Antifibrotic treatment adherence, efficacy and outcomes for patients with idiopathic pulmonary fibrosis in Spain: a real-world evidence study.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a rare disorder associated with increased mortality and morbidity. There are currently two drugs approved for IPF but their safety and efficacy profile in real-world settings in Spain is not well understood. METHODS: An observational, multicentre, prospective study was carried out among patients with IPF who started treatment with pirfenidone or nintedanib from 2015 to 2021. Data regarding clinical characteristics, drug adherence, safety profiles and clinical outcomes between these two drugs were collected. RESULTS: 232 patients were included in the analysis. There were no meaningful differences between both groups at baseline. Patients who started pirfenidone showed a decreased risk for treatment withdrawal compared with those starting nintedanib (HR 0.65 (95% CI 0.46 to 0.94; p=0.002)). Time to first adverse event and all-cause mortality was similar between study groups. Risk factors for withdrawal were female sex, diarrhoea and photosensitivity. CONCLUSIONS: in this real-world study, both pirfenidone and nintedanib showed similar efficacy profiles. Pirfenidone was associated with less treatment discontinuations due to side effects.

Impact of the implementation of a telemedicine program on patients diagnosed with asthma.

BACKGROUND: Asthma is one of the most common respiratory ailments worldwide. Despite broad understanding of the illness and of the available therapeutic options for it, patients with serious asthma suffer poor monitoring of their illness in 50% of cases. AIM: To assess the impact of the implementation of a mobile application (ESTOI) to control asthma in patients diagnosed with the illness, their adherence to treatment, and their perceived quality of life. METHODOLOGY: Randomized clinical trial with 52 weeks' follow-up of patients with asthma seen in a specialized hospital for their treatment in Spain. Some 108 included patients will be divided into two groups. The intervention group will undergo more exhaustive follow-up than normal, including access to the ESTOI application, which will have various categories of attention: control of symptoms, health recommendations, current treatment and personalized action plan, PEF record, nutritional plan, and chat access with a medical team. The asthma control questionnaire ACT is the main assessment variable. Other variables to be studied include an adherence test for the use of inhalers (TAI), the number of exacerbations, maximum exhalation flow, exhaled nitric oxide test, hospital anxiety and depression scale, asthma quality-of-life questionnaire, forced spirometry parameters (FVC, FEV1, and PBD), and analytic parameters (eosinophilia and IGE). The data will be collected during outpatient visits. TRIAL REGISTRATION: This trial has registered at ClinicalTrials.gov (Identifier: NCT06116292).

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).

Abnormal Alpha-1 Antitrypsin Levels and Other Risk Factors Associated with Lung Function Impairment at 6 and 12 Months after Hospitalization Due to COVID-19: A Cohort Study.

Respiratory function deficits are common sequelae for COVID-19. In this study, we aimed to identify the medical conditions that may influence lung function impairment at 12 months after SARS-CoV2 infection and to analyze the role of alpha-1 antytripsin (AAT) deficiciency (AATD). A cohort study was conducted on hospitalized COVID-19 pneumonia patients in Granada (Spain) during the first infection wave who were referred to a post-COVID-19 hospital clinic. The patients were monitored with three follow-up visits from May 2020 to May 2021. Previous medical history, hospital admission data, baseline parameters and physical examination data were collected at the first visit. Pulmonary function tests were performed at 6 and 12 months together with the determination of AAT level and AATD genotype. After 12 months, 49 out of 157 patients (31.2%) continued to have lung function impairment. A multivariate analysis showed a statistically significant association of lung function impairment with: higher Charlson index; pneumonia with a central and/or mixed distribution; anemia on admission; time in intensive care; need for corticosteroid boluses; abnormal respiratory sounds at 6 months; elevated lactate dehydrogenase at 12 months; abnormal AAT; and MZ genotype. Our results suggest that these medical conditions predispose COVID-19 patients to develop long-term lung function sequelae.

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].

Infectious Exacerbations of Idiopathic Pulmonary Fibrosis

No disponible

On the single and multiple associations of COVID-19 post-acute sequelae: 6-month prospective cohort study.

Medical research is progressing to clarify the full spectrum of sub-acute and long-term effects of the post-COVID-19 syndrome. However, most manuscripts published to date only analyze the effects of post-COVID-19 in patients discharged from hospital, which may induce significant bias. Here, we propose a pioneering study to analyze the single and multiple associations between post-COVID-19 characteristics with up to 6-months of follow-up in hospitalized and non-hospitalized COVID-19 patients. The cohort study was conducted from May to October 2020 at the University Hospital Virgen de la Nieves, the leading hospital assigned for patients with COVID-19 in Granada, Spain. A total of 372 and 217 patients-with 217 and 207 included in the first and second follow-up visits-were referred 2 and 6 months after diagnosing COVID-19, respectively. We find out that post-COVID-19 clinical and mental health impairment symptoms are correlated with patient gender. Logistic adjustments showed strong statistically robust single and multiple associations of demographic, clinical, mental health, X-ray, laboratory indices, and pulmonary function variables. The functional lung tests are good predictors of chest CT imaging abnormalities in elderly patients. Bilateral lung involvement, subpleural reticulum, ground-glass opacity, peripheral lung lesions, and bronchiectasis were the most common findings of the high-resolution computed tomography images. Non-hospitalized patients suffer more severe thromboembolic events and fatigue than those hospitalized.

Small cell lung cancer: Recent changes in clinical presentation and prognosis.

BACKGROUND: Small cell lung cancer (SCLC) is a leading cause of death all over the world. Diagnostic and therapeutic arsenals have improved in recent years, but we are unsure as to whether these advances have been transferred to clinical practice. The aim of this study was to evaluate differences in SCLC diagnostic processes and short-term survival rates between two recent cohorts. METHODS: A prospective, observational study was conducted with patients diagnosed with SCLC (either at extensive or limited stages) in the 2011-2016 period. Patients were divided into two cohorts (2011-2013 and 2014-2016) and followed up for 1 year after diagnosis. RESULTS: Around 713 patients with lung cancer were selected, 134 of whom had SCLC (74 patients in the 2011-2013 cohort and 60 in the 2014-2016 cohort). We observed a chronological increase in the use of endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) and positron emission tomography-computed tomography (PET-CT) between the cohorts. Overall, short-term survival was similar between the two groups and improved survival was associated with age and limited stage. CONCLUSIONS: Changes in diagnostic process in SCLC have been observed towards a more precise stadification. Although short-term survival has not changed for SCLC, it is unclear that the real benefit of PET-CT and EBUS-TBNA is far from correct disease staging.

Edema agudo de pulmón por altura tras 48 horas de estancia en una estación de esquí / High-Altitude Acute Pulmonary Edema after 48 Hours in a Ski Station

No disponible

Changes in non-small cell lung cancer diagnosis, molecular testing and prognosis 2011-2016.

BACKGROUND: Non-small cell lung cancer (NSCLC) is a leading cause of death all over the world. Diagnostic and therapeutic arsenals have improved in recent years, but we are unsure as to whether these advances have been transferred to clinical practice. The aim of this study was to evaluate differences in NSCLC diagnostic processes and short-term survival rates between two recent cohorts. METHODS: A prospective, observational study was conducted with patients diagnosed with NSCLC in the period of 2011-2016. Patients were divided into two cohorts (2011-2013 and 2014-2016), and monitored for up to 1 year after diagnosis. RESULTS: A total of 713 patients with lung cancer were selected, 500 of whom had NSCLC (222 patients in the 2011-2013 cohort, and 278 in the 2014-2016 cohort). We observed a chronological increase in the use of endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) and ultrasound-guided transthoracic puncture (US-TTP) between the cohorts. Overall short-term survival was similar between the two groups, both for locally and for advanced disease. Treatment with tyrosine kinase inhibitors (TKI) was the only therapeutic factor associated with an improved likelihood of survival. CONCLUSIONS: Changes in diagnostic process in NSCLC have been observed towards a more precise stratification. Although short-term survival has not changed for advanced NSCLC, some of the newer therapeutic options are associated with increased survival in real-world scenarios.

Reacción sistémica y afectación mediastínica secundaria a rotura de implante protésico de mama / Systemic reaction and mediastinal involvement secondary to breast implant rupture

No disponible

Cuidados paliativos en la enfermedad pulmonar intersticial difusa: resultados de una encuesta de ámbito nacional / Palliative Care in Diffuse Interstitial Lund Disease: Results of a Spanish Survey

Introducción: Las enfermedades pulmonares intersticiales difusas (EPID) y, concretamente, la fibrosis pulmonar idiopática, pueden tener un elevado impacto en la supervivencia de los pacientes. Recientes estudios destacan la necesidad de implementar los cuidados paliativos (CP) en el manejo del enfermo con EPID. El objetivo del estudio fue conocer la situación actual de los CP en nuestro país. Métodos: Se diseñó una encuesta de 36 preguntas, que abordaba los principales aspectos de los CP en el paciente con EPID. Esta encuesta fue remitida a través de correo electrónico a todos los miembros de la Sociedad Española de Neumología y Cirugía Torácica, cuya participación fue voluntaria. Resultados: Cientosesenta y cuatro participantes respondieron a la encuesta. El 98% manifestó tener interés en los CP, 46% habían recibido formación específica. El 44% refirió ser el responsable de los CP en sus pacientes EPID. El control de síntomas y la fase final de vida fueron los motivos más frecuentes de derivación a los equipos de CP. Referente a la fase final de vida el 78% refirió consensuar con los pacientes la limitación del esfuerzo terapéutico, el 35% realizar un documento de voluntades anticipadas y el 22% consensuar el lugar de fallecimiento. Conclusión: A pesar de la conocida necesidad del CP en los pacientes con EPID y el notable interés de los participantes de la encuesta en este tema, existen claras lagunas formativas y organizativas, que deberían ser contempladas para mejorar la atención sobre esta área de salud en los pacientes con EPID de nuestro país (AU)

Introduction: Interstitial lung diseases (ILD) and, in particular, idiopathic pulmonary fibrosis, may have a significant impact on patient survival. Recent studies highlight the need for palliative care (PC) in the management of ILD patients. The aim of this study was to determine the current situation of PC in patients in Spain. Methods: A 36-question survey addressing the main aspects of PC in ILD patients was designed. The survey was sent via email to all members of the Spanish Society of Pulmonology and Thoracic Surgery. Participation was voluntary. Results: One hundred and sixty-four participants responded to the survey. Ninety-eight percent said they were interested in PC, 46% had received specific training, and 44% reported being responsible for PC in their ILD patients. Symptom control and end-of-life stage were the most frequent reasons for referral to PC teams. Regarding end-of-life, 78% reported consensual agreement with patients on the limitation of therapeutic efforts, 35% helped prepare an end-of-life advance directive, and 22% agreed on the place of death. Conclusion: Despite the well-known need for PC in patients with ILD and the notable interest of the survey participants in this subject, there are clear formative and organizational gaps that should be addressed to improve care in this area in ILD patients in Spain (AU)

[Epidemiological Evolution of Lung Cancer in the South of Spain from 1990 to 2010].

BACKGROUND: Changes in lung cancer has been characterized by the increase of cases among women and the increase in adenocarcinomas among other histological subtypes. METHODS: Descriptive analysis of cases diagnosed with lung cancer in Hospital Virgen de las Nieves (Spain) from 1990 to 2010, based on five variables (age, sex, smoking, histology and pathological anatomy). The study establishes associations between these variables and compares the results with the literature. RESULTS: 2,026 patients were diagnosed with lung cancer in this period; 1,838 were males (90.7%) and 188 women (9.3%); 1,892 patients (93.4%) were smokers or ex-smokers and 134 (6.6%) had never smoked; the most frequent non-small cell histology types were squamous cell carcinoma and adenocarcinoma and it was the most frequent neoplasia in women and were associated with a lower tobacco consumption. CONCLUSIONS: The large majority of lung cancer cases is associated with a history of smoking tobacco and there are histopathological differences according to gender and cumulative tobacco smoke load.

Palliative Care in Diffuse Interstitial Lund Disease: Results of a Spanish Survey. / Cuidados paliativos en la enfermedad pulmonar intersticial difusa: resultados de una encuesta de ámbito nacional.

INTRODUCTION: Interstitial lung diseases (ILD) and, in particular, idiopathic pulmonary fibrosis, may have a significant impact on patient survival. Recent studies highlight the need for palliative care (PC) in the management of ILD patients. The aim of this study was to determine the current situation of PC in patients in Spain. METHODS: A 36-question survey addressing the main aspects of PC in ILD patients was designed. The survey was sent via email to all members of the Spanish Society of Pulmonology and Thoracic Surgery. Participation was voluntary. RESULTS: One hundred and sixty-four participants responded to the survey. Ninety-eight percent said they were interested in PC, 46% had received specific training, and 44% reported being responsible for PC in their ILD patients. Symptom control and end-of-life stage were the most frequent reasons for referral to PC teams. Regarding end-of-life, 78% reported consensual agreement with patients on the limitation of therapeutic efforts, 35% helped prepare an end-of-life advance directive, and 22% agreed on the place of death. CONCLUSION: Despite the well-known need for PC in patients with ILD and the notable interest of the survey participants in this subject, there are clear formative and organizational gaps that should be addressed to improve care in this area in ILD patients in Spain.

Síndrome de Hermansky-Pudlak: descripción de un caso / Hermansky-Pudlak syndrome: A case report

No disponible