue de 47,9±21,8 meses. En los pacientes octogenarios la gravedad de la EPOC medida por el FEV1% o por el índice BODEX fue similar a la de sujetos más jóvenes, pero la disnea fue mayor. En estos pacientes fue más frecuente el fenotipo bronquitis crónica y el exacerbador frecuente, y menos prevalente el enfisema. Los octogenarios tenían más prevalencia de comorbilidad cardiovascular y de insuficiencia renal. Los ingresos hospitalarios y la mortalidad fueron mayores en los octogenarios. La mayoría de los índices pronósticos tuvieron utilidad para predecir mortalidad en los pacientes ancianos, y en ambos grupos etarios el índice CODEX fue el mejor a este respecto. Conclusión: Los pacientes octogenarios con EPOC tienen unas características diferenciales que podrían implicar un abordaje terapéutico diferente. Los índices pronósticos son útiles para predecir mortalidad en esta población

Background and objective: Most studies on chronic obstructive pulmonary disease (COPD) exclude octogenarian patients. Therefore, the disease is not well characterized in this age group. The objective of this study is to analyze the clinical characteristics of octogenarian patients with COPD and the usefulness of the prognostic indexes used most frequently in this age group. Patients and method: Retrospective study of consecutive patients seen at a clinic between 2009 and 2017. The following variables were analyzed: lung function parameters, distribution of clinical phenotypes, income history, mortality, comorbidities and usefulness of the Charlson, BODEX, COTE and CODEX indexes to predict mortality. Results: The sample comprised 698 patients, 82 aged (11.7%)≥80 years old. Mean follow-up time was 47.9±21.8 months. In octogenarian patients, the severity of the COPD, assessed by means of the FEV1% or BODEX index, was similar to that of younger patients, but dyspnea was worse in the elderly group. In these patients, the chronic bronchitis and frequent exacerbator phenotypes were the most frequent, whilst the emphysema phenotype was the least common. Octogenarians had a greater prevalence of cardiovascular comorbidities and renal diseases. Moreover, hospital admissions were more frequent and mortality was higher in these elderly patients. Most prognostic indexes were useful in predicting mortality in elderly patients. CODEX was the most useful index to predict mortality, both in octogenarian and younger patients. Conclusion: Octogenarian patients with COPD have differential characteristics which could imply the need for different therapeutic approaches. Prognostic indexes are useful for predicting mortality in this population

Mortality in COPD patients according to clinical phenotypes.

Purpose: Grouping COPD subjects into clinical phenotypes might be useful for the management of the disease, but the clinical implications of such classification are still not totally clear, especially regarding prognosis. The primary objective of this study was to assess whether the mortality rates were different between four predefined clinical phenotypes. Patients and methods: This is a retrospective, observational study carried out at the COPD clinic of a University Hospital. A total of 891 COPD patients were classified, according to the Spanish COPD guidelines, into the following four phenotypes: asthma-COPD overlap (ACO; 75 subjects), nonexacerbator (NONEX; 531 subjects), exacerbator with chronic bronchitis (EXCB; 194 subjects), and exacerbator with emphysema (EXEMPH; 91 subjects). We compared the mortality outcomes between the phenotypes. Results: After a follow-up of 48.4±25.2 months, there were 194 deaths (21.8%). There were significant differences in all-cause mortality between phenotypes. The ACO phenotype had the best long-term prognosis, whereas EXEMPH had the highest risk of death. NONEX and EXCB mortality figures were in between the other two groups. We also found some differences in the causes of death, and patients with EXEMPH were at a higher risk of dying because of COPD itself. The differences in mortality did not seem related to the classification into phenotypes in itself but to disparities in COPD severity and comorbidity load between groups. Conclusion: Classifying COPD patients according to several predefined clinical phenotypes can identify clusters of subjects with different mortality outcomes. Some phenotypes are associated with a specific cause of death. The mechanisms that underlie these differences seem to be related to COPD severity and comorbidities.

Octogenarian patients with chronic obstructive pulmonary disease: Characteristics and usefulness of prognostic indexes. / Pacientes octogenarios con enfermedad pulmonar obstructiva crónica: características y utilidad de los índices pronósticos.

BACKGROUND AND OBJECTIVE: Most studies on chronic obstructive pulmonary disease (COPD) exclude octogenarian patients. Therefore, the disease is not well characterized in this age group. The objective of this study is to analyze the clinical characteristics of octogenarian patients with COPD and the usefulness of the prognostic indexes used most frequently in this age group. PATIENTS AND METHOD: Retrospective study of consecutive patients seen at a clinic between 2009 and 2017. The following variables were analyzed: lung function parameters, distribution of clinical phenotypes, income history, mortality, comorbidities and usefulness of the Charlson, BODEX, COTE and CODEX indexes to predict mortality. RESULTS: The sample comprised 698 patients, 82 aged (11.7%)≥80 years old. Mean follow-up time was 47.9±21.8 months. In octogenarian patients, the severity of the COPD, assessed by means of the FEV1% or BODEX index, was similar to that of younger patients, but dyspnea was worse in the elderly group. In these patients, the chronic bronchitis and frequent exacerbator phenotypes were the most frequent, whilst the emphysema phenotype was the least common. Octogenarians had a greater prevalence of cardiovascular comorbidities and renal diseases. Moreover, hospital admissions were more frequent and mortality was higher in these elderly patients. Most prognostic indexes were useful in predicting mortality in elderly patients. CODEX was the most useful index to predict mortality, both in octogenarian and younger patients. CONCLUSION: Octogenarian patients with COPD have differential characteristics which could imply the need for different therapeutic approaches. Prognostic indexes are useful for predicting mortality in this population.

Enfermedad del pulmón del granjero: análisis de 75 casos / Farmer's lung disease: Analysis of 75 cases

Introducción: La enfermedad del pulmón de granjero (EPG) es una forma frecuente de neumonitis por hipersensibilidad, posiblemente infradiagnosticada. El objetivo de este estudio es describir las características clínicas, la evolución y los factores que influyen en el pronóstico de los pacientes con EPG. Pacientes y métodos: Estudio retrospectivo que incluyó a todos los pacientes diagnosticados de EPG por haber presentado una exposición ambiental de riesgo, una clínica, una función pulmonar y una radiología compatible, en los que se demostró sensibilización antigénica y/o una anatomía patológica concordante. Resultados: Se incluyeron 75 pacientes con EPG, 50 con forma aguda o subaguda (FAS) y 25 con forma crónica (FC). Un 44% de los pacientes (n=33) fue diagnosticado durante los meses de marzo y abril, especialmente aquellos con FAS en comparación con las FC (52 vs. 28%; p=0,0018). En las FAS la DLco presentó una mejoría durante el seguimiento (p=0,047). La determinación de anticuerpos IgG específicos fue positiva en 39 pacientes (78%) con FAS (44% de ellos frente a Aspergillus) y en 12 con FC (48%). La realización de la evitación antigénica (OR 9,26, IC 95% 1,3-66,7, p=0,026) y la administración de tratamiento inmunodepresor (OR 16,13, IC 95% 1,26-200, p=0,033) fueron los factores predictores de mejor evolución de la enfermedad. Conclusiones: La EPG presenta un inicio con predominio estacional en nuestro medio. Las FC presentan habitualmente determinación de anticuerpos IgG específicos negativos, a diferencia de las FAS, donde los anticuerpos frente Aspergillus son las más frecuentes. La evitación antigénica y el tratamiento inmunodepresor son posibles predictores de mejor evolución de la enfermedad (AU)

Introduction: Farmer's lung disease (FLD) is a common form of hypersensitivity pneumonitis possibly underdiagnosed in our midst. The aim of this study was to describe clinical characteristics, evolution and factors that influence the prognosis of patients with FLD. Patients and methods: A retrospective study that included all patients diagnosed with FLD presenting an environmental exposure risk, a clinic, lung function and a compatible radiology, in which antigen sensitisation was demonstrated and/or a concordant pathology. Results: We selected 75 patients with FLD, 50 with acute or subacute form (ASF) and 25 with chronic form (CF). Forty-four percent of patients (n=33) were diagnosed during the months of March and April, especially those with ASF compared to CF (52 vs. 28%; P=.0018). In the ASF group, DLco showed an improvement during follow-up (P=.047). The determination of specific IgG antibodies was positive in 39 patients (78%) with ASF (44% of them against Aspergillus) and CF 12 (48%). The realisation of antigenic avoidance (OR 9.26, 95% CI 1.3-66.7, P=.026) and the administration of immunosuppressive therapy (OR 16.13, 95% CI 1.26-200, P=.033) were predictors of better disease progression. Conclusions: FLD is predominantly seasonal in our environment. CF usually has a negative specific IgG antibodies unlike ASF, where antibodies against Aspergillus are the most common. The realisation of antigenic avoidance and immunosuppressive treatment are possible predictors of better disease progression (AU)

Farmer's lung disease: Analysis of 75 cases. / Enfermedad del pulmón del granjero: análisis de 75 casos.

INTRODUCTION: Farmer's lung disease (FLD) is a common form of hypersensitivity pneumonitis possibly underdiagnosed in our midst. The aim of this study was to describe clinical characteristics, evolution and factors that influence the prognosis of patients with FLD. PATIENTS AND METHODS: A retrospective study that included all patients diagnosed with FLD presenting an environmental exposure risk, a clinic, lung function and a compatible radiology, in which antigen sensitisation was demonstrated and/or a concordant pathology. RESULTS: We selected 75 patients with FLD, 50 with acute or subacute form (ASF) and 25 with chronic form (CF). Forty-four percent of patients (n=33) were diagnosed during the months of March and April, especially those with ASF compared to CF (52 vs. 28%; P=.0018). In the ASF group, DLco showed an improvement during follow-up (P=.047). The determination of specific IgG antibodies was positive in 39 patients (78%) with ASF (44% of them against Aspergillus) and CF 12 (48%). The realisation of antigenic avoidance (OR 9.26, 95% CI 1.3-66.7, P=.026) and the administration of immunosuppressive therapy (OR 16.13, 95% CI 1.26-200, P=.033) were predictors of better disease progression. CONCLUSIONS: FLD is predominantly seasonal in our environment. CF usually has a negative specific IgG antibodies unlike ASF, where antibodies against Aspergillus are the most common. The realisation of antigenic avoidance and immunosuppressive treatment are possible predictors of better disease progression.

Prevalence of Major Comorbidities in Chronic Obstructive Pulmonary Disease Caused by Biomass Smoke or Tobacco.

BACKGROUND: Comorbidities are very common in chronic obstructive pulmonary disease (COPD), contributing to the overall severity of the disease. The relative prevalence of comorbidities in COPD caused by biomass smoke (B-COPD), compared with COPD related to tobacco (T-COPD), is not well known. OBJECTIVES: To establish if both types of COPD are associated with a different risk for several major comorbidities. METHOD: The prevalence of comorbidities was compared in 863 subjects with B-COPD (n = 179, 20.7%) or T-COPD (n = 684, 79.2%). Multivariate analysis was carried out to explore the independent relationship between comorbidities and type of exposure. RESULTS: Three comorbidities were more frequent in T-COPD than in B-COPD: ischemic heart disease (11.5 vs. 5.0%, respectively, p = 0.01), peripheral vascular disease (9.2 vs. 2.7%, p = 0.006), and peptic ulcer disease (4.8% vs. 0, p = 0.005). After correcting for potential confounding variables, the risk of ischemic heart disease was lower in B-COPD than in T-COPD (OR: 0.33, 95% CI: 0.16-0.69, p = 0.003). CONCLUSIONS: The prevalence of ischemic heart disease is significantly lower in B-COPD than in T-COPD, suggesting a different systemic effect of both types of smoke in COPD patients.

Revisión de la enfermedad del pulmón de granjero / Farmer's Lung Disease. A Review

La enfermedad del pulmón de granjero (EPG) es una forma de neumonitis por hipersensibilidad (NH) producida por la inhalación de microorganismos procedentes del heno o grano almacenado en condiciones de alta humedad en el ámbito laboral agrícola. Se trata de una enfermedad probablemente infradiagnosticada, sobre todo en el Norte de España, donde las condiciones climáticas son propicias para el desarrollo de la misma. Según estudios previos los antígenos más frecuentes suelen ser hongos y actinomicetos termofílicos. La epidemiología de la enfermedad no es del todo bien conocida, y se basa en estudios realizados por grupos centroeuropeos y asiáticos. La presentación clínica puede ser variada, diferenciándose las formas agudas (tras exposición a elevadas concentraciones del antígeno) y las crónicas (exposición a menores concentraciones del antígeno, pero más prolongada en el tiempo). En estos casos es esencial, en aquellos pacientes con clínica respiratoria durante la exposición laboral agrícola, demostrar una radiología y función pulmonar compatible, así como una sensibilización al antígeno, una linfocitosis en el lavado broncoalveolar en su caso y/o una anatomía patológica concordante. El tratamiento principal es la evitación antigénica, por lo que la educación de los pacientes en las medidas preventivas es fundamental. Por el momento, no existen estudios controlados que permitan evaluar el papel de tratamientos inmunosupresores en esta enfermedad. El tratamiento con corticosteroides solo ha demostrado acelerar la resolución de las formas agudas, pero no hay estudios que demuestren su efectividad a largo plazo, con el fin de evitar la progresión de la enfermedad ni disminuir su mortalidad

Farmer's lung disease (FLD) is a form of hypersensitivity pneumonitis (HP) caused by inhaling microorganisms from hay or grain stored in conditions of high humidity in the agricultural workplace. It is probably underdiagnosed, especially in northern Spain, where climatic conditions favor the development of this disease. According to previous studies, the most common antigens are usually thermophilic actinomycetes and fungi. The epidemiology of the disease is not well known, and is based on studies conducted by Central European and Asian groups. The clinical presentation may vary, differentiating the chronic (exposure to lower concentrations of the antigen over a longer period time) and the acute forms (after exposure to high concentrations of the antigen). In patients with respiratory symptoms and agricultural occupational exposure, radiological, lung function and/or anatomical pathology findings must be compatible with FLD, bronchoalveolar lavage must show lymphocytosis, and tests must find sensitivity to the antigen. The main treatment is avoidance of the antigen, so it is essential to educate patients on preventive measures. To date, no controlled studies have assessed the role of immunosuppressive therapy in this disease. Corticosteroid treatment has only been shown to accelerate resolution of the acute forms, but there is no evidence that it is effective in preventing disease progression in the long-term or reducing mortality

Farmer's Lung Disease. A Review. / Revisión de la enfermedad del pulmón de granjero.

Farmer's lung disease (FLD) is a form of hypersensitivity pneumonitis (HP) caused by inhaling microorganisms from hay or grain stored in conditions of high humidity in the agricultural workplace. It is probably underdiagnosed, especially in northern Spain, where climatic conditions favor the development of this disease. According to previous studies, the most common antigens are usually thermophilic actinomycetes and fungi. The epidemiology of the disease is not well known, and is based on studies conducted by Central European and Asian groups. The clinical presentation may vary, differentiating the chronic (exposure to lower concentrations of the antigen over a longer period time) and the acute forms (after exposure to high concentrations of the antigen). In patients with respiratory symptoms and agricultural occupational exposure, radiological, lung function and/or anatomical pathology findings must be compatible with FLD, bronchoalveolar lavage must show lymphocytosis, and tests must find sensitivity to the antigen. The main treatment is avoidance of the antigen, so it is essential to educate patients on preventive measures. To date, no controlled studies have assessed the role of immunosuppressive therapy in this disease. Corticosteroid treatment has only been shown to accelerate resolution of the acute forms, but there is no evidence that it is effective in preventing disease progression in the long-term or reducing mortality.