[Influence of Clinical Practice Guidelines on the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis. Data from the Registry of the Spanish Society of Pulmonology and Thoracic Surgery]. / Influencia de las guías de práctica clínica en el diagnóstico y tratamiento de la fibrosis pulmonar idiopática. Datos del Registro de la Sociedad Española de Neumología y Cirugía Torácica.

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.

Effect of Encapsulated Purple Garlic Oil on Microvascular Function and the Components of Metabolic Syndrome: A Randomized Placebo-Controlled Study-The ENDOTALLIUM Study.

Endothelial dysfunction (ED) is associated with progressive changes contributing to clinical complications related to macro- and microvascular diseases. Garlic (Allium sativum L.) and its organosulfur components have been related to beneficial cardiovascular effects and could improve endothelial function. The ENDOTALLIUM Study aimed to evaluate the effect of the regular consumption of encapsulated purple garlic oil on microvascular function, endothelial-related biomarkers, and the components of metabolic syndrome (MetS) in untreated subjects with cardiometabolic alterations. Fifty-two individuals with at least one MetS component were randomized (1:1) in a single-center, single-blind, placebo-controlled, parallel-group study. The participants received encapsulated purple garlic oil (n = 27) or placebo (n = 25) for five weeks. Skin microvascular peak flow during post-occlusive reactive hyperemia significantly increased in the purple garlic oil group compared to the placebo group (between-group difference [95%CI]: 15.4 [1.5 to 29.4] PU; p = 0.031). Likewise, hs-CRP levels decreased in the purple garlic group compared to the control group (-1.3 [-2.5 to -0.0] mg/L; p = 0.049). Furthermore, we observed a significant reduction in the mean number of MetS components in the purple garlic group after five weeks (1.7 ± 0.9 vs. 1.3 ± 1.1, p = 0.021). In summary, regular consumption of encapsulated purple garlic oil significantly improved microvascular function, subclinical inflammatory status, and the overall MetS profile in a population with cardiometabolic alterations.

Epileptic encephalopathies secondary to hypothalamic hamartomas treated with radiosurgery: A case series.

OBJECTIVE: Hypothalamic hamartomas are congenital lesions that typically present with gelastic seizures, refractory epilepsy, neurodevelopmental delay, and severe cognitive impairment. Surgical procedures have been reported to be effective in removing the hamartomas, however, they are associated with significant morbidity. Therefore, it is not considered a safe therapeutic modality. Image-guided robotic radiosurgery (CyberKnife® Radiosurgery System) has been shown to provide good outcomes without lasting complications. METHODS: This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®. RESULTS: All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24-26 months of follow-up, in three patients, their intelligence quotient scores increased. No complications were reported. SIGNIFICANCE: This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases.

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.

Complex regional pain syndrome type II localized to the index finger. A case report translating scientific evidence into clinical practice.

BACKGROUND: Complex regional pain syndrome type II (CRPS-II) is a rare condition associated with peripheral nervous system lesions. Its localized distribution in the fingers is unique, and its treatment is unclear. CASE DESCRIPTION: A 56-year-old male presented to the emergency department with a saw-cut index finger injury with associated tendon and nerve injuries. After surgery, he was admitted to physical therapy (PT) with persistent pain, joint stiffness, allodynia, and trophic changes compatible with CRPS-II localized in the index finger. The diagnosis was confirmed after applying the Budapest Criteria, and PT was progressive and individualized according to the patient's needs, including graded motor imagery, mobilizations, exercises, and education. OUTCOMES: After 12 weeks of PT, a clinically significant decrease in pain intensity and improvements in mobility and index finger and upper limb functionality was observed, reducing CRPS symptomatology. DISCUSSION: This report provides information about a unique case of a localized form of CRPS-II. After reviewing the literature on clinical cases of both CRPS-II and localized forms of CRPS, we highlight that the clinical features of this patient and his positive therapeutic response support the importance of translating the scientific evidence on CRPS into clinical practice.

Asociación entre el tiempo de enfermedad previo a broncoscopia y tiempo de alta posbroncoscopia en pacientes pediátricos con aspiración de cuerpo extraño: estudio de cohorte retrospectivo en un centro de referencia peruano, 2014-2019 / Association between pre-bronchoscopy time of illness and post-bronchoscopy discharge time in pediatric patients with foreign body aspiration: retrospective cohort study in a Peruvian referral center, 2014-2019

RESUMEN Objetivos. Determinar la asociación entre el tiempo de enfermedad previo a broncoscopia y el tiempo de alta posbroncoscopia en pacientes pediátricos con aspiración de cuerpo extraño. Materiales y métodos. Estudio de cohorte retrospectivo. El estudio y la revisión de historias clínicas se realizó en el Hospital de Emergencias Pediátricas de Lima, Perú. Se revisaron 324 historias clínicas, de las cuales, 183 historias fueron seleccionadas por contar con diagnóstico de cuerpo extraño en vías aéreas y estar adecuadamente llenadas. Para el análisis bivariado se utilizó la prueba exacta de Fisher y U de Mann Whitney, mientras que para el cálculo del riesgo relativo (RR) y su respectivo intervalo de confianza (IC) al 95% se utilizó regresión de Poisson. Resultados. Se incluyeron 183 pacientes, de los cuales, el 65,6% fueron hombres con una media de 2,4 años. La localización más frecuente fue el árbol bronquial derecho y cuerpo extraño de material orgánico. La mayoría (72,7%) de pacientes tuvieron un alta temprana antes de las 24 horas. Se encontró una asociación entre el tiempo de enfermedad previo a la broncoscopia y el tiempo alta posbroncoscopia (RR: 2,94, IC 95%: 1,72-5,01). Conclusiones. Existe una asociación estadísticamente significativa entre el tiempo de enfermedad previo a la broncoscopia y el tiempo de estancia hospitalaria posextracción del cuerpo extraño al ajustar por edad, sexo tipo de cuerpo extraño y maniobra de boca como medida de rescate. Este hallazgo es relevante debido a que pone en evidencia la importancia de una atención temprana, diagnóstico oportuno y manejo precoz en el paciente pediátrico.

ABSTRACT Objective. To determine the association between pre-bronchoscopy time of illness and post-bronchoscopy discharge time in pediatric patients with foreign body aspiration. Materials and methods. Retrospective cohort study. Medical records were studied and reviewed at the Pediatric Emergency Hospital of Lima, Peru. We reviewed 324 medical records, and selected 183 because they had the diagnosis of foreign body aspiration and complete data. Fisher's exact test and Mann Whitney U test were used for the bivariate analysis, while Poisson regression was used to calculate the Relative Risk (RR) and its 95% confidence interval (CI). Results. We included 183 patients, of whom 65.6% were men with a mean age of 2.4 years. The most frequent location of the foreign body was the right bronchial tree and most of them were made of organic material. The majority (72.7%) of patients were discharged before 24 hours. We found an association between the time of illness prior to bronchoscopy and post-bronchoscopy discharge time (RR: 2.94, 95%CI: 1.72 - 5.01). Conclusions. The time of illness prior to bronchoscopy and the length of hospital stay after removal of the foreign body were significantly associated when adjusted for age, sex, type of foreign body and mouth sweep maneuver as a rescue measure. Our findings are relevant because they highlight the importance of early care, timely diagnosis and early management of pediatric patients.

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.

Association between pre-bronchoscopy time of illness and post-bronchoscopy discharge time in pediatric patients with foreign body aspiration: retrospective cohort study in a Peruvian referral center, 2014-2019. / Asociación entre el tiempo de enfermedad previo a broncoscopia y tiempo de alta posbroncoscopia en pacientes pediátricos con aspiración de cuerpo extraño: estudio de cohorte retrospectivo en un centro de referencia peruano, 2014-2019.

OBJECTIVE.: To determine the association between pre-bronchoscopy time of illness and post-bronchoscopy discharge time in pediatric patients with foreign body aspiration. MATERIALS AND METHODS.: Retrospective cohort study. Medical records were studied and reviewed at the Pediatric Emergency Hospital of Lima, Peru. We reviewed 324 medical records, and selected 183 because they had the diagnosis of foreign body aspiration and complete data. Fisher's exact test and Mann Whitney U test were used for the bivariate analysis, while Poisson regression was used to calculate the Relative Risk (RR) and its 95% confidence interval (CI). RESULTS.: We included 183 patients, of whom 65.6% were men with a mean age of 2.4 years. The most frequent location of the foreign body was the right bronchial tree and most of them were made of organic material. The majority (72.7%) of patients were discharged before 24 hours. We found an association between the time of illness prior to bronchoscopy and post-bronchoscopy discharge time (RR: 2.94, 95%CI: 1.72 - 5.01). CONCLUSIONS.: The time of illness prior to bronchoscopy and the length of hospital stay after removal of the foreign body were significantly associated when adjusted for age, sex, type of foreign body and mouth sweep maneuver as a rescue measure. Our findings are relevant because they highlight the importance of early care, timely diagnosis and early management of pediatric patients.

OBJETIVOS.: Determinar la asociación entre el tiempo de enfermedad previo a broncoscopia y el tiempo de alta posbroncoscopia en pacientes pediátricos con aspiración de cuerpo extraño. MATERIALES Y MÉTODOS.: Estudio de cohorte retrospectivo. El estudio y la revisión de historias clínicas se realizó en el Hospital de Emergencias Pediátricas de Lima, Perú. Se revisaron 324 historias clínicas, de las cuales, 183 historias fueron seleccionadas por contar con diagnóstico de cuerpo extraño en vías aéreas y estar adecuadamente llenadas. Para el análisis bivariado se utilizó la prueba exacta de Fisher y U de Mann Whitney, mientras que para el cálculo del riesgo relativo (RR) y su respectivo intervalo de confianza (IC) al 95% se utilizó regresión de Poisson. RESULTADOS.: Se incluyeron 183 pacientes, de los cuales, el 65,6% fueron hombres con una media de 2,4 años. La localización más frecuente fue el árbol bronquial derecho y cuerpo extraño de material orgánico. La mayoría (72,7%) de pacientes tuvieron un alta temprana antes de las 24 horas. Se encontró una asociación entre el tiempo de enfermedad previo a la broncoscopia y el tiempo alta posbroncoscopia (RR: 2,94, IC 95%: 1,72-5,01). CONCLUSIONES.: Existe una asociación estadísticamente significativa entre el tiempo de enfermedad previo a la broncoscopia y el tiempo de estancia hospitalaria posextracción del cuerpo extraño al ajustar por edad, sexo tipo de cuerpo extraño y maniobra de boca como medida de rescate. Este hallazgo es relevante debido a que pone en evidencia la importancia de una atención temprana, diagnóstico oportuno y manejo precoz en el paciente pediátrico.

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)

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.

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.

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.

¿Nuevo horizonte tras el reinicio escolar? Estudio de 45 casos de COVID-19 en un centro de salud / A new horizon after restarting school activity? Study of 45 cases of covid-19 in a primary care center

INTRODUCCIÓN: desde la aparición del SARS-CoV-2 se está observando que los niños de todo el mundo no parecen vulnerables a la infección, pero casi todos los datos se recogieron durante periodos de cierre escolar y medidas de distancia social. El temor a un colapso de los sistemas sanitarios lleva a los Gobiernos a mantener políticas restrictivas globales que pueden causar más daño que beneficio, especialmente en los niños y sus familias. Por ello, es necesario confirmar el papel epidemiológico de los menores en esta pandemia, en condiciones de vida cotidiana, tras la reapertura de los colegios, que conlleva un aumento de sus contactos físicos y sociales. PACIENTES Y MÉTODOS: se presenta el análisis de 45 casos consecutivos diagnosticados de COVID-19 entre las semanas 34 y 44 de 2020 -que incluyen los primeros dos meses del reinicio escolar- recogidos entre los 5250 niños adscritos a los cuatro cupos de Pediatría de un centro de salud urbano de la Comunidad de Madrid. Se constata que los niños presentan una clínica leve e inespecífica y apenas transmiten la infección en el entorno familiar, al igual que han mostrado otros estudios publicados desde el inicio de 2020. CONCLUSIÓN: son necesarios y urgentes amplios estudios poblacionales para crear una base sólida que permita relajar las medidas restrictivas sobre los niños y sus familias. Atención Primaria es una plataforma privilegiada para llevarlos a cabo, pues permite observar de cerca y en condiciones reales el papel que juegan los niños en la dinámica de la infección

INTRODUCTION: since the emergence of SARS-CoV-2, it has been observed that children around the world do not seem vulnerable to the infection, but almost all the data was collected during periods of school closure and firm measures of social distance. The fear of a collapse of health systems leads governments to maintain global restrictive policies that can do more harm than good, especially for children and their families. Therefore, it is necessary to confirm the epidemiological role of children in this pandemic, in daily living conditions, after the reopening of schools and the increase in their physical and social contacts. METHODS: the analysis of 45 consecutive cases diagnosed of COVID-19 between weeks 34 to 44 of 2020 -which includes the first two months after restanting school activity- collected in an urban Primary Care in the Community of Madrid is presented. It is found that children present mild and nonspecific symptoms and barely transmit the infection in their immediaty environment, as has been shown in other studies published since the beginning of 2020. CONCLUSION: Primary Care is a privileged platform to observe in real conditions the role that children play in the dynamics of infection. It is urgent to carry out extensive population studies in order to confirm the low contagiousness of children, since the restriction of their movements and social relationships can cause more harm than good, at different levels

Therapeutic options for primary meningeal angiosarcoma: A case report.

BACKGROUND: Primary angiosarcoma (AS) of the central nervous system (PACNS) is an extremely rare malignancy. The meninges represent an uncommon site of origin of PACNS. This report describes a recurrent meningeal PACNS treated with surgery, radiotherapy, stereotactic radiosurgery, and paclitaxel at different stages of the disease. CASE DESCRIPTION: A 36-year-old Asian male presented to our facility with a 4-month history of worsening headaches and complete right homonymous hemianopia. Neuroimaging revealed a left occipital lobe hematome with an underlying left tentorial tumor. After subtotal resection, neuropathological examination revealed features of a malignant endothelial cell AS. He received a course of adjuvant radiation therapy but experienced disease progression. He subsequently received additional stereotactic radiosurgery followed by weekly paclitaxel. Magnetic resonance imaging during the course of treatment revealed stable disease until patient died following another progression of his tumor. CONCLUSION: This case of a meningeal PACNS highlights the importance of considering this entity in the face of a malignant lesion presenting with intracranial hemorrhagic activity. Our observations suggest that the use of paclitaxel provided a modest clinical response in PACNS, highlighting the need to consider a combined approach structured mainly on surgery and radiotherapy. Stereotactic radiosurgery appears to be a promising treatment option.

Características clínicas y patológicas de pacientes con tumores del sistema nervioso central en un centro en Suramérica desde 2010 hasta 2015 / Clinico-pathological characteristics of central nervous system tumors in a South American center from 2010 to 2015

Resumen Objetivo: Caracterizar los pacientes diagnosticados con tumores del sistema nervioso central en el Instituto Neurológico de Colombia durante el periodo 2010-2015. Métodos: Estudio descriptivo retrospectivo. Para los tumores primarios se usó la clasificación de la Organización Mundial de la Salud y para los metastásicos se usó la Clasificación Internacional de Enfermedades en Oncología. Resultados: Se identificaron 288 pacientes, 194 de ellos con tumores primarios y 94 tumores metastásicos. No se lograron clasificar los tumores primarios en el 23,7 % de los casos y para los metastásicos no se obtuvo la clasificación morfológica (histológica) en el 35,1 % de los casos. Los hombres presentaron con mayor frecuencia tumores de comportamiento maligno tipo glioblastoma NEO (no especificado de otra manera) (14,9 %) y en las mujeres predominaron los tumores de comportamiento benigno tipo meningioma (23,2 %). En mayores de 65 años, tanto el glioblastoma NEO como el meningioma fueron más frecuentes en mujeres con 17,4 % y 28,3 %, respectivamente. Entre los pacientes con tumores metastásicos, de acuerdo con la clasificación topográfica, los tumores primarios más frecuentes se localizaron en pulmón (39,4 %) y mama (17 %). No se identificó el sitio primario de metástasis en el 11,7 % de los casos. La histología más comúnmente identificada fue el adenocarcinoma (14,9 %), seguido del carcinoma (8,5 %). Conclusiones: Para una vigilancia efectiva de la enfermedad es necesario realizar un monitoreo epidemiológico y clínico de tumores primarios y metastásicos, mediante el uso de registros institucionales de cáncer, incluyendo datos topográficos, histológicos y moleculares, según disponibilidad.

Abstract Objective: The aim of this study was to characterize patients with a diagno- sis of a central nervous system (CNS) tumors at the Instituto Neurologico de Colombia during the period between 2010 to 2015. Methods: A retrospective descriptive study was conducted. The Classification of the World Health Organization was used for CNS primary tumors whereas the International Classification of Diseases for oncology (ICD-O) was used for CNS metastatic tumors. Results: 288 patients were identified, 194 of them with primary tumors of CNS and 94 with metastatic tu- mors from systemic cancer. It was not possible to classify primary tumors in 23.7% of the cases and regarding the metastatic tumors it was not possible to obtain the classification in 35.1 % of the cases. Men presented more frequently tumors of ma- lignant behavior such as glioblastoma NOS (not otherwise specified) (14.9 %) while in women benign behavior tumors such as meningioma predominated (23.2 %). For population older than 65 years old, both glioblastoma NOS and meningioma were more frequent in women with 17.4 % and 28.3 %, respectively. In patients with CNS metastatic tumors, according to the topographic classification, the most frequent primary tumors were lung (39.4 %), followed by breast (17 %). Its origin was not iden- tified in 11.7 % of the cases. The most identified histology was adenocarcinoma (14.9 %), followed by carcinoma (8.5 %). Conclusion: For disease surveillance, it is necessary to complete epidemiological and clinical monitoring of primary and metastatic tumors of the CNS by using institutional cancer registries including topographic, histological and molecular data according to availability.