Lista de comprobación para la atención farmacéutica del paciente con enfermedad pulmonar intersticial (CheckEPID): un consenso basado en el método Delphi / Checklist for Pharmaceutical Care of the Patient with interstitial lung disease (CheckEPID): A Delphi-based consensus

Objetivo desarrollar una lista de comprobación para facilitar la atención farmacéutica al paciente con enfermedad pulmonar intersticial que requieren o están en tratamiento con antifibróticos. Método 5 especialistas en farmacia hospitalaria desarrollaron un listado inicial de 37 ítems divididos en 4 bloques: 1) primera visita del paciente, que incluía datos generales del paciente y datos del primer tratamiento; 2) visitas de seguimiento, valorando aspectos del seguimiento del tratamiento con nintedanib o pirfenidona; 3) telefarmacia, consistente en la evaluación de la inclusión de pacientes en un programa de este tipo, evolución de la enfermedad e identificación del contacto con el servicio de farmacia y 4) tratamiento no farmacológico e información al paciente. Para decidir su potencial inclusión en el listado de comprobación se realizaron 2 rondas del Delphi en las que los panelistas tenían que valorar de cada ítem propuesto su grado de acuerdo con su «utilidad», que fue el criterio determinante para su inclusión y su «aplicabilidad». Resultados se contactó con 48 farmacéuticos hospitalarios, 30 (63%) aceptaron por escrito participar, 28 (58%) completaron la primera ronda del Delphi y 27 (56%) completaron la segunda ronda. Después de la primera ronda el cuestionario se modificó y quedó constituido por 40 ítems. De los 40 ítems evaluados tras las 2 rondas del Delphi, hubo 2 que, basados en la utilidad, los participantes del Delphi no alcanzaron el consenso para su inclusión en el listado: el referido a «Antecedentes de intervención quirúrgica, específicamente cirugía abdominal en las últimas 4 semanas» (finalmente mantenido en el listado por su implicación en la indicación de nintedanib) y el de realizar recomendaciones sobre «Relajación». En 2 de los ítems no se alcanzó consenso sobre su aplicabilidad: «Estratificación del paciente según el modelo del paciente crónico de la SEFH» y «Recogida de resultados comunicados por el paciente». Conclusiones... (AU)

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 ... (AU)

[Translated article] Checklist for the pharmaceutical care of patients with interstitial lung disease (CheckEPID): A Delphi-based consensus.

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.

Eficacia y tolerabilidad Resultados del tratamiento para la fibrosis pulmonar idiopática. Experiencia de la vida real / Efficacy and Tolerability Outcomes of Treatment for Idiopathic Pulmonary Fibrosis. Real-life Experience

Introducción: la fibrosis pulmonar idiopática (FPI) es una enfermedad progresiva y cró-nica con muy mal pronóstico. Actualmente, existen dos fármacos para esta patología. El propósito de nuestro estudio es evaluar los efectos del tratamiento en los pacientes de una consulta en vida real.

Introduction: idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease with a very poor prognosis. Two drugs are currently available for this disease. The purpo-se of our study is to evaluate the effects of treatment in patients in a real-life practice.

Checklist for Pharmaceutical Care of the Patient with interstitial lung disease (CheckEPID): A Delphi-based consensus. / Lista de comprobación para la atención farmacéutica del paciente con enfermedad pulmonar intersticial (CheckEPID): un consenso basado en el método Delphi.

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.

Caracterización clínica de pacientes con fibrosis pulmonar idiopática en un hospital de tercer nivel en la ciudad de Quito-Ecuador / Clinical Characterization of patients with Idiopathic Pulmonary Fibrosis in a Tertiary Hospital of the City of Quito-Ecuador

RESUMEN Introducción: La fibrosis pulmonar idiopática es una enfermedad progresiva y fa tal caracterizada por el hallazgo de neumonía intersticial usual en tomografía de alta resolución o biopsia pulmonar, o en discusión multidisciplinar y el descarte de otras etiologías como enfermedades del tejido conectivo o exposicionales. En cuanto a los objetivos de este trabajo, consisten en conocer las características clí nicas, la función pulmonar y la supervivencia del grupo de pacientes con diagnóstico de fibrosis pulmonar idiopática evaluados en la clínica de intersticiales del Hospital Carlos Andrade Marín. Material y métodos: Se trata de un estudio transversal, retrospectivo, observacional. La población de estudio la constituyeron los pacientes con diagnóstico de fibrosis pul monar idiopática atendidos en la clínica de intersticiales del Hospital Carlos Andrade Marín entre enero del 2018 y febrero del 2020. Resultados: De 35 pacientes con fibrosis pulmonar idiopática incluidos para el análi sis, el 85,7% fueron del sexo masculino. Al momento del diagnóstico, la edad pro medio fue de 69,7 años (DE: 9,26, Rango: 38-87 años). El 20% y 37,1% presentaron disnea de grado 3 y grado 4, respectivamente. El 60% presentaron antecedentes de tabaquismo. El 45,7% de los diagnósticos se hicieron tanto con evaluación clínica multidisciplinaria y tomografía axial computarizada de alta resolución. Conclusiones: Hemos informado la mayor cohorte de fibrosis pulmonar idiopática en el Ecuador, nuestros resultados han identificado poblaciones similares con otros gru pos de estudio en los que la tomografía computarizada de alta resolución y el análisis multidisciplinar son los métodos más utilizados en el diagnóstico.

ABSTRACT Background: idiopathic pulmonary fibrosis is a progressive, fatal disease character ized by the findings of usual interstitial pneumonia in a high resolution tomography or lung biopsy, or in a multidisciplinary discussion, also discarding other etiologies such as connective tissue diseases or diseases associated with toxic exposure. The objective of this work was to know the clinical characteristics, lung function and survival of the group of patients diagnosed with idiopathic pulmonary fibrosis who were evaluated at the Interstitial Lung Disease Clinic of the Hospital Carlos Andrade Marín. Methods: retrospective, cross-sectional, observational study. The study population consisted of patients diagnosed with idiopathic pulmonary fibrosis who had been treat ed at the Interstitial Lung Disease Clinic of the Hospital Carlos Andrade Marín between January, 2018 and February, 2020. Results: 85.7% of the 35 patients with idiopathic pulmonary fibrosis included in the analysis were male. At the time of the diagnosis, the mean age was 69.7 years (SD [standard deviation]: 9.26, range: 38-87 years). 20% and 37.1% of patients showed dyspnea grade 3 and 4, respectively. 60% had smoking history. 45.7% of the diagnoses were made with a multidisciplinary clinical evaluation and high resolution computed axial tomography. Conclusions: we have reported the largest cohort of patients with idiopathic pulmo nary fibrosis in Ecuador; our results identified similar populations with other study groups where the high resolution computed tomography and multidisciplinary analysis are the most used methods for the diagnosis.

Tratamiento de fibrosis pulmonar idiopática con células madre adultas autólogas de médula ósea / Treatment of idiopathic pulmonary fibrosis with autologous adult bone marrow stem cells

Introducción: La fibrosis pulmonar idiopática representa una enfermedad degenerativa con pronóstico nefasto, cuyas opciones terapéuticas son muy limitadas, por lo que es necesario evaluar alternativas asequibles a la población. Objetivo: Evaluar el tratamiento con células madre adultas autólogas de médula ósea de paciente de 68 años con diagnóstico radiológico de fibrosis pulmonar y disnea en reposo. Presentación del caso: La paciente acudió a consulta con disnea en reposo y saturación digital de 74 por ciento, la cual después de su primer tratamiento endovenoso con células madre adultas de médula ósea, logró una mejoría significativa con saturaciones que oscilaron entre 85 y 90 por ciento, a los 4 meses de la primera terapia. A los 8 meses de la primera sesión recibió una segunda dosis, con la cual subió la saturación a valores entre 94 y 97 por ciento, a las tres semanas de ésta última. Conclusión: El tratamiento con células madre adultas autólogas de médula ósea podría mejorar los signos y síntomas de los pacientes con fibrosis pulmonar idiopática(AU)

Introduction: Idiopathic pulmonary fibrosis represents a degenerative disease with a dire prognosis, whose therapeutic options are very limited, so it is necessary to evaluate alternatives available to the population. Objective: To evaluate treatment with autologous adult bone marrow (BM) stem cells in a 68-year-old patient with a radiological diagnosis of pulmonary fibrosis and dyspnea at rest. Case presentation: The patient attended the consultation with dyspnea at rest, and digital saturation of 74 percent, which after her first intravenous treatment with adult bone marrow stem cells, achieved significant improvement, with saturations ranging between 85 and 90 percent at 4 months after the first therapy. Eight months after the first session, she received a second dose, with which she increased the saturation to values between 94 and 97 percent, three weeks after the latter. Conclusion: Conclusion: Treatment with autologous adult bone marrow stem cells could improve the signs and symptoms of patients with idiopathic pulmonary fibrosis(AU)

Seguimiento de 20 pacientes portadores de fibrosis pulmonar idiopática tratados con nintedanib / Follow-up of 20 patients with idiopathic pulmonary fibrosis treated with nintedanib

Se presenta un estudio observacional compasivo de seguimiento de 20 pacientes portadores de Fibrosis Pulmonar Idiopática tratados con Nintedanib, que muestra que Nintedanib es un medicamento en general bien tolerado, sin efectos adversos serios, que otorga una sobrevida más prolongada que la que cabría esperar en pacientes con esta enfermedad.

A compassionate observational follow-up study of 20 patients with Idiopathic Pulmonary Fibrosis treated with Nintedanib is presented, showing that Nintedanib is a generally well-tolerated drug, with no serious adverse effects, that grants a longer survival in real-life patients.

Fibrosis pulmonar idiopática / Idiopathic pulmonary fibrosis

La fibrosis pulmonar idiopática se define como una neumonía intersticial fibrosante crónica progresiva de etiología desconocida. Existen factores de riesgo, tanto intrínsecos como extrínsecos, que pueden fomentar el desarrollo de la enfermedad en individuos con predisposición genética. El diagnóstico se realiza mediante hallazgos radiológicos y/o histológicos característicos en la tomografía axial computarizada de alta resolución y la biopsia pulmonar, respectivamente, en ausencia de causa específica identificable. La supervivencia media de la enfermedad sin tratamiento es de 3-5años desde el inicio de los síntomas, aunque su historia natural es variable e impredecible. Actualmente existen dos fármacos antifibróticos que enlentecen la progresión de la enfermedad. El abordaje multidisciplinar considerará el estado nutricional, el estado emocional, el acondicionamiento físico y el tratamiento de comorbilidades, así como el trasplante pulmonar y los cuidados paliativos en fases avanzadas. En este artículo se revisan los aspectos fundamentales para el diagnóstico y el tratamiento de la fibrosis pulmonar idiopática. (AU)

Idiopathic pulmonary fibrosis is defined as a chronic progressive fibrosing interstitial pneumonia of unknown etiology. There are intrinsic and extrinsic risk factors that could favor the development of the disease in individuals with a genetic predisposition. The diagnosis is made by characteristic radiological and/or histological findings on high-resolution computed tomography and lung biopsy, respectively, in the absence of a specific identifiable cause. The median survival of the disease for patients without treatment is 3-5years from the onset of symptoms, although its natural history is variable and unpredictable. Currently, there are two antifibrotic drugs that reduce disease progression. The multidisciplinary approach will consider the nutritional and emotional status, physical conditioning, and treatment of comorbidities, as well as lung transplantation and palliative care in advanced stages. The following article reviews the fundamental aspects for the diagnosis and treatment of idiopathic pulmonary fibrosis. (AU)

Idiopathic pulmonary fibrosis. / Fibrosis pulmonar idiopática.

Idiopathic pulmonary fibrosis is defined as a chronic progressive fibrosing interstitial pneumonia of unknown etiology. There are intrinsic and extrinsic risk factors that could favor the development of the disease in individuals with a genetic predisposition. The diagnosis is made by characteristic radiological and/or histological findings on high-resolution computed tomography and lung biopsy, respectively, in the absence of a specific identifiable cause. The median survival of the disease for patients without treatment is 3-5years from the onset of symptoms, although its natural history is variable and unpredictable. Currently, there are two antifibrotic drugs that reduce disease progression. The multidisciplinary approach will consider the nutritional and emotional status, physical conditioning, and treatment of comorbidities, as well as lung transplantation and palliative care in advanced stages. The following article reviews the fundamental aspects for the diagnosis and treatment of idiopathic pulmonary fibrosis.

Biomarcadores biológicos en las enfermedades respiratorias / Biological Biomarkers in Respiratory Diseases

En los últimos años la llamada «medicina personalizada o de precisión» ha irrumpido con fuerza en el manejo de las enfermedades, entre ellas las respiratorias. La posibilidad de implantar esta forma de trabajar pasa indefectiblemente por el hallazgo y validación de biomarcadores biológicos que se relacionen bien con el diagnóstico, tratamiento o pronóstico de los pacientes respiratorios. En este sentido, la mayoría de enfermedades respiratorias o grupo de las mismas ya cuentan con biomarcadores biológicos de mayor o menor fiabilidad, y se están realizando un gran número de estudios en busca de nuevos de estos indicadores. El objetivo de la presente revisión es poner al día al lector y analizar la literatura científica existente sobre la existencia y validez diagnóstica, terapéutica o pronóstica de los biomarcadores biológicos más importantes en la actualidad en las principales enfermedades respiratorias, así como sobre los retos futuros en este sentido. (AU)

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area. (AU)

[Translated article] Biological Biomarkers in Respiratory Diseases / Biomarcadores biológicos en las enfermedades respiratorias

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area. (AU)

En los últimos años la llamada «medicina personalizada o de precisión» ha irrumpido con fuerza en el manejo de las enfermedades, entre ellas las respiratorias. La posibilidad de implantar esta forma de trabajar pasa indefectiblemente por el hallazgo y validación de biomarcadores biológicos que se relacionen bien con el diagnóstico, tratamiento o pronóstico de los pacientes respiratorios. En este sentido, la mayoría de enfermedades respiratorias o grupo de las mismas ya cuentan con biomarcadores biológicos de mayor o menor fiabilidad, y se están realizando un gran número de estudios en busca de nuevos de estos indicadores. El objetivo de la presente revisión es poner al día al lector y analizar la literatura científica existente sobre la existencia y validez diagnóstica, terapéutica o pronóstica de los biomarcadores biológicos más importantes en la actualidad en las principales enfermedades respiratorias, así como sobre los retos futuros en este sentido. (AU)

Biological Biomarkers in Respiratory Diseases. / Biomarcadores biológicos en las enfermedades respiratorias.

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area.

Impacto clínico de un grupo de discusión multidisciplinaria en el diagnóstico de fibrosis pulmonar idiopática en Colombia / Clinical impact of a multidisciplinary discussion group on the diagnosis of idiopathic pulmonary fibrosis in Colombia

Resumen Introducción: la fibrosis pulmonar idiopática (FPI) es una enfermedad pulmonar intersticial (EPID) de mal pronóstico, considerada huérfana en Colombia. Un diagnóstico correcto tiene implicaciones para el paciente y los costos de atención. Los grupos de discusión multidisciplinaria (GDM) se consideran el estándar de oro en el diagnóstico. No hay estudios previos en Colombia de la experiencia de un GDM. Objetivos: evaluar el impacto de un GDM en una institución de cuarto nivel en Bogotá en cambio de diagnóstico de pacientes con EPID y la concordancia entre el diagnóstico inicial y final de FPI. Material y métodos: pacientes con EPID evaluados entre 2015-2018 por el GDM conformado por neumólogos, radiólogo, patólogo y reumatólogos. Criterios ATS/ERS/JRS/ALAT de diagnóstico de FPI. Descripción del cambio en el diagnóstico y concordancia entre el diagnóstico inicial y del GDM en FPI. Resultados: de 165 pacientes con EPID se cambió el diagnóstico en 35.2%. En 77.3% pacientes con diagnóstico inicial de FPI y en 6.7% con diagnóstico inicial diferente a FPI el GDM confirmó FPI. Al descartar FPI, los principales diagnósticos fueron neumonitis de hipersensibilidad en fase crónica (29.4%) y neumonía intersticial no específica (23.5%). El índice kappa entre el diagnóstico inicial y final de FPI fue 0.71 (0.60-0.82). Conclusiones: el GDM en EPID tuvo un importante impacto clínico demostrado por un alto porcentaje de cambió del diagnóstico de remisión. Se descartó el diagnóstico inicial de FPI en un porcentaje significativo de pacientes y se ratificó en un grupo menor sin esta sospecha clínica inicial. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2017).

Abstract Introduction: idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease (ILD) with a poor prognosis, considered an orphan disease in Colombia. An accurate diagnosis has implications for the patient and healthcare costs. Multidisciplinary discussion groups (MDGs) are considered the gold standard for diagnosis. There are no prior studies in Colombia on the experience of an MDG. Objectives: to evaluate the impact of an MDG in a quaternary care institution in Bogotá on the change in the diagnosis of patients with ILD and the concordance between the initial and final diagnosis of IPF. Materials and methods: patents with ILD evaluated from 2015-2018 by the MDG made up of pulmonologists, a radiologist, a pathologist and rheumatologists. The ATS/ERS/JRS/ALAT diagnostic criteria for IPF. A description of changes in the diagnosis and the agreement between the initial diagnosis and the MDG diagnosis of IPF. Results: out of 165 patients with ILD, the diagnosis was changed in 32.5%. The MDG confirmed IPF in 77.3% of patients with an initial diagnosis of ILD and 6.7% of those with a different initial diagnosis. When IPF was ruled out, the main diagnoses were chronic hypersensitivity pneumonitis (24.8%) and nonspecific interstitial pneumonia (23.5%). The Kappa index between the initial and final IPF diagnoses was 0.71 (0.60-0.82). Conclusions: the MDG on ILD had a significant clinical impact evidenced by a high percentage of change in the referral diagnosis. The initial diagnosis of IPF was ruled out in a significant percentage of patients and confirmed in a smaller group which did not have this initial clinical suspicion. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2017).

Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity / Diferentes caras de la fibrosis pulmonar idiopática con capacidad vital forzada preservada

Introduction Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials. Methods Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed. Results 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern. Conclusions Patients with preserved FVC but presenting UIP radiological pattern and moderate–severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

Introducción La fibrosis pulmonar idiopática (FPI) es progresiva e irreversible. Existen algunas discrepancias sobre la estadificación de la FPI, especialmente en las fases leves. La capacidad vital forzada (FVC) superior al 80% se ha considerado una FPI temprana o leve incluso para el diseño de ensayos clínicos. Métodos Estudio español multicéntrico, observacional, retrospectivo de pacientes con FPI diagnosticados entre 2012-2016, en función de los criterios ATS/ERS, que presentaban FVC mayor o igual al 80% al diagnóstico. Se analizaron características clínicas y demográficas, función pulmonar, patrón radiológico, tratamiento y seguimiento. Resultados Se incluyeron 225 pacientes con FPI, el 72,9% eran varones. La edad media fue de 69,5 años. El patrón predominante en la tomografía computarizada de alta resolución (TCAR) fue compatible con neumonía intersticial usual (NIU) (51,6%). El 84,7% de los pacientes presentó síntomas respiratorios (disnea de esfuerzo o tos) y el 33,33% mostró desaturación con una saturación de oxígeno inferior al 90% en la prueba de la marcha de los 6min (PM6M). El tratamiento antifibrótico se inició en el momento del diagnóstico en el 55,11% de los pacientes. La mediana de la CVF fue del 89,6% (RIC 17) y el 58,7% de los pacientes presentó una disminución de la capacidad pulmonar de difusión del monóxido de carbono (DLCO) por debajo del 60% del valor teórico; la mayoría presentó progresión funcional (61,4%) y una mayor mortalidad a los 3 años (20,45%). Se observó una correlación estadísticamente significativa de la mortalidad a los 3 años entre una DLCO<60% y el patrón radiológico compatible con UIP. Conclusiones Los pacientes con FVC conservada pero que presentan patrón radiológico de NIU y disminución moderada-grave de la de DLCO en el momento del diagnóstico se asociaron a un mayor riesgo de progresión, fallecimiento o tener que recibir un trasplante de pulmón. Por lo tanto, en estos casos, una FVC preservada no sería representativa de una FPI temprana o leve

Efectividad y seguridad de pirfenidona en la fibrosis pulmonar idiopática / Effectiveness and safety of pirfenidone in idiopathic pulmonary fibrosis

Objetivo: La fibrosis pulmonar idiopática (FPI) es una enfermedad caracterizada por una cicatrización progresiva de los pulmones provocando su deterioro a largo plazo. Su diagnóstico requiere la existencia del patrón radiológico denominado neumonía intersticial usual (NIU). Su prevalencia es mayor en hombres. Entre los síntomas principales encontramos la disnea y la tos. En la actualidad, no existe una cura conocida, sin embargo, existen diversos tratamientos antifibróticos enfocados a disminuir la progresión de esta patología y prolongar la supervivencia. El objetivo es evaluar la efectividad y seguridad de la pirfenidona en esta enfermedad.Métodos: Estudio descriptivo retrospectivo entre enero de 2016 y diciembre de 2019. Se incluyeron 40 pacientes diagnosticados de fibrosis pulmonar idiopática que iniciaron tratamiento con pirfenidona.Resultados: Los parámetros de función pulmonar mostraron valores mantenidos durante el periodo de estudio. Hubo mejoría de la tos. Las reacciones adversas más comunes fueron alteraciones gastrointestinales y cutáneas.Conclusión: El tratamiento con pirfenidona requiere de un riguroso seguimiento por el porcentaje de pacientes que abandonó esta terapia. (AU)

Objective: Idiopathic pulmonary fibrosis (IPF) is a disease characterized by progressive scarring of the lungs causing their long-term deterioration. Its diagnosis requires the existence of the radiological pattern called usual interstitial pneumonia (UIP). Its prevalence is higher in men. Among the main symptoms, we find dyspnea and cough. At present, there is no known cure, however, there are various antifibrotic treatments focused on slowing the progression of this pathology and prolonging survival. The aim of this study was to evaluate the effectiveness and safety of pirfenidone in this disease.Methods: Retrospective descriptive study from January 2016 to December 2019. The study included 40 patients diagnosed with idiopathic pulmonary fibrosis who started treatment with pirfenidone.Results: The pulmonary function parameters showed values that were maintained during the study period. There was an improvement in the cough. The most common adverse reactions were gastrointestinal and skin disorders.Conclusion: Pirfenidone treatment requires rigorous monitoring due to the percentage of patients who abandoned this therapy. (AU)

Idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease. It is associated with a very poor prognosis. Treatments can delay the progression of IPF, so early diagnosis is fundamental. Radiologists play a fundamental role in the evaluation and accurate diagnosis of IPF. Identifying the characteristic patterns of IPF on high-resolution computed tomography (HRCT) is key in the process of multidisciplinary diagnosis, often obviating the need for surgical lung biopsies. This review describes and illustrates the clinical and imaging findings in IPF in the context of the most recent international guidelines, as well as the differential diagnosis and the role of HRCT in follow-up and assessment of complications.

Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials. METHODS: Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed. RESULTS: 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern. CONCLUSIONS: Patients with preserved FVC but presenting UIP radiological pattern and moderate-severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

Tendencia de la mortalidad por fibrosis pulmonar idiopática en Chile / Trend in idiopathic pulmonary fibrosis in Chile

INTRODUCCIÓN: La Fibrosis Pulmonar Idiopática (FPI) es una de las enfermedades respiratorias crónicas del adulto de mayor impacto y letalidad, diversos estudios epidemiológicos muestran tendencias progresivas al aumento de las tasas de mortalidad por FPI. En Chile no existen reportes sobre las tendencias de las tasas de mortalidad por FPI. El objetivo del presente estudio es determinar las tendencias de la mortalidad por FPI en Chile entre los años 2002 y 2015. MÉTODO: Estudio descriptivo de diseño ecológico, a partir de la información de bases de datos secundarias de libre disposición de las estadísticas vitales del Departamento de Estadísticas e Información de Salud (DEIS) y del Instituto Nacional de Estadísticas (INE) de Ministerio de Salud de Chile entre los años 2002 y 2015 se obtuvieron las tasas crudas de mortalidad por fibrosis pulmonar idiopática en población de 45 años y más en ambos sexos y las tasas ajustadas por sexo y edad por regiones, se calculó también la frecuencia mensual de las muertes por FPI y se compararon las tasas medias de mortalidad por regiones. RESULTADOS: Se observó un incremento progresivo de la tasa nacional cruda de mortalidad por FPI entre los años 2002 a 2015, la que fue de 18,5 fallecidos por 100.000 habitantes en el año 2002 hasta 24,6 fallecidos por 100.000 habitantes en el año 2015 con una pendiente de ascenso por año de +0,27 por 100.000 habitantes (p = 0,013). En las mujeres las tasas fueron más altas que en los hombres, pero las pendientes de ascenso no presentaron diferencias entre sexos. En la gran mayoría de las regiones las tasas ajustadas presentaron tendencias significativas al ascenso y las tasas medias más altas se presentaron en las regiones del norte de Chile. Se observó un comportamiento estacional de las muertes siendo las frecuencias más altas en los meses de invierno. CONCLUSIONES: Las tasas de mortalidad por FPI en Chile presentan una tendencia progresiva al aumento, con marcadas diferencias regionales lo que lleva a considerar, entre otros factores, influencia ambiental y contaminación del aire y de suelos que se debieran investigar para poder realizar intervenciones de salud pública que permitan reducir la mortalidad de esta enfermedad en nuestro país.

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is one of the chronic respiratory diseases in adults with the greatest impact and high case fatality rate. Various epidemiological studies show progressive trends towards increasing IPF mortality rates. In Chile there are no national reports on country and regional trends in IPF mortality rates. The objective of this study is to determine trends in mortality due to IPF in Chile from year 2002 to 2015. METHOD: Epidemiological study of ecological design based on information from public databases of vital statistics of the Department of Health Statistics and Information (DEIS) and the National Institute of Statistics (INE) of the Ministry of Health of Chile. Crude mortality rates due to IPF in the population aged 45 years and over in both sexes were obtained from years 2002 to 2015. Besides the adjusted mortality rates for sex and age by region, the monthly frequency of IPF deaths during the same period and the average mortality rates by Chilean regions were calculated. RESULTS: A progressive increasing trend in the crude national IPF mortality rate was observed between years 2002 to 2015, which went from 18.5 deaths per 100,000 inhabitants in 2002 to 24.6 deaths per 100,000 inhabitants in 2015 with a slope of ascent per year of +0.27 per 100,000 inhabitants (p = 0.013); female rates were higher than men rates, but without differences in the slopes between sexes; in the vast majority of the regions the rates showed significant upward trends with the higher ones in the northern regions of Chile. A seasonal behavior of the death's frequency was observed being the highest in the winter term. CONCLUSIONS: Mortality rates due to IPF in Chile show a progressive upward trend, with marked regional differences which leads to consider, among other factors, environmental influence and air and soil contamination that should be investigated to carry out public health interventions that allow reducing the mortality of this disease in our country.

Fibrosis pulmonar idiopática. Características clínicas de un registro en Panamá. 2017-2020

Introducción: La fibrosis pulmonar idiopática es una enfermedad rara, asociada al envejecimiento, con una alta tasa de mortalidad entre los 2.5 a 3 años. El diagnóstico se realiza con la sospecha clínica y la confirmación del patrón de neumonía intersticial usual, en tomografía del tórax o en biopsia pulmonar; el tratamiento actual se basa en fármacos anti fibróticos. Material y método: Se llevó a cabo un estudio descriptivo y prospectivo tipo registro, de pacientes con fibrosis pulmonar idiopática, que estuviesen en seguimiento por médicos especialistas en neumología en 4 consultorios privados de la ciudad de Panamá entre el 2017 y el 2020. Resultado: Se evaluaron 32 pacientes, edad media de 69 años (rango 53 ­ 91); 18/32 (56.3%) fueron masculinos, y de estos, 16/18 (88.9%) eran fumadores. Los crepitantes tipo velcro subescapulares se encontraron en 29/32 (90.6%). El patrón de neumonía intersticial usual en tomografía se encontró en 30/32 (93.7%), y 2/32 (6.3%) en biopsia. Los medicamentos anti fibróticos fueron usados en 6/32 (18.6%); el 50% usó nintedanib y el 50% pirfenidona. Fallecieron 4/32 (12.5%) pacientes en el periodo de estudio. Conclusión: La fibrosis pulmonar idiopática es frecuente en mayores de 50 años, principalmente varones, con historial de tabaquismo y reflujo gastroesofágico. Los crepitantes tipo velcro subescapulares son un hallazgo frecuente. El diagnóstico se realizó en su mayoría con tomografía de tórax de alta resolución; la difusión de monóxido de carbono y la saturación en la caminata de 6 minutos son las pruebas funcionales más alteradas. Menos de un tercio de los pacientes recibe tratamiento con anti fibróticos. (provisto por Infomedic International)

Introduction: Idiopathic pulmonary fibrosis is a rare disease, associated with aging, with a high mortality rate between 2.5 to 3 years. Diagnosis is made with clinical suspicion and confirmation of the usual interstitial pneumonia pattern in chest tomography or lung biopsy; current treatment is based on antifibrotic drugs. Methods: A descriptive and prospective registry-type study was carried out on patients with idiopathic pulmonary fibrosis who were being followed up by pulmonology specialists in 4 private practices in Panama City between 2017 and 2020. Results: 32 patients were evaluated, mean age 69 years (range 53 - 91); 18/32 (56.3%) were male, and of these, 16/18 (88.9%) were smokers. Subscapularis velcro-type crackles were found in 29/32 (90.6%). The usual interstitial pneumonia pattern on CT was found in 30/32 (93.7%), and 2/32 (6.3%) on biopsy. Antifibrotic drugs were used in 6/32 (18.6%); 50% used nintedanib and 50% pirfenidone. Four/32 (12.5%) patients died during the study period. Conclusion: Idiopathic pulmonary fibrosis is frequent in patients older than 50 years, mainly males, with a history of smoking and gastroesophageal reflux. Subscapular Velcro-like crackles are a frequent finding. Diagnosis was mostly made with high-resolution chest CT; carbon monoxide diffusion and 6-minute walk saturation are the most altered functional tests. Less than one third of patients receive treatment with antifibrotic drugs. (provided by Infomedic International)

Clinical profile of idiopathic pulmonary fibrosis patients treated with nintedanib in routine clinical practice in Spain: the BROAD Study / Perfil clínico de pacientes con fibrosis pulmonar idiopática tratados con nintedanib en la práctica clínica habitual en españa: estudio broad

Background. Few data are available on the clinical profile of patients diagnosed with idiopathic pulmonary fibrosis (IPF) treated with nintedanib. The primary objective of the study was to describe, based on pulmonary function variables, disease severity in IPF patients who initiated treatment with nintedanib in routine clinical practice. The secondary objectives were to analyze their clinical characteristics and comorbidities. Methods. A multicenter, retrospective study including 173 patients from 32 Spanish hospitals. Patients were stratified by their forced vital capacity (FVC) % predicted and diffusing capacity for carbon monoxide (DLCO) % predicted. These measures were taken as a marker of IPF severity. Results. Mean age ± SD at treatment initiation was 70.1 ± 8.1, and 76.6% of patients were male. Based on FVC, 57% of patients had mild IPF (FVC ≥ 70%), 38.4% moderate IPF (FVC 50%-69%), and 4.7% severe IPF (FVC < 50%). Based on DLCO, 42.5% of patients had mild IPF (DLCO ≥ 50%), 35.5% moderate IPF (DLCO 35%-49%), and 22.2% severe IPF (DLCO < 35%). Eighty-nine percent of patients had at least one comorbid condition. The most prevalent comorbidities were high blood pressure (45.9%), dyslipidemia (42.4%), gastroesophageal reflux (25.6%), diabetes (19.8%), emphysema (15.7%), and cardiovascular diseases (15.7%). Most patients received concomitant treatment (79.7%). Conclusions. The study provides relevant information on the clinical characteristics of IPF patients who initiate nintedanib treatment. Classification of severity depends on the lung function parameter used. The proportion of patients classified as having severe IPF was up to 4 times greater when DLCO, instead of FVC, was used (AU)

Introducción. Se dispone de pocos datos sobre el perfil clínico de los pacientes diagnosticados de fibrosis pulmonar idiopática (FPI) tratados con nintedanib. El objetivo principal del estudio fue describir, basándose en variables de función pulmonar, la gravedad de la enfermedad en pacientes con FPI que iniciaron tratamiento con nintedanib en la práctica clínica habitual. Los objetivos secundarios fueron analizar sus características clínicas y comorbilidades. Métodos. Estudio retrospectivo y multicéntrico que incluyeron a 173 pacientes de 32 hospitales españoles. Los pacientes fueron estratificados por su capacidad vital forzada (CVF) % predicho y por la capacidad de difusión de monóxido de carbono (DLCO) % predicho. Estas variables se consideraron como marcadores de la gravedad de la FPI. Resultados. La edad media ± DE al inicio del tratamiento fue de 70,1 ± 8,1 y el 76,6% de los pacientes eran varones. Según la CVF, el 57% de los pacientes tenían FPI leve (CVF ≥ 70%), el 38,4% FPI moderada (CVF 50%-69%) y el 4,7% FPI grave (CVF < 50%). Según la DLCO, el 42,5% de los pacientes tenían FPI leve (DLCO ≥ 50%), el 35,5% FPI moderada (DLCO 35%-49%) y el 22,2% FPI grave (DLCO < 35%). El 89% de los pacientes tenían al menos una comorbilidad, siendo las más prevalentes la hipertensión arterial (45,9%), dislipidemia (42,4%), reflujo gastroesofágico (25,6%), diabetes (19,8%), enfisema (15,7%) y enfermedades cardiovasculares (15,7%). La mayoría de los pacientes recibieron tratamientos concomitantes (79,7%) (AU)