Moderate-High Blood Eosinophilia Is Associated with Increased Hospitalization and Other Asthma Comorbidities.

(1) Background: Eosinophilia has traditionally been linked to eosinophilic asthma, for which it is the gold-standard prognostic biomarker. However, the association between eosinophilia and the presence of other diseases and comorbidities is yet unclear. (2) Methods: For this retrospective study, we reviewed the electronic medical records of 49,909 subjects with blood eosinophilia to gather data on the presence of asthma, COPD, sleep apnea, tuberculosis, dyslipidemia, hypertension, and other cardiovascular diseases and severe CRSwNP among these subjects. Demographic features including age, sex, and smoking habits were collected, as well as the number of hospitalizations and emergency department visits. T-tests, ANOVA, Fisher test, and logistic regression models were used. (3) Results: For all age groups studied, eosinophilia was significantly more prevalent among asthmatic subjects than nonasthmatics, especially in patients also presenting CRSwNP, hypertension, and dyslipidemia. The likelihood of developing asthma, COPD, and CRSwNP, and hospitalization, was increased when BEC was above 600 eosinophils/µL. The association between asthma, CRSwNP, and BEC was corroborated by multiple logistic regressions models. (4) Conclusions: We demonstrated the association of having over 600 blood eosinophils/µL with a higher number of hospitalizations and comorbidities (CRSwNP and COPD), which proves that BEC is a highly useful parameter to consider in subjects who present blood eosinophilia.

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).

Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry.

BACKGROUND: 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. METHODS: 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. MAIN OUTCOME: 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. STATISTICS: 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]). RESULTS: 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. CONCLUSIONS: 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.

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].

Education and Use of Lung Ultrasound in Rheumatology and Pneumology in Spain: A SER-SEPAR Survey.

INTRODUCTION: Lung ultrasound (LUS) is a clinical and research tool with great potential in the diagnosis and monitoring of diffuse interstitial lung disease (ILD) present in systemic autoimmune diseases (SAD). Appropriate training in LUS is essential for the correct and safe use of this technique. OBJECTIVE: To document the current state of LUS education and use among Spanish rheumatologists and pneumologists. MATERIAL AND METHODS: A national online survey was designed for members of the Spanish Society of Rheumatology and the ILD Area of the Spanish Society of Pneumology and Thoracic Surgery. The survey consisted of 22 questions on demographics, professional activity, performance and training in LUS. RESULTS: One hundred and thirty-five (56.72% rheumatologists, 41.79% pneumologists) responded to the survey. Of these, 56.30% were part of an ILD Unit in their centre. LUS in clinical practice was performed by 35.82% but only 14.93% performed it in ILD, mainly for diagnostic purposes. Training in LUS of responders had been diverse in format, content and sponsors. The vast majority (87.79%) considered that the optimal model of education in LUS should be standardized and structured and consist of a combination of theoretical-practical courses and the conduct of a minimum number of supervised LUS examinations, with competency assessment. CONCLUSIONS: The current lack of formal structured education in LUS is an opportunity to develop quality educational programmes in this emerging field.

[Management of Interstitial Lung Diseases (ILD) Associated with Autoimmune Diseases by the Pulmonologyst in the Differents ILD Units in Spain]. / Manejo de las enfermedades pulmonares intersticiales difusas (EPID) asociadas a enfermedades autoinmunes, por el neumólogo en las diferentes unidades de EPID en España.

Introduction: The aim of the study was to know the management of patients with diffuse interstitial lung disease (ILD) associated with a systemic autoimmune diseases (SAD) in pulmonology outpatient clinics in Spain. Methodology: The ILD work area of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) designed a self-completed questionnaire of 25 questions, on aspects related to the diagnosis and treatment of ILD-SAD. This was distributed among the attendees of the winter meeting of the ILD Area and later via e-mail to all the members of the ILD area of SEPAR. Participation was anonymous, voluntary and without consideration. Results: 74 pulmonologists from 58 hospitals participated. 77% had a specialized ILD consultation. All Units with SEPAR accreditation had a committee made up of pulmonologists and radiologists and a majority participation of pathologists and rheumatologists. In 75% of the centers there was a close collaboration with Rheumatology for the management of ILD-SAD. 85% considered that the frequency of ILD-SAD consults is increasing, the most frequent being ILD associated with rheumatoid arthritis. The treatment of ILD-SAD is decided by consensus between pulmonologist and rheumatologist in 91.3% of the cases. 67% of pulmonologists consider that immunosuppressants and biological therapies can slow down the progression of ILD-SAD. 51% use antifibrotics therapies in these pathologies. Conclusions: Almost all of the accredited Spanish ILD Units by SEPAR have established collaborations with Rheumatology for the adequate management of patients with ILD-SAD, this practice having been extended to units not yet accredited.

Education and Use of Lung Ultrasound in Rheumatology and Pneumology in Spain: A SER-SEPAR Survey. / Educación y uso de la ecografía pulmonar en reumatología y neumología en España: encuesta SER-SEPAR.

INTRODUCTION: Lung ultrasound (LUS) is a clinical and research tool with great potential in the diagnosis and monitoring of diffuse interstitial lung disease (ILD) present in systemic autoimmune diseases (SAD). Appropriate training in LUS is essential for the correct and safe use of this technique. OBJECTIVE: To document the current state of LUS education and use among Spanish rheumatologists and pneumologists. MATERIAL AND METHODS: A national online survey was designed for members of the Spanish Society of Rheumatology and the ILD Area of the Spanish Society of Pneumology and Thoracic Surgery. The survey consisted of 22 questions on demographics, professional activity, performance and training in LUS. RESULTS: One hundred and thirty-five (56.72% rheumatologists, 41.79% pneumologists) responded to the survey. Of these, 56.30% were part of an ILD Unit in their centre. LUS in clinical practice was performed by 35.82% but only 14.93% performed it in ILD, mainly for diagnostic purposes. Training in LUS of responders had been diverse in format, content and sponsors. The vast majority (87.79%) considered that the optimal model of education in LUS should be standardized and structured and consist of a combination of theoretical-practical courses and the conduct of a minimum number of supervised LUS examinations, with competency assessment. CONCLUSIONS: The current lack of formal structured education in LUS is an opportunity to develop quality educational programmes in this emerging field.

Compassionate use of tocilizumab in severe SARS-CoV2 pneumonia.

INTRODUCTION: Tocilizumab (TCZ) is an interleukin-6 receptor antagonist, which has been used for the treatment of severe SARS-CoV-2 pneumonia (SSP), which aims to ameliorate the cytokine release syndrome (CRS) induced acute respiratory distress syndrome (ARDS). However, there are no consistent data about who might benefit most from it. METHODS: We administered TCZ on a compassionate-use basis to patients with SSP who were hospitalized (excluding intensive care and intubated cases) and who required oxygen support to have a saturation >93%. The primary endpoint was intubation or death after 24 h of its administration. Patients received at least one dose of 400 mg intravenous TCZ from March 8, 2020 to April 20, 2020. RESULTS: A total of 207 patients were studied and 186 analyzed. The mean age was 65 years and 68% were male patients. A coexisting condition was present in 68% of cases. Prognostic factors of death were older age, higher IL-6, d-dimer and high-sensitivity C-reactive protein (HSCRP), lower total lymphocytes, and severe disease that requires additional oxygen support. The primary endpoint (intubation or death) was significantly worst (37% vs 13%, p < 0·001) in those receiving the drug when the oxygen support was high (FiO2 >0.5%). CONCLUSIONS: TCZ is well tolerated in patients with SSP, but it has a limited effect on the evolution of cases with high oxygen support needs.

Anthracofibrosis or anthracostenosis.

Anthracofibrosis is a bronchial stenosis due to local mucosal fibrosis that also presents anthracotic pigment in the mucosa. The cause has not been well clarified, although there is a frequent association with tuberculosis and the exposure to smoke from biofuel or biomass combustion. It is an entity that has not been reported in Spain, although the influx of people from rural areas of developing countries or rural areas of our own country should make us contemplate this entity in the differential diagnosis of our patients. We present 3 cases detected in Spain (2 of them natives) diagnosed by bronchoscopy and bronchial biopsy, which are techniques necessary to confirm the diagnosis. There is no specific treatment, except for tuberculostatic treatment in cases with coexisting tuberculosis.

[Pulmonary paragonimiasis]. / Paragonimiasis pulmonar.

Paragonimiasis is a food-borne zoonosis caused by a trematode of the genus Paragonimus(1,2). Infestation is rare in Spain, but the influx of people from endemic areas should make us keep this condition in the differential diagnosis of our patients(2,5). We report the case a patient from Ecuador and resident in Spain for 7 years with active pulmonary tuberculosis on arrival in Spain and later diagnosed with of pulmonary paragonimiasis due to persistent haemoptysis. The diagnosis was established by surgical lung specimen showing granulomas containing parasite eggs and the macroscopic view of the fluke within a lung cavity. Initial tuberculosis treatment and current treatment with praziquantel controlled both conditions.