Prevalence and prognostic meaning of interstitial lung abnormalities in remote CT scans of patients with interstitial lung disease treated with antifibrotic therapy.

OBJECTIVES: To describe the prevalence and characteristics of interstitial lung abnormalities (ILA) in CT scans performed prior to the initiation of antifibrotics in a series of patients with interstitial lung disease (ILD), and to identify characteristics apparent on early CT scans that could help to predict outcomes. METHODS: We conducted a retrospective observational study. The original cohort consisted of 101 patients diagnosed with ILD and treated with antifibrotics in a tertiary hospital. Patients were included if they had a thoracic CT scan performed at least one year before initiation of therapy. They were classified radiologically in three groups: without ILA, with radiological ILA and extensive abnormalities. ILA were classified as subpleural fibrotic, subpleural non-fibrotic and non-subpleural. The initial scan and the latest CT scan performed before treatment were read for assessing progression. The relationship between CT findings of fibrosis and the radiological progression rate and mortality were analyzed. RESULTS: We included 50 patients. Only 1 (2%) had a normal CT scan, 25 (50%) had extensive alterations and 24 (48%) had radiological criteria for ILA, a median of 98.2 months before initiation of antifibrotics, of them 18 (75%) had a subpleural fibrotic pattern. Significant bronchiectasis and obvious honeycombing in the lower zones were associated with shorter survival (p = 0.04). Obvious honeycombing in the lower zones was also significantly (p < 0.05) associated with a faster progression rate. CONCLUSIONS: Fibrotic ILAs are frequent in remote scans of patients with clinically relevant ILD, long before they require antifibrotics. Findings of traction bronchiectasis and honeycombing in the earliest scans, even in asymptomatic patients, are related to mortality and progression later on.

Riesgo cardiovascular en pacientes con esclerosis sistémica / Cardiovascular Risk in Patients with Systemic Sclerosis

Introducción: El riesgo cardiovascular es importante en la evaluación de los pacientes con esclerosis sistémica. Objetivo: Determinar el riesgo cardiovascular en pacientes con esclerosis sistémica. Métodos: Se realizó un estudio transversal y descriptivo en pacientes protocolizados del Servicio de Reumatología, en el período de enero 2020 a enero 2022. Se recogieron variables demográficas, clínicas, y se aplicó la calculadora de riesgo cardiovascular Framingham. Resultados: Se incluyeron 105 pacientes con edad media de 48,6 ± 15,3 años, el grupo más frecuente de 50 a 59 años (36,2 por ciento), predominó el sexo femenino 92,2 por ciento el color de piel blanca (74,3 por ciento), el tiempo de evolución fue mayor a 5 años (66,7 por ciento) con una media de 10,5 ± 9,3. El valor promedio de la escala de gravedad modificada de Medsger fue 5,1 ± 2,7 y el 72,4 por ciento con afectación leve. El fenómeno de Raynaud y la fibrosis pulmonar fueron más frecuentes con un 89,5 por ciento y 55,2 por ciento. El índice de Rodnan en promedio fue de 13,1 ± 8,0 y los reactantes de fase aguda normales en la mayoría. Los factores de riesgo cardiovascular más frecuentes fueron la HTA (30,2 por ciento) y dislipidemia (19,9 por ciento). El índice de masa corporal que predominó fue de peso adecuado (54,3 por ciento). Predominó el riesgo cardiovascular bajo según score de Framingham (86 por ciento). Existieron diferencias significativas entre las medias del tiempo de evolución y el riesgo cardiovascular (10 ± 6,9 frente a 9,6 ± 8,8 frente a 16,9 ± 10,8; p = 0,032). Conclusiones: El riesgo cardiovascular en los pacientes con esclerosis sistémica fue bajo(AU)

Introduction: Cardiovascular risk is important in the evaluation of patients with systemic sclerosis. Objective: To determine the cardiovascular risk in patients with systemic sclerosis. Methods: A cross-sectional and descriptive study was carried out in protocolized patients of Rheumatology Service, from January 2020 to January 2022. Demographic and clinical variables were collected, and Framingham cardiovascular risk calculator was used. Results: One hundred five patients were included with a mean age of 48.6 ± 15.3 years, the most frequent group was 50 to 59 years (36.2percent), female sex (92.2percent) predominated, as well as white skin color (74.3percent). The evolution time was greater than 5 years (66.7percent) with a mean of 10.5 ± 9.3. The average value of modified Medsger severity scale was 5.1 ± 2.7 and 72.4percent had mild involvement. Raynaud's phenomenon and pulmonary fibrosis were more common at 89.5percent and 55.2percent. Rodnan index on average was 13.1 ± 8.0 and the acute phase reactants were normal in the majority. The most frequent cardiovascular risk factors were HBP (30.2percent) and dyslipidemia (19.9percent). The predominant body mass index was adequate weight (54.3percent). Low cardiovascular risk according to Framingham score prevailed (86percent). There were significant differences between the mean duration of evolution and cardiovascular risk (10 ± 6.9 vs. 9.6 ± 8.8 vs. 16.9 ± 10.8; p = 0.032). Conclusions: The cardiovascular risk in patients with systemic sclerosis was low(AU)

Un paciente con... Tos / A patient with... Cough

El 3,3-10% de la población presenta tos crónica, siendo un motivo frecuente de consulta en Atención Primaria (AP). Se plantea el caso de una mujer de 61 años con tos de 3 meses de evolución. La sospecha diagnóstica es de fibrosis pulmonar en el contexto de una posible artritis reumatoide versus neumonía intersticial descamativa secundaria a aripiprazol. El caso clínico sirve de reflexión sobre la importancia de una buena anamnesis en AP, así como un correcto uso de las pruebas complementarias para valorar causas menos frecuentes de un síntoma común. Debe desarrollarse una capacidad inductiva y valorar el contexto del síntoma guía para confeccionar un diagnóstico sindrómico lo más acertado posible, lo cual es muy valioso en este nivel asistencial. (AU)

Chronic cough is a common reason for consultation in primary care (prevalence approximately 3.3% to 10.0%). We report a case of a 61-year-old woman with a three-month history of cough. The most accurate diagnosis is pulmonary fibrosis in the context of rheumatoid arthritis versus desquamative interstitial pneumonia secondary to aripiprazole. This case study serves as a reflection on the importance of a good history in primary care. Moreover, it is relevant to correctly use complementary tests. It is especially important to make a good syndromic diagnosis and evaluate the context of the patient in front of us. (AU)

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.

Toxicidad pulmonar secundaria a bleomicina. Reporte de un caso / Pulmonary Toxicity Secondary to Bleomycin. A Case Report

La toxicidad pulmonar por antineoplásicos es muy variable dependiente del grupo far-macológico; la bleomicina es uno de los medicamentos en los que se ha reportado este evento. Este citostático puede lesionar el endotelio pulmonar y el epitelio alveolar para llevar a un proceso inflamatorio y fibrótico del intersticio con repercusiones potencial-mente fatales.A continuación, se presenta un caso de enfermedad intersticial tipo neumonía organi-zada asociada a bleomicina en un paciente de 68 años con diagnóstico linfoma Hodg-kin clásico de tipo esclerosis nodular, con estudio imagenológico normal previo al tratamiento

Antineoplastic pulmonary toxicity is highly variable depending on the pharmacological group; bleomycin is one of the drugs in which this event has been reported. This cyto-static can injure the pulmonary endothelium and the alveolar epithelium to lead to an in-flammatory and fibrotic process of the interstitium with potentially fatal repercussions. The following is a case of interstitial disease type organizing pneumonia associated with bleomycin in a 68-year-old patient diagnosed with classical Hodgkin lymphoma of nodular sclerosis type, with imaging study prior to normal treatment

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.

Fibroelastosis pleuroparenquimatosa e hipertension pulmonar: un fenotipo diferente?

La fibroelastosis pleuroparenquimatosa es una enfermedad pulmonar inusual con características clínicas, radiológicas y patológicas únicas, que se ha incluido recientemente en el consenso actualizado sobre neumonías intersticiales idiopáticas. Su nombre hace referencia a una combinación de fibrosis que involucra la pleura visceral y cambios fibroelastóticos que predominan en el parénquima pulmonar subpleural. Aunque se han descrito varias asociaciones de enfermedades, no se ha identificado de manera inequívoca ninguna causa única. El diagnóstico se sustenta en criterios clínicos-radiológicos y de hallazgos histopatológicos cuando se dispone de biopsias. Son escasos los reportes sobre la asociación entre esta entidad y la hipertensión pulmonar. No existe tratamiento hasta la fecha, aunque se relatan sucesos en algunos casos de transplante pulmonar. Describimos las características clínicas de un paciente atendido en un hospital del Paraguay.

Pleuroparenchymal fibroelastosis is an unusual lung disease with unique clinical, radiological, and pathological characteristics that has recently been included in the updated consensus on idiopathic interstitial pneumonia. Their name refers to a combination of fibrosis involving the visceral pleura and fibro-elastic changes that predominate in the subpleural pulmonary parenchyma. Although several associations between diseases have been described, no single cause has been unequivocally identified. The diagnosis is based on clinical-radiological criteria and histopathological findings when biopsies are available. There are few reports on the association between this entity and pulmonary hypertension. There is no treatment to date, although some cases of lung transplantation have been successful. We describe the clinical characteristics of a patient treated in a hospital in Paraguay.

Fibrosis pulmonar familiar en el síndrome de Hermansky-Pudlak

La fibrosis pulmonar familiar en el contexto del síndrome de Hermansky-Pudlak es una entidad nosológica poco frecuente y cuyo único enfoque terapéutico definitivo es el trasplante pulmonar en los estadios avanzados. Describimos algunos aspectos clínicos diagnósticos y terapéuticos que configuran el manejo de las enfermedades huérfanas en países como el nuestro.

Family pulmonary fibrosis in the context of Hermansky-Pudlak syndrome is a rare nosological entity whose only definitive therapeutic approach is lung transplantation in the advanced stages. We describe some diagnostic and therapeutic clinical aspects that shape the management of orphan diseases in countries like ours.

Síndrome combinado de fibrosis pulmonar y enfisema: albores del tratamiento antifibrótico en el Paraguay

La concomitancia de fibrosis pulmonar en regiones inferiores del pulmón y enfisema en lóbulos superiores caracterizan a una entidad poco frecuente denominada síndrome combinado de fibrosis pulmonar y enfisema, que usualmente se asocia a alta carga de tabaquismo, aunque se describen otras asociaciones y que conlleva un pronóstico sombrío. La hipertensión pulmonar, el cáncer de pulmón y la exacerbación aguda son complicaciones posibles. En el contexto de un enfoque multidisciplinar de manejo de tres casos, relatamos el curso del tratamiento con pirfenidona.

The concomitance of pulmonary fibrosis in the lower regions of the lung and emphysema in the upper lobes characterize a rare entity called combined pulmonary fibrosis and emphysema syndrome, which is usually associated with a high burden of smoking, although other associations have been described and which carries a poor prognosis. Pulmonary hypertension, lung cancer, and acute exacerbation are possible complications. In the context of a multidisciplinary approach to the management of three cases, we report the course of treatment with pirfenidone.

Caracterización de los hallazgos tomográficos en pacientes con la COVID 19 y sospecha de fibrosis pulmonar

Introducción: La evaluación tomográfica del tórax permite conocer las complicaciones en pacientes con COVID-19, es la fibrosis pulmonar una de las más temidas, por su repercusión en la funcionabilidad pulmonar. Objetivo: Caracterizar los hallazgos tomográficos en pacientes con COVID-19 y sospecha de fibrosis pulmonar. Métodos: Se realizó un estudio observacional descriptivo retrospectivo en el Hospital Cubano de Qatar, en el período comprendido entre abril de 2020 a abril de 2021, el universo de estuvo constituido por 129 pacientes con COVID-19, la muestra quedó conformada por 85 pacientes con sospecha radiográfica de fibrosis pulmonar. La información se obtuvo de las historias clínicas. Se consideraron los principios bioéticos. Resultados: Predominó el sexo masculino, el grupo de edad de 45-59 años. El patrón de vidrio deslustrado fue el más frecuente en el 60 % de los casos. Existieron signos tomográficos de fibrosis pulmonar en el 58,62 %, con diagnóstico en fase avanzada, extensión moderada, localización bilateral y difusa. La afectación multilobar fue la más frecuente en pacientes con fibrosis evidente y en los dudosos de fibrosis pulmonar. Conclusiones: Predominaron los pacientes del sexo masculino con edades entre 45-59 años. El patrón de vidrio deslustrado fue el más frecuente. En más de la mitad de los pacientes se evidenciaron signos de fibrosis pulmonar en la fase avanzada de la COVID-19, con extensión moderada.

Introduction: The tomographic evaluation of the thorax allows us to know the complications in patients with COVID-19, pulmonary fibrosis being one of the most feared, due to its impact on lung function. Objective: To characterize the tomographic findings in patients with COVID-19 and suspected pulmonary fibrosis. Methods: A retrospective descriptive observational study was carried out at the Cuban Hospital in Qatar, between April 2020 and April 2021, the universe consisted of 129 patients with COVID 19, the sample was made up of 85 patients with radiographic suspicion of Pulmonary Fibrosis. The information was obtained from the medical records. Bioethical principles were taken into account. Results: The male sex prevailed, the age group of 45-59 years. The ground glass pattern was the most frequent in 60% of the cases. There were tomographic signs of Pulmonary Fibrosis in 58.62%, with diagnosis in advanced phase, moderate extension, bilateral and diffuse location. Multilobar involvement was the most frequent in patients with evident fibrosis and in those with doubtful pulmonary fibrosis. Conclusions: Male patients aged between 45-59 years predominated. The ground glass pattern was the most frequent. In more than half of the patients, signs of pulmonary fibrosis were evidenced in the advanced phase of COVID -19, with moderate extension. Bilateral and diffuse localization predominated. Multilobar involvement was the most frequent in patients with evident fibrosis and in those with doubtful pulmonary fibrosis. The contribution of this research lies in the fact that with the characterization of the tomographic findings in patients with COVID 19, future complications are prevented.

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.

Principales secuelas respiratorias del Covid-19: una revisión exploratoria / oria Main respiratory sequelae of Covid-19: an exploratory review

La Covid-19 es una enfermedad infecciosa que ha causado importantes tasas de morbi-mortalidad en la población mundial, la evidencia científica ha procurado describir la fisiopatogenia del SARS-CoV-2. En fase inicial, se produce un proceso inflamatorio agudo que empieza acentuándose en el tracto respiratorio y que afecta primera y principalmente al pulmón; lo que, posteriormente, puede desencadenar disfunciones respiratorias que perduran aún después del alta médico, lo que se considera como un síntoma persistente. De acuerdo con la metodología de la revisión sistemática exploratoria, se realizó esta investigación cuyo propósito se centró en encontrar referencias bibliográficas que sustente e identifiquen las principales secuelas respiratorias del Covid-19; para ello, se realizó una búsqueda de la literatura a través de las bases de datos PubMed, Scielo, Medline, Web of Science y Scopus. La indagación bibliográfica se llevó a cabo en enero de 2022, se dectectaron 70 documentos, los cuales fueron sometidos a evaluación de calidad. Se incluyeron 38 estudios observaionales, 3 artículos de revisión y 1 metaanálisis. Hasta la fecha no se ha llegado a un consenso respecto a la secuela más importante en el campo respiratorio post Covid, fundamentalmente destacan: fatiga, disnea, dolor torácico y la tos. Además, un porcentaje considerable de pacientes persisten con anomalías residuales de la función pulmonar, sugerentes fibrosis pulmonar, requiriendo asistencia médica aun despúes del alta hospitalario. Se recomienda continuar con investigaciones orientadas la valoración de las secuelas por Covid-19, para obtener basamentos claros relacionados con la capacidad funcional respiratoria post infección(AU)

Covid-19 is an infectious disease that has caused significant rates of morbidity and mortality in the world population, scientific evidence has sought to describe the pathophysiology of SARS-CoV-2. In the initial phase, an acute inflammatory process occurs that begins to be accentuated in the respiratory tract and affects the lungs first and foremost; which, subsequently, can trigger respiratory dysfunctions that persist even after medical discharge, which is considered a persistent symptom. In accordance with the methodology of the exploratory systematic review, this research was carried out whose purpose was focused on finding bibliographic references that support and identify the main respiratory sequelae of Covid-19; For this, a search of the literature was carried out through the PubMed, Scielo, Medline, Web of Science and Scopus databases. The bibliographic investigation was carried out in January 2022, 70 documents were detected, which were subjected to quality evaluation. 38 observational studies, 3 review articles and 1 meta-analysis were included. So far, no consensus has been reached regarding the most important sequela in the post-COVID respiratory field, fundamentally the following stand out: fatigue, dyspnea, chest pain and cough. In addition, a considerable percentage of patients persist with residual pulmonary function abnormalities, suggestive of pulmonary fibrosis, requiring medical assistance even after hospital discharge. It is recommended to continue with research aimed at assessing the sequelae of Covid-19, to obtain clear foundations related to post-infection respiratory functional capacity(AU)

COVID-19: Some unresolved issues / COVID-19: Algunos asuntos no resueltos

Two years after the COVID-19 pandemic, many uncertainties persist about the causal agent, the disease and its future. This document contains the reflection of the COVID-19 working group of the Official College of Physicians of Madrid (ICOMEM) in relation to some questions that remain unresolved. The document includes considerations on the origin of the virus, the current indication for diagnostic tests, the value of severity scores in the onset of the disease and the added risk posed by hypertension or dementia. We also discuss the possibility of deducing viral behavior from the examination of the structure of the complete viral genome, the future of some drug associations and the current role of therapeutic resources such as corticosteroids or extracorporeal oxygenation (ECMO). We review the scarce existing information on the reality of COVID 19 in Africa, the uncertainties about the future of the pandemic and the status of vaccines, and the data and uncertainties about the long-term pulmonary sequelae of those who suffered severe pneumonia. (AU)

Cuando han transcurrido ya dos años de la pandemia de COVID-19 persisten muchas incertidumbres sobre el agente causal, la enfermedad y su futuro. El presente documento contiene la reflexión del grupo de trabajo sobre COVID-19 del Ilustre Colegio Oficial de Médicos de Madrid (ICOMEM) en relación a algunas preguntas que nos parecen sin resolver. El documento incluye reflexiones sobre el origen del virus, la indicación actual de pruebas diagnósticas, el valor de los “scores” de gravedad en el comienzo de la enfermedad y el riesgo añadido que supone la hipertensión o la demencia. Se discute también, la posibilidad de deducir del examen de la estructura del genoma viral completo el comportamiento viral, el futuro de algunas asociaciones de fármacos y el papel actual de recursos terapéuticos como los corticoides o la oxigenación extracorpórea (ECMO). Revisamos la escasa información existente sobre la realidad de la COVID-19 en África, las incertidumbres sobre el futuro de la pandemia y la situación de las vacunas y los datos e incertidumbres sobre las secuelas pulmonares a largo plazo de los que padecieron neumonía grave. (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.

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)

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.

Post-COVID-19 Pneumonia Pulmonary Fibrosis 3 Cases Reported / Fibrosis pulmonar posneumonía causada por COVID-19. Reporte de 3 casos

ABSTRACT The prevalence of respiratory complications subsequent to COVID-19 pneumonia is currently unknown, but the data obtained from previous coronavirus outbreaks may provide important information. The preliminary evidence supports the hypothesis that some survivors could develop long-term respiratory sequelae, being the pulmonary fibrosis the most important. We report three cases of patients hospitalized in the ward with moderate to severe COVID-19, never requiring mechanical respiratory assistance (MRA). Follow-up computed tomography scans after discharge showed images compatible with post-pneumonia pulmonary fibrosis.

RESUMEN La prevalencia de complicaciones respiratorias posteriores a la neumonía por COVID-19 se desconoce actualmente, pero los datos obtenidos de brotes anteriores de coronavirus, pueden proporcionar información importante. La evidencia preliminar apoya la hipótesis de que algunos sobrevivientes podrían desarrollar secuelas respiratorias a largo plazo, entre ellas, la principal sería la fibrosis pulmonar. Se presentan tres casos de pacientes que fueron internados en sala por COVID-19 de moderado a grave, pero sin requerimiento de ARM en ningún momento, y que, en controles tomográficos posteriores al alta, presentaron imágenes compatibles con fibrosis pulmonar posterior a la neumonía.