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Resumen: Objetivo: El objetivo de este estudio fue analizar el proceso diagnóstico de los pacientes con fibrosis pulmonar idiopática en España, desde el inicio de los síntomas hasta el diagnóstico y tratamiento antifibrótico, en relación con la publicación de las sucesivas guías de práctica clínica. Material y métodos: Estudio multicéntrico, observacional, ambispectivo, en el que se analizaron los pacientes incluidos en el registro de la fibrosis pulmonar idiopática de la Sociedad Española de Neumología y Cirugía Torácica. Para ello se habilitó un cuaderno electrónico de recogida de datos en la web de la sociedad. Se recogieron variables sociodemográficas y clínicas al diagnóstico y seguimiento de los pacientes. Resultados: Desde enero de 2012 hasta diciembre de 2019 se incluyeron 1.064 pacientes, siendo finalmente analizados 929. El proceso diagnóstico varió en función del año en el que se realizó el diagnóstico y el patrón radiológico observado en la tomografía computarizada de alta resolución. En 244 (26,3%) pacientes, el diagnóstico se realizó con tomografía computarizada de alta resolución de tórax y evaluación clínica. La biopsia quirúrgica se utilizó hasta en el 50,2% de los casos diagnosticados antes del 2011, y en un 14,2% a partir de 2018. La mediana de tiempo que transcurre desde el inicio de los síntomas hasta el diagnóstico es de 360 días (RIC 120-720), siendo mayor de 2 años en el 21,0% de los pacientes. Recibieron tratamiento antifibrótico al 79,4% de los pacientes. El tiempo desde el diagnóstico hasta el inicio del tratamiento fue de 309 ± 596,5 días, con una mediana de 49 (RIC 0-307). Conclusiones: El proceso diagnóstico, incluyendo el tiempo hasta el diagnóstico y el tipo de pruebas utilizadas, ha ido cambiando desde 2011 hasta 2019, probablemente debido el avance en la investigación clínica y la publicación de guías consenso diagnóstico-terapéuticas. Abstract: 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.

Objectives: To evaluate the treatment modalities and their effects in primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD)., Methods: In this chart review study, patients diagnosed with pSS-related ILD (pSS-ILD) between January 2004 and August 2022 were screened. Glucocorticoid use and administered disease-modifying antirheumatic drugs (DMARDs) were determined. The difference between forced vital capacity (FVC) and diffusion capacity of the lungs for carbon monoxide (DLCO) before and after treatment was evaluated., Results: ILD was present in 44 of 609 patients (7.2%) diagnosed with pSS. In 27 patients included in the study, steroid usage was 81.5%. There was a statistically insignificant increase in FVC% (from 80.20±22.1 to 81.6±23.0) and a decrease in DLCO% (53.7±15.3-52.2±19.3) with DMARD treatment (p=0.434 and p=0.652, respectively). There was no significant difference between the treatment groups (azathioprine [AZA], mycophenolate mofetil [MMF], and rituximab [RTX]) in terms of the change in FVC% and DLCO% compared with baseline levels. The effect of treatment on FVC and DLCO was similar in UIP and NSIP patterns., Conclusions: AZA, MMF, and RTX have similar effects on pulmonary functions in pSS-ILD and provide disease stabilization., (Copyright © 2024 Elsevier España, S.L.U. All rights reserved.)

Introduction: Krebs von den Lungen 6 (KL-6) is a mucin-1 glycoprotein produced by type II pneumocytes. High levels of KL-6 in blood may be found in patients with lung fibrosis. In Asia this biomarker is used for diagnosis and prognosis in interstitial lung diseases (ILD). There is a lack of information regarding KL-6 cut-off point for diagnosis and prognosis in European population. The aim of this study was to establish the cut-off point for serum KL-6 associated with the presence of ILD in the Spanish population., Methods: Prospective study including subjects who underwent chest HRCT, PFTs and autoimmune blood analysis. Two groups were created: non-ILD subjects and ILD patients. Serum KL-6 concentrations were measured using a Lumipulse KL-6 reagent assay and the optimal cut-off value was evaluated by a ROC analysis. Data on demographics and smoking history was also collected., Results: One hundred seventy-nine patients were included, 102 with ILD. Median serum KL-6 values overall were 762U/mL, 1080 (±787)U/mL for the ILD group vs 340 (±152)U/mL for the non-ILD group (p<0.0001). The main radiological pattern was NSIP (43%). ROC analysis showed greater specificity (86%) and sensitivity (82%) for KL-6 465U/mL for detecting ILD patients. The multivariate logistic regression model pointed to the male sex, higher KL-6 values, lower FVC and low DLCO values as independent factors associated with ILD., Conclusion: Serum KL-6 values greater than 465U/mL have excellent sensitivity and specificity for detecting ILD in our Spanish cohort. Multicentre studies are needed to validate our results., (Copyright © 2024 SEPAR. Published by Elsevier España, S.L.U. All rights reserved.)

Interstitial lung diseases are rare in pediatrics. They include dysfunctions in the metabolism of pulmonary surfactant, an amphipathic molecule that reduces surface tension and prevents alveolar collapse. Here we describe the case of a 6-month-old infant controlled for low weight, who presented with acute respiratory distress and cyanosis; his chest X-ray showed interstitial infiltrate, pneumomediastinum, and bilateral pneumothorax. During history-taking, it was noted that his mother had a history of hospitalization at 1 year old with unknown diagnosis, requiring prolonged oxygen therapy; she now shows signs of chronic hypoxia. The patient was hospitalized and required oxygen therapy. Ancillary tests were done to look for the etiology of the condition, with no positive results. A chest computed tomography showed groundglass opacities, thickening of the septal interstitium, and areas of air trapping; based on the results of a lung biopsy and a genetic study, pulmonary surfactant metabolism dysfunction was diagnosed., (Sociedad Argentina de Pediatría.)

Introduction. Glucocorticoids (GKS) hormones with strong immunosuppressive and anti-inflammatory activity are used, inter alia, in interstitial lung diseases (ILD). GKS inhibit excessive activity of inflammatory genes in the airways to induce apoptosis of immune cells, such as alveolar lymphocytes (AL). Objective. Assessment of cytoimmunological changes including apoptosis occurring in alveolar lymphocytes in patients with selected ILD after treatment with systemic corticosteroids. Methods. The material of the bronchoalveolar lavage (BAL) derived from patients with sarcoidosis (PS, n=66), idiopathic pulmonary fibrosis (IPF, n=27) and non-specific interstitial pneumonia (NSIP, n = 25; adequate number of patients treated with systemic GKS were 23, 8 and 6) were analyzed in cytoimmunological tests: a) percentage and the total values of BAL inflammatory cell populations; b) AL subset typing; c) CD4/CD8 index calculation; d) AL cell cycle analysis (DNA staining with propidium iodide, PI); in techniques mentioned in b) to d) items flow cytometry was used. Results. In all patient groups, treatment with GKS resulted in a decrease in the total cell number, e.g. for PS (untreated: 310 ± 80x103/ml; treated: 188 ± 43x103/ml, median ± SEM, p

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., (© 2024 Sociedad Española de Neumología y Cirugía Torácica (SEPAR). Published by Elsevier España, S.L.U.)

Introduction: Systemic sclerosis is a chronic autoimmune disease, characterized by the triad of small vessel vasculopathy, immune system activation and increased deposits of extracellular matrix in the skin and internal organs. In the last few years, pulmonary involvement has gained relevance since the introduction of angiotensin enzyme converter inhibitors with the subsequent decline in scleroderma renal crisis mortality, transforming scleroderma lung disease in the leading cause of mortality. Pulmonary involvement can manifest as hypertension or interstitial lung disease, which usually occurs in patients with generalized scleroderma in the first three years of the disease. Its prognosis is poor without treatment which is aimed at stopping pulmonary function deterioration. Among treatment options, cyclophosphamide has the best evidence, and mycophenolate mofetil, rituximab, and stem cell and lung transplantation are currently under investigation with positive preliminary results. Objective: To describe, according to reports in the literature, epidemiology, pathophysiology, diagnostic methods, and treatment of interstitial lung disease in systemic sclerosis. Methods: Structured, non-systematic literature review, focused on the aforementioned aspects of interest. It included 52 articles.

Background: ILD is a common manifestation in pSS and is associated with an increased risk of death. APCA are strongly expressed by hyperplastic alveolar epithelial cells in the fibrotic lung and are associated with an accelerated decline in lung function in IPF. In the present study, we aimed to evaluate the clinical utility of APCA in ILD patients with pSS., Methods: Clinical, laboratory, PFTs and imaging data from pSS patients were reviewed, and the ESSDAI was utilized to evaluate disease activity. HRCT semiquantitative scoring was conducted. We compared the clinical characteristics of pSS patients with and without ILD and carried out logistic regression analysis of risk factors for ILD in pSS., Results: A total of 74 patients with pSS and 40 HCs were included in the study. ILD was more commonly observed in the APCA-positive group than in the APCA-negative group. The quantitative levels of APCA were positively correlated with the imaging score. Multivariate analysis found that the long disease duration, elevated APCA and elevated KL-6 level were independent risk factors for ILD in pSS patients. The area under ROC curve for APCA was 0.6618, and the threshold concentration was 153.82ng/ml (sensitivity 45.24%, specificity 87.50%)., Conclusion: APCA level is an independent risk factor and might be a potential biomarker for ILD in patients with pSS., (Copyright © 2023. Published by Elsevier España, S.L.U.)

Introduction: The anti-MDA5-associated autoimmune disease represents a poorly understood entity. The study's objectives were to describe a cohort of interstitial lung disease (ILD) patients who were positive for anti-MDA5 autoantibody and identify clinical risk factors associated with survival., Methods: This single-center cohort study included ILD patients positive for anti-MDA5 autoantibody. Baseline clinical features were registered, and survival analysis was performed to identify risk factors associated with worse survival., Results: Fifty-three ILD-MDA5 positive patients were included; twelve died during follow-up due to rapidly progressive interstitial lung disease (RP-ILD). Dermatological signs of anti-MDA5 (Gottron papules, Gottron sign, palmar papules, V-neck sign, facial dermatomyositis rashes, and skin ulcers) were strongly associated with death secondary to RP-ILD (HR: 3.7, 95% CI: 1.02-13.35). Patients with dermatological signs were younger, had higher anti-MDA5 autoantibodies titers, more frequent inflammatory patterns in HRCT evaluation, and less fibrosis extent in HRCT., Conclusion: Dermatological manifestation in ILD patients to anti-MDA5 autoantibodies are associated with RP-ILD and short-term fatal outcomes. Dermatological signs may identify a subgroup of ILD-positive to anti-MDA5 patients with a high risk of RP-ILD., (Copyright © 2023 Elsevier España, S.L.U. All rights reserved.)

Objective: To develop a checklist to facilitate pharmaceutical care for patients with interstitial lung disease who require or are undergoing treatment with antifibrotic drugs., Method: Five hospital pharmacists developed an initial list of 37 items divided into 4 blocks: (1) First visit, which included general patient data and data from the first treatment; (2) follow-up visits, assessing aspects of the follow-up of the treatment with nintedanib or pirfenidone; (3) telepharmacy, consisting of the evaluation of the inclusion of patients in a program of this type, course of the disease, and identification of the contact with the pharmacy service; (4) non-pharmacological treatment and patient information. To decide its potential inclusion in the checklist, 2 rounds of the Delphi were carried out in which the panelists had to assess the degree of agreement of each proposed item according to its "utility", which was the determining criterion for its inclusion, and its "applicability"., Results: Forty-eight hospital pharmacists were contacted, 30 (63%) agreed in writing to participate, 28 (58%) completed the first round of the Delphi, and 27 (56%) completed the second round. After the first round of the Delphi, the questionnaire was amended and comprised 40 items. Of the 40 items evaluated after the 2 rounds of the Delphi, there were 2 that, based on utility, the participants did not reach consensus for inclusion in the checklist: the one referring to "History of surgical intervention, specifically abdominal surgery in the last 4 weeks" (finally kept on the checklist due to its involvement in the indication of nintedanib) and to make recommendations on "Relaxation". No consensus was reached on their applicability for 2 of the items: "Patient stratification according to the Spanish Society of Hospital Pharmacy (SEFH) chronic patient model" and "Collection of Results Reported by the Patient"., Conclusions: The management of patients with ILD and/or pulmonary fibrosis is complex and requires a multidisciplinary approach where the hospital pharmacist plays a key role, especially, although not only, in monitoring drug treatment. We believe that this checklist can contribute from pharmaceutical care to improving the integrated care of patients with ILD who require or are undergoing treatment with antifibrotic drugs., Competing Interests: Conflicts of interest Marta Calvin Lamas has received funding for scientific training, talks, or scientific advice from the following pharmaceutical companies: AstraZeneca, GlaxoSmithKline, CSL Behring, and Boehringer Ingelheim. Miguel Ángel Calleja Hernández has received funding for medical training, conference presentations, and research from the following pharmaceutical companies: Abbvie, Alexion, Almirall, Amgen, Astellas, AstraZeneca, Bayer, Biogen, Boehringer, Clovis, Genzyme, GSK, Grunenthal, Incyte, Janssen, Jazz, Kyowa, Lilly, Merck, MSD, Novartis, Novo Nordisk, Pfizer, Roche, Sanofi, Seagen, Takeda, TEVA, UCB, and Viiv. Emilio Monte-Boquet has received honorariums or funding from various companies, such as Abbott/Abbvie, Amgen, Astellas, Astra Zeneca, Baxalta, Bayer, Biogen, Bristol-Myers Squibb, Celgene, Chiesi, Eisai, Fresenius, Gilead, GSK, Ipsen, Janssen, Leo Pharma, Lilly, MSD, Merck-Serono, Novartis, Pfizer, Roche, Sanofi, Shire, Theramex, UCB, and ViiV for his participation in education, training, and research projects. Miguel Ángel Rodríguez Sagrado has been remunerated for activities for Abbvie, Advanz, Astellas, Amgen, Astra Zeneca, Biogen, Boehringer Ingelheim, Galapagos, Gilead, GSK, Intercept, Janssen, Leo Pharma, Lundbeck, MerckSerono, MSD, Novartis, Roche, Sandoz, Serono, Tesaro, UCB, Vertex and ViiV. The sponsors have not influenced, other than formal aspects, the design and communication of the scientific results and talks. Pere Ventayol has received funding for medical training, giving presentations, and conducting basic research from the following pharmaceutical companies: Sanofi-Genzyme, Janssen, Gilead, Kern, Teva, and Boehringer Ingelheim., (Copyright © 2023 Sociedad Española de Farmacia Hospitalaria (S.E.F.H). Publicado por Elsevier España, S.L.U. All rights reserved.)

Objective: To develop a checklist to facilitate pharmaceutical care for patients with interstitial lung disease who require or are undergoing treatment with antifibrotic drugs., Method: Five hospital pharmacists developed an initial list of 37 items divided into 4 blocks: 1) First visit, which included general patient data and data from the first treatment; 2) Follow-up visits, assessing aspects of the follow-up of the treatment with nintedanib or pirfenidone; 3) Telepharmacy, consisting of the evaluation of the inclusion of patients in a program of this type, course of the disease, and identification of the contact with the pharmacy service; 4) Non-pharmacological treatment and patient information. To decide its potential inclusion in the checklist, two rounds of the Delphi were carried out in which the panelists had to assess the degree of agreement of each proposed item according to its "utility", which was the determining criterion for its inclusion, and its "applicability"., Results: 48 hospital pharmacists were contacted, 30 (63%) agreed in writing to participate, 28 (58%) completed the first round of the Delphi, and 27 (56%) completed the second round. After the first round of the Delphi the questionnaire was amended and comprised 40 items. Of the 40 items evaluated after the two rounds of the Delphi, there were two that, based on utility, the participants did not reach consensus for inclusion in the checklist: The one referring to "History of surgical intervention, specifically abdominal surgery in the last 4 weeks" (finally kept on the checklist due to its involvement in the indication of nintedanib) and to make recommendations on "Relaxation". No consensus was reached on their applicability for two of the items: "Patient stratification according to the Spanish Society of Hospital Pharmacy (SEFH) chronic patient model" and "Collection of Results Reported by the Patient"., Conclusions: The management of patients with ILD and/or pulmonary fibrosis is complex and requires a multidisciplinary approach where the hospital pharmacist plays a key role, especially, although not only, in monitoring drug treatment. We believe that this checklist can contribute from pharmaceutical care to improving the integrated care of patients with ILD who require or are undergoing treatment with antifibrotic drugs., (Copyright © 2023 Sociedad Española de Farmacia Hospitalaria (S.E.F.H). Publicado por Elsevier España, S.L.U. All rights reserved.)

Resumen INTRODUCCIÓN La proteinosis alveolar pulmonar es una enfermedad intersticial difusa poco frecuente, en la cual se produce obstrucción alveolar, debido al acúmulo de surfactante pulmonar. REPORTE DEL CASO Varón de 30 años de edad, presentó disnea progresiva y tos seca de un año de evolución. Antecedente personal: estibador de caña de azúcar. Presentó infecciones respiratorias recurrentes. Al examen físico se encontró cianosis, crepitantes difusos bilaterales y subcrepitantes en bases pulmonares. En la tomografía torácica con contraste se encontró un patrón de "empedrado loco". Se realizó videobroncoscopia con lavado broncoalveolar, aspirando material lechoso, espumoso y mucoso. Por citología se encontró linfocitos y material eosinofílico proteináceo acelular. El estudio anatomopatológico de la biopsia transbronquial reveló proteinosis alveolar pulmonar. El paciente reunió los criterios para tratamiento con lavado broncoalveolar total. Luego de este procedimiento, evolucionó favorablemente. CONCLUSIÓN La proteinosis alveolar pulmonar constituye una enfermedad importante a considerar, por el desafío diagnóstico y terapéutico que representa.

OBJETIVO: Devido à escassez de estudos populacionais, apresentamos um estudo epidemiológico em síndrome antissintetase anti-Jo-1 (SAS). PACIENTES E MÉTODOS: Estudo coorte retrospectivo realizado em um centro de 1980 a 2010. Dados clínico-laboratoriais e demográficos foram obtidos dos prontuários médicos. Todos os pacientes preenchiam critério de Bohan e Peter (1975) e apresentavam anti-Jo-1, além de envolvimento articular, muscular e pulmonar. Dezoito pacientes com SAS anti-Jo-1 foram analisados. RESULTADOS: A média de idade ao início da doença foi de 39,9 ± 15,7 anos, e a média da duração da doença, 9,7 ± 7,0 anos. Todos os pacientes eram brancos, e 94,4% eram mulheres. Sintomas constitucionais ocorreram em metade dos casos. Envolvimento cutâneo e do trato gastrointestinal ocorreram, respectivamente, em 66,6% e 55,6% dos casos. Não houve casos de envolvimento neurológico ou cardíaco. Metade dos pacientes apresentava pneumopatia incipiente, opacidade em vidro-fosco e fibrose pulmonar basal. Houve um caso de tuberculose, três de herpes zoster e um linfoma não Hodgkin. Um óbito ocorreu devido ao choque séptico (broncopneumonia hospitalar). Todos os pacientes receberam prednisona (1mg/kg/dia) e 12 (66,7%) receberam pulsoterapia com metil prednisolona (1 g/dia, 3 dias). Diferentes imunossupressores foram utilizados como poupadores de corticosteroide, dependendo da tolerância, efeitos colaterais e/ou refratariedade da doença. Embora a recidiva da doença (clínica e/ou laboratorial) tenha ocorrido em 87,5% dos casos, 12 dos 16 pacientes (75%) estavam com a remissão da doença no desfecho do presente estudo. CONCLUSÃO: A maioria dos pacientes eram mulheres brancas e com alta taxa de recidiva da doença.OBJECTIVE: Given a lack of population-based studies, we report an epidemiological-clinic study of anti-Jo-1 antisynthetase syndrome (ASS). PATIENTS AND METHODS: To study a retrospective cohort of a single-center from 1980 to 2010. Clinical-laboratory and demographic data were obtained from medical files. All patients fulfilled the Bohan and Peter criteria (1975) and presented anti-Jo-1, articular, muscle and lung involvement. Eighteen patients with anti-Jo-1 ASS were analyzed. RESULTS: The mean age at disease onset was 39.9 ± 15.7 years and average disease duration was 9.7 ± 7.0 years. All subjects were white, and 94.4% were female. Constitutional symptoms occurred in 50 % of cases. There was cutaneous and gastrointestinal tract involvement in 66.6% and 55.6% of cases, respectively. No cases manifested neurologic or cardiac involvement. Half of the patients showed incipient pneumopathy, ground-glass opacities and basal pulmonary fibrosis. There was one case of tuberculosis, three of herpes zoster and one of non-Hodgkin lymphoma. One death occurred due to sepsis shock (hospital bronchopneumonia). All patients received prednisone (1mg/kg/day) and 12 (66.7%) participants received methyl prednisolone pulse therapy (1g/day, 3 days). Various immunosuppressants were used as corticosteroid tapers, depending on tolerance, side effects and/or refractoriness. Although disease relapse (clinical and/or laboratory) occurred in 87.5% of cases, 12 out of 16 patients (75%) were in disease remission at study endpoint. CONCLUSION: In the present study, almost all patients were white females and the disease relapse rate was high.

Diffuse interstitial lung diseases (ILD) are a heterogeneous group of respiratory disorders that are usually classified together because they have similar clinical, radiological, physiological or pathological manifestations. In the last decade there have been important scientific advances in the study of these entities, which has led to a better understanding of their pathophysiology and to the appearance of treatments that have brought about a paradigm shift in the disease. This document presents a series of questions and answers on ILD, with special emphasis on the most relevant changes in terms of pathophysiology, diagnosis, and treatment., (© 2023 Sociedad Española de Neumología y Cirugía Torácica (SEPAR). Published by Elsevier España, S.L.U.)

La decisión de realizar una biopsia por toracoscopia videoasistida para evaluar un paciente con enfermedad pulmonar intersticial, se basa en la posibilidad de obtener un diagnóstico específico con este método y de esta manera lograr un cambio en el tratamiento de estos pacientes. En el período comprendido entre enero de 1995 y octubre de 2003, se realizaron biopsias a un total de 128 pacientes que presentaban manifestaciones de enfermedad pulmonar intersticial. De ellos, 95 eran hombres y 33, mujeres. La edad media fue de 48,2 años. Obtuvimos el diagnóstico específico en 183 pacientes (66 %), mientras que en el 34 % de los casos, éste resultó ser inespecífico. Los diagnósticos específicos más frecuentes fueron el carcinoma pulmonar y la sarcoidosis, condiciones inespecíficas como: la neumonitis intersticial, la fibrosis pulmonar intersticial, etcétera. En los casos sintomáticos se indicó tratamiento médico con esteroides y se observó una evolución favorable. La biopsia pulmonar por toracoscopia videoasistida resultó ser un proceder muy exacto y con alta especificidad para el diagnóstico de las enfermedades pulmonares intersticiales. El número de complicaciones fue ínfimo y la recuperación de los pacientes, muy rápida. La estadía hospitalaria menor de 4 días demostró que este proceder resulta más ventajoso que la biopsia pulmonar abierta para el diagnóstico de enfermedades de este tipoThe decision to perform biopsy by video-assisted thoracoscopy to evaluate a patient with interstitial lung disease is based on the possibility to have a specific diagnosis with this method and, this way, to make a change in the treatment of these patients. From January 1995 to October 2003, biopsies were performed among 128 patients presenting manifestations of interstitial lung disease.Of them, 95 were males and 33 females. Mean age was 48.2 years old. A specific diagnosis was attained in 183 patients (66 %), whereas it was unspecific in 34 % of the cases. The commonest specific diagnoses were lung carcinoma and sarcoidosis, unparticular conditions such as interstitial pneumonitis, interstitial lung fibrosis, etc. In the symptomatic cases, medical treatment with steroids was indicated and a favorable evolution was observed. . Lung biopsy by video-assisted thoracoscopy proved to be a very accurate method with a high specificity for the diagnosis of interstitial lung diseases. The hospital stay of less than 4 days showed that this procedure is more advantageous than open lung biopsy for diagnosing this type of diseases

Background: Platelets have an effect on the hemostatic defense of the lung. Immature platelet fractions (iPF) reflects the number of young platelets containing ribonucleic acid in the circulation and real-time production. Information about their roles in rheumatic diseases is limited and there are no studies on iPF in RA with interstitial lung disease (ILD). Our aim is to investigate the association between the iPF level and occurrence of ILD in RA and the correlation of iPF with disease activity in general or only in RA with ILD., Methods: The study included 50 RA patients without ILD, 33 RA patients with ILD, and 30 healthy controls. Demographic data, Disease Activity Score 28 (DAS28), autoantibodies, and iPF were evaluated. ILD was diagnosed by using high-resolution computed tomography with clinical findings and chest X-ray. The samples were analyzed for complete blood count with platelet indices included, on Mindray BC-6800 hematology analyzer, Hamburg, Germany., Results: iPF levels were higher in RA patients with ILD compared to healthy controls and RA patients without ILD. A weakly positive correlation between DAS28 with iPF was found in all RA patients. iPF levels were found as 2.85 to detect ILD with 66.7% sensitivity and 65% specificity., Conclusions: Our results showed that the iPF was detected higher in RA with ILD compared to RA without ILD. iPF, a routine cheap and easy test during hemogram, can provide important information in terms of disease activity and lung involvement in RA., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Background: Clinically evident interstitial lung disease (ILD) affects 10%-42% of RA patients with prognostic implications. The aim of this study was to discern which factors are associated with the presence of ILD in RA patients and to develop a score that could help to stratify the risk of having ILD in RA patients., Methods: Case-control study. We included RA patients recruited from ILD and rheumatology clinics. We retrieved the following data: gender, age, presence of extra articular manifestations, disease activity scores, antibodies status, ESR, and medication use. Multivariate logistic regression was performed. A risk indicator score was developed., Results: Of 118 patients included in this study, 52 (44%) had RA-ILD (cases) and 66 (56%) had RA without ILD (controls). Twenty-six patients were male (22%), the mean age was 56.6±15.6 years. Five variables were significantly associated with the presence of ILD: male gender, smoking, extraarticular manifestations, a CDAI score>28, and ESR>80mm/h. The AUC of the final model curve was 0.86 (95%CI 0.79-0.92). Two potential cut-off points of the risk indicator score were chosen: a value of 2 points showed a sensitivity of 90.38% and a specificity of 63.64%, while a value of 4 points showed a sensitivity of 51.9% and a specificity of 90.9%., Conclusion: This study identified risk factors that could help identify which RA patients are at risk of having ILD through the development of a risk indicator score. This score needs to be validated in an independent cohort., (Copyright © 2019 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Objectives: To study the differences between rheumatoid arthritis (RA)-interstitial lung disease (ILD) patients and RA patients without ILD in severity markers and disease activity and to identify factors associated with the presence of ILD in RA patients., Patients and Methods: Patients: RA-ILD patients selected from a multicentre cohort in Andalusia, Spain., Controls: RA-patients without ILD paired by sex, age and disease duration., Protocol: RA patients are reviewed every 3-6months in rheumatology consultation. All patients are reviewed according to a predetermined protocol with systematic data collection., Outcomes: description of ILD type, differences in severity markers and disease activity in both groups. Other variables: ILD type by imaging technique (HRCT): nonspecific interstitial pneumonia (NSIP)/usual interstitial pneumonia (UIP). Lung function by PTF. Activity and severity markers of arthritis by DAS28-ESR, HAQ, RF, ACPA and erosions. Treatment with DMARD., Statistical Analysis: descriptive and paired T-test or Chi-square test followed by binary logistic regression (DV: ILD in patients with RA)., Results: Eighty-two patients were included, 41 RA-ILD and 41 RA controls. RF and ACPA positivity, serositis and osteoporosis were more frequent in RA-ILD patients. No significant differences in DAS28 were observed (P=.145) between RA-ILD and RA control patients. RA-ILD patients presented worse HAQ scores (P=.006). All patients were treated with disease modifying antirheumatic drugs (DMARDs). The risk of developing ILD in RA patients is tripled by a history of smoking or the presence of erosive arthritis (R 2 =.36)., Conclusions: The results of our study support the higher frequency of UIP and NSIP in RA patients. DLCO is the most sensitive parameter to detect ILD in RA patients. Our study showed that ILD in RA patients was associated with RA severity (presence of erosions and ACPA) and with a history of smoking., (Copyright © 2019 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Ultrasound is a non-invasive, innocuous, reproducible, cost-efficient imaging technique that provides immediate information, as it can be performed in our consultations. The good profile of ultrasound and the technological advances that have taken place in recent years, which have allowed a substantial improvement in the resolution of the image to make it almost anatomical, have promoted research on its application in the comprehensive study of systemic inflammatory diseases. At present, the threshold of using ultrasound to exclusively investigate musculoskeletal manifestations has been crossed, to also apply it to the study of extra-articular manifestations and comorbidities associated with rheumatic diseases. In this review we will revise its usefulness for the diagnosis of glandular involvement in Sjögren's syndrome, interstitial lung disease or giant cell arteritis and for stratification of cardiovascular risk in patients with chronic inflammatory rheumatic diseases., (Copyright © 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Introduction: Interstitial lung disease (ILD) is a common comorbidity present in patients with systemic sclerosis (SSc). Employment of high-resolution computed tomography (HRCT) is very limited and lung ultrasound (LUS) can be an alternative tool for the early evaluation of ILD., Objective: To determine the validity of LUS in the early detection of ILD in patients with SSc., Methods: Sixty-eight patients with SSc ≥18 years without respiratory symptoms were included. A rheumatologist rated the subclinical respiratory condition, another rheumatologist blinded to the clinical assessment performed the LUS. To determine validity HRCT was performed as well., Results: Prevalence of ILD in SSc patients was 41.2% in contrast to the 4.8% healthy controls (P=.0001). Variables associated with LUS and HRCT findings were anti-centromere antibodies (P=.005) and the Rodnan skin score (P=.004). A positive correlation was present between the findings of HRCT and LUS (P=.001). Sensitivity and specificity were 91.2% and 88.6% respectively. Good reliability in the LUS findings was found between observers (k=.72)., Conclusions: By proving to be a valid, trustworthy and feasible alternative tool, we consider that LUS can be implemented for the early detection of ILD in SSc., (Copyright © 2019 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Background and Objectives: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up., Methods: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model., Results: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI., Conclusions: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

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., (Copyright © 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Cryobiopsy in interstitial lung disease: a real-life experience from a single center in Argentina. Transbronchial lung cryobiopsy (TBLC) has been recently introduced for the histological assessment of interstitial lung diseases. The objectives were to assess the diagnostic yield and safety of TBLC for interstitial lung diseases in our setting. A retrospective review of subjects who underwent TBLC in a tertiary care center from September 2016 to November 2019 was performed. Data regarding subject and procedure characteristics, complications, histopathological and multidisciplinary diagnosis was recorded. Logistic regression was used to analyze potential factors associated to diagnosis and complications. A total of 52 subjects were included. Mean number of samples was 5.7 (SD 1.7) with a median sample diameter of 6 mm (interquartile range 6-8). Pneumothorax developed in 13 (25%) participants, and no cases of severe bleeding or death were registered. A 73.1% and 84.5% diagnostic yield was recorded through histology and added multidisciplinary evaluation respectively. The number of samples was associated to the diagnostic yield in a multivariate analysis (OR 2.15 - CI95% 1.16- 3.99). TBLC appears to be safe and effective for the assessment of interstitial lung diseases in a real life setting when applied in a center with access to multidisciplinary evaluation.

Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder characterized by a proliferation of neuroendocrine cells within the lung. It is classically described as a disease with persistent cough, dyspnea and wheezing in non-smoker middle aged females. CT of the chest reveals diffuse air trapping with mosaic pattern., Patients and Methods: We present two cases of DIPNECH that were sent to our department to perform a lung biopsy with the diagnostic suspicion of diffuse interstitial disease. Both cases were women with a history of chronic cough and moderate effort dyspnea., Results and Discussion: The aim of this paper is that physicians take into account this diagnostic entity before treating as an asthmatic a patient with these characteristics, not forgetting that they are prenoplastic lesions., (Copyright © 2018 Elsevier España, S.L.U. All rights reserved.)

ABSTRACT Objective: To assess clinical utility of serum Clara cell 16-kDa protein measurements in relation with staging system for systemic sclerosis associated interstitial lung disease. Materials and methods: Serum levels of Clara cell 16-kDa protein were determined by ELISA in 28 systemic sclerosis patients and 30 healthy controls, and correlated with staging system for systemic sclerosis associated interstitial lung disease in systemic sclerosis patients. Lung involvement was assessed functionally (body plethysmography, diffusing capacity of the lung for carbon monoxide) and radiologically (an average disease extent on high resolution computed tomography of the lungs) in SSc patients. Results: We observed statistically significant differences in serum Clara cell 16-kDa protein levels between systemic sclerosis patients and healthy controls only in non-smokers. However, serum Clara cell 16-kDa protein concentrations were significantly elevated in patients with high resolution computed tomography extent >20% in comparison to patients with high resolution computed tomography extent

Microscopic polyangiitis (MPA) is a systemic disease included in the Chapel Hill 2012 Classification as necrotizing vasculitis affecting capillaries, venules and arterioles. It usually expresses antineutrophil cytoplasmic antibodies (ANCA) and has a perinuclear immunofluorescence pattern and correlation with anti-myeloperoxidase (MPO) antibodies. Capillaritis with alveolar hemorrhage is the most common manifestation of lung disease. Interstitial lung disease (ILD) is uncommon, with usual interstitial pneumonia being the predominant pattern. However, other patterns such as organizing pneumonia have been described. No guidelines exist for treating patients with ILD and, currently, ANCA-associated vasculitis (AAV) is managed along the lines of small vessel vasculitis. The prognosis with this association is uncertain, with possibilities of relapse and a fatal outcome. We present a case in which ILD was the first manifestation of MPA, without alveolar hemorrhage, with subsequent renal involvement and, in which, the established treatment produced a significant clinical improvement., (Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

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., (Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Interstitial lung disease (ILD) is one of the major causes of death in systemic sclerosis (SSc). This study investigated exhaled breath (EB) and exhaled breath condensate (EBC) biomarkers in patients with SSc and analyzed their role as a prognostic tool in SSc-related ILD., Methods: Fraction exhaled nitric oxide (FeNO) and exhaled carbon monoxide (eCO) measured in EB, together with pH, nitrite, nitrate and interleukin-6 levels measured in EBC were prospectively analyzed in 35 patients with SSc. Twelve patients had established ILD by chest high-resolution computed tomography (HRCT), and 23 patients showed no evidence of ILD. EB and EBC biomarkers were determined at inclusion, and pulmonary function tests were annually performed during 4 years of follow-up., Results: No differences at baseline biomarkers levels were found between groups. In all patients studied, low EBC pH levels were associated with a decreased diffusing capacity for carbon monoxide (DLCO) during follow-up. Low FeNO levels were correlated with lower forced vital capacity (FVC) at baseline, 4years of follow-up and with a decrease in FVC and DLCO during monitoring. Among ILD patients, high eCO levels were correlated with lower baseline FVC. In the global cohort, a worse progression-free survival was identified in patients with EBC pH values lower than 7.88 and FeNO levels lower than 10.75ppb (Log Rank P=.03 and P<.01, respectively)., Conclusions: EB and EBC could help to detect patients likely to present a deterioration on lung function during follow up., (Copyright © 2016 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Dyspnoea is a complex, highly personalized and multidimensional sensory experience, and its underlying cause and mechanisms are still being investigated. Exertional dyspnoea is one of the most frequently encountered symptoms of patients with cardiopulmonary diseases, and is a common reason for seeking medical help. As the symptom usually progresses with the underlying disease, it can lead to an avoidance of physical activity, peripheral muscle deconditioning and decreased quality of life. Dyspnoea is closely associated with quality of life, exercise (in)tolerance and prognosis in various conditions, including chronic obstructive pulmonary disease, heart failure, interstitial lung disease and pulmonary hypertension, and is therefore an important therapeutic target. Effective management and treatment of dyspnoea is an important challenge for caregivers, and therapeutic options that attempt to reverse its underlying cause have been only partially successful This "review" will attempt to shed light on the physiological mechanisms underlying dyspnoea during exercise and to translate/apply them to a broad clinical spectrum of cardio-respiratory disorders., (Copyright © 2016 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)