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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.

(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.

(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.

[Background]: 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., [Methods]: 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., [Results]: 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., [Conclusion]: 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., Funded by grants PI14–01495 and PI17–01401 (Fondo de Investigaciones Sanitarias, Instituto de Salud Carlos III, Spain supported by FEDER funds) and by one ACCI project from CIBERER and one grant to the FPI cohort from CIBERES., We acknowledge support of the publication fee by the CSIC Open Access Publication Support initiative through its Unit of Information Resources for Research (URICI).

Introduction: REFIPIMAD registry was created to known the epidemiological and clinical characteristics of idiopathic pulmonary fibrosis patients (IPF) in the community of Madrid (Spain). Aim: To describe the clinical, safety and tolerability characteristics of IPF patients included in REFIPIMAD registry and treated with Nintedanib in real life. Results: 44 patients are described, mean age 66 ±7.9 years (range 79-44), 35 (79%) were men, 30 (68%) were smokers or ex-smokers with an average of 32 paq/year. Comorbidity: Previous AMI in 4 patients, 6 diabetes mellitus, 3 peptic ulcer and 1 peripheral arteriopathy. The HRCT radiological pattern was UIP in 28 (64%), possible UIP in 14 (32%) and 9 (20%) had associated emphysema. Surgical lung biopsy was performed in 14 patients. Pulmonary function tests at the beginning of the treatment were (mean ± SD [range]) FVC 83% ± 18 (55%-122%) of the predicted value, DLCO 59% ± 17 (34%-103%) of the predicted value, six-minute walk distance 479 ± 98 (98-479) metres, initial SpO2 94 ± 1.7, final SpO2 87 ± 4.4. The average treatment time was 10.3 ± 11.3 months (range 44.93-0.43); 20 patients (50%) suffer some side effects: diarrhea 15 (36%) , weight loss 9 (21%), other gastro-intestinal alterations 15 (12%), liver disorders 5 (12%), hiporexia 6 (14%) and other effects 3 (4%). The treatment was definitively withdrawn in 4 patients (16%) who switched to pirfenidone and other 3 with pirfenidone switched to Nintedanib. Pulmonary function test follow-up are available in 16 patients who showed stability. Conclusion: Preliminary data of Nintedanib treatment in real life show a good safety profile and data consistent with those published in clinical trials.