(1) Background: We aimed to analyze temporal trends in hospitalization and in-hospital mortality (IHM) in patients with sarcoidosis in Spain from 2001−2020. (2) Methods: Using the Spanish National ...Hospital Discharge Database, we included patients (aged ≥ 20 years) hospitalized with a sarcoidosis code in any diagnostic field. (3) Results: We included 44,195 hospitalizations with sarcoidosis (56.34% women). The proportion of women decreased over time, from 58.76% in 2001 and 2002 to 52.85% in 2019 and 2020 (p < 0.001). The crude rates per 100,000 inhabitants increased by 4.02% per year among women and 5.88% among men. These increments were confirmed using Poisson regression analysis, which yielded an IRR of 1.03; 95% CI 1.01−1.04 for women and 1.04; 95% CI 1.02−1.06 for men. During the study period, no significant sex differences in IHM were recorded. Older age, COVID-19, respiratory failure, and the need for mechanical ventilation were independent predictors of IHM in men and women hospitalized with sarcoidosis, with IHM remaining stable over time. (4) Conclusions: The number of hospital admissions among patients with sarcoidosis in Spain increased threefold from 2001 to 2020. Although the incidence rates were higher in women, the trend followed that the incidence rates between sexes became closer. IHM was similar among men and women, with no significant change over time in either sex after multivariable analysis.
(1) Background: To assess sex differences in the incidence, characteristics, procedures and outcomes of patients admitted with idiopathic pulmonary fibrosis (IPF); and to analyze variables associated ...with in-hospital mortality (IHM). (2) Methods: We analyzed data collected by the Spanish National Hospital Discharge Database, 2016–2019. (3) Results: We identified 13,278 hospital discharges (66.4% men) of IPF (primary diagnosis 32.33%; secondary diagnosis: 67.67%). Regardless of the diagnosis position, IPF incidence was higher among men than women, increasing with age. Men had 2.74 times higher IPF incidence than women. Comorbidity was higher for men in either primary or secondary diagnosis. After matching, men had higher prevalence of pulmonary embolism and pneumonia, and women of congestive heart failure, dementia, rheumatoid disease and pulmonary hypertension. Invasive ventilation, bronchoscopy and lung transplantation were received more often by men than women. IHM was higher among men with IPF as primary diagnosis than among women and increased with age in both sexes and among those who suffered cancer, pneumonia or required mechanical ventilation. (4) Conclusions: Incidence of IPF was higher among men than women, as well as comorbidity and use of bronchoscopy, ventilation and lung transplantation. IHM was worse among men than women with IPF as primary diagnosis, increasing with age, cancer, pneumonia or mechanical ventilation use.
(1) Background: This paper aims to assess temporal trends (2016-2020) in incidence, patient's characteristics, complications, length of hospital stay (LOHS) and in-hospital mortality (IHM) among ...patients with and without idiopathic pulmonary fibrosis (IPF) undergoing lung transplantation (LTx). We also analyse the effect of the COVID-19 pandemic on LTx in these populations. (2) Methods: A retrospective, population-based observational study was conducted using the Spanish National Hospital Discharge Database. Multivariable adjustment was conducted with logistic regression to analyse the IHM. (3) Results: We identified 1777 admissions for LTx during the study period, of which 573 (32.2%) were performed in patients with IPF. The number of hospital admissions for LTx rose from 2016 to 2020, both in patients with and without IPF, but a marked reduction was observed from year 2019 to year 2020. Over time, the proportion of single LTx decreased and bilateral LTx increased significantly in both groups. The incidence of LTx complications increased significantly over time along with the increase in the incidence of IPF. No significant differences in the incidence of complications or in the IHM between patients with and without IPF were found. Suffering any complication of the LTx and pulmonary hypertension were conditions positively associated with IHM in patients with and without IPF. The IHM remained stable from 2016 to 2020 in both study populations and was not affected by the COVID pandemic. (4) Conclusions: Patients with IPF account for almost a third of all lung transplants. The number of LTx increased over time in patients with and without IPF, but a marked reduction was observed from 2019 to 2020. Although the proportion of LTx complications increased significantly over time in both groups, the IHM did not change. IPF was not associated with increased complications or IHM after LTx.
The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory ...impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients.
A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis.
poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests.
Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval CI. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and CI).
79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 19.9-28.8, and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 0.34-0.83), age (HR: 1.04 1.01-1.07), and Ro-antibodies (HR: 0.36 0.19-0.65) influenced the prognosis.
IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF.
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.
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.
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.
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.
ObjectivesReal-world data regarding rheumatoid arthritis (RA) and its association with interstitial lung disease (ILD) is still scarce. This study aimed to estimate the prevalence of RA and ILD in ...patients with RA (RAILD) in Spain, and to compare clinical characteristics of patients with RA with and without ILD using natural language processing (NLP) on electronic health records (EHR).MethodsObservational case–control, retrospective and multicentre study based on the secondary use of unstructured clinical data from patients with adult RA and RAILD from nine hospitals between 2014 and 2019. NLP was used to extract unstructured clinical information from EHR and standardise it into a SNOMED-CT terminology. Prevalence of RA and RAILD were calculated, and a descriptive analysis was performed. Characteristics between patients with RAILD and RA patients without ILD (RAnonILD) were compared.ResultsFrom a source population of 3 176 165 patients and 64 241 683 EHRs, 13 958 patients with RA were identified. Of those, 5.1% patients additionally had ILD (RAILD). The overall age-adjusted prevalence of RA and RAILD were 0.53% and 0.02%, respectively. The most common ILD subtype was usual interstitial pneumonia (29.3%). When comparing RAILD versus RAnonILD patients, RAILD patients were older and had more comorbidities, notably concerning infections (33.6% vs 16.5%, p<0.001), malignancies (15.9% vs 8.5%, p<0.001) and cardiovascular disease (25.8% vs 13.9%, p<0.001) than RAnonILD. RAILD patients also had higher inflammatory burden reflected in more pharmacological prescriptions and higher inflammatory parameters and presented a higher in-hospital mortality with a higher risk of death (HR 2.32; 95% CI 1.59 to 2.81, p<0.001).ConclusionsWe found an estimated age-adjusted prevalence of RA and RAILD by analysing real-world data through NLP. RAILD patients were more vulnerable at the time of inclusion with higher comorbidity and inflammatory burden than RAnonILD, which correlated with higher mortality.
Alveolar Proteinosis in COVID-19: Clinical Case Martin Bote, Silvia; Herrera Morueco, Maria Angeles; Arias Arcos, Beatriz ...
Case reports in pulmonology,
2022, Volume:
2022
Journal Article
Peer reviewed
Open access
Pulmonary alveolar proteinosis (PAP) is a rare, diffuse lung disease characterized by accumulation of lipoprotein in lung surfactant in the alveolar space and terminal bronchioles, leading to ...impaired gas exchange and arterial hypoxemia. We present the case of a 51-year-old woman who was admitted with a diagnosis of severe SARS-CoV-2 pneumonia. Her condition did not improve with corticosteroids. A chest CT scan revealed ground-glass opacities in all lung lobes, with septal thickening. A differential diagnosis was proposed with other diseases. Bronchoscopy revealed milky bronchoalveolar lavage fluid, and staining with periodic acid–Schiff was positive, thus indicating PAP. Therefore, the patient underwent whole lung lavage, which led to clinical, radiological, and functional improvement. In the context of the COVID-19 pandemic, differential diagnosis ensures that appropriate attention is given to less prevalent entities such as PAP.
Telomeres are nucleoprotein structures present at the terminal region of the chromosomes. Mutations in genes coding for proteins involved in telomere maintenance are causative of a number of ...disorders known as telomeropathies. The genetic origin of these diseases is heterogeneous and has not been determined for a significant proportion of patients.
This article describes the genetic characterization of a cohort of patients. Telomere length was determined by Southern blot and quantitative PCR. Nucleotide variants were analyzed either by high-resolution melting analysis and Sanger sequencing of selected exons or by massive sequencing of a panel of genes.
Forty-seven patients with telomere length below the 10% of normal population, affected with three telomeropathies: dyskeratosis congenita (4), aplastic anemia (22) or pulmonary fibrosis (21) were analyzed. Eighteen of these patients presented known pathogenic or novel possibly pathogenic variants in the telomere-related genes TERT, TERC, RTEL1, CTC1 and ACD. In addition, the analyses of a panel of 188 genes related to haematological disorders indicated that a relevant proportion of the patients (up to 35%) presented rare variants in genes related to DNA repair or in genes coding for proteins involved in the resolution of complex DNA structures, that participate in telomere replication. Mutations in some of these genes are causative of several syndromes previously associated to telomere shortening.
Novel variants in telomere, DNA repair and replication genes are described that might indicate the contribution of variants in these genes to the development of telomeropathies. Patients carrying variants in telomere-related genes presented worse evolution after diagnosis than the rest of patients analyzed.
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