ABSTRACT
Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial lung disease with a poor prognosis. 68Ga-labeled FAP ligands exhibited highly promising results due to the crucial role of activated fibroblasts in fibrosis imaging of the lung. However, 18F-labeled FAP ligands might provide qualitatively much higher imaging results with accompanying economic benefits due to large-scale production. Thus, we sought to investigate the potential of [18F]FAPI-74 prospectively in a small patient cohort. METHODS: Eight patients underwent both [18F]FAPI-74-PET/CT and HRCT scans and were then compared with a control group without any fibrosing pulmonary disease. The tracer uptake of fibrotic lung areas was analyzed in synopsis with radiological and clinical parameters. RESULTS: We observed a positive correlation between the fibrotic active volume, the Hounsfield scale, as well as the vital and diffusing capacity of the lung. CONCLUSION: The initial results confirm our assumption that [18F]FAPI-74 offers a viable non-invasive assessment method for pulmonary fibrotic changes in patients with IPF.
Subject(s)
Idiopathic Pulmonary Fibrosis , Positron Emission Tomography Computed Tomography , Humans , Idiopathic Pulmonary Fibrosis/diagnostic imaging , Male , Female , Aged , Middle Aged , Lung/diagnostic imagingABSTRACT
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and frequently fatal disease. Currently there are national and multinational registries in Europe, United States, Australia and China to better understand the magnitude of the problem and the characteristics of the IPF patients. However, there are no national or regional registries in Latin America, so the objective of this study was to carry out a Latin American registry that would allow the identification of IPF patients in our region. METHODOLOGY: A system consisting of 3 levels of control was designed, ensuring that patients met the diagnostic criteria for IPF according to international guidelines ATS/ERS/ALAT/JRS 2011. Demographic, clinical, serological, functional, tomographic, histological and treatment variables were recorded through a digital platform. RESULTS: 761 IPF patients from 14 Latin American countries were included for analysis, 74.7% were male, with a mean age of 71.9+8.3 years. In general there was a long period of symptoms before definitive diagnosis (median 1 year). In functional tests, an average reduction of FVC (70.9%) and DLCO (53.7%) was detected. 72% received at least one antifibrotic drug (pirfenidone or nintedanib) and 11.2% of the patients had an acute exacerbation, of which 38 (45.2%) died from this cause. CONCLUSIONS: Like other registries, we found that there is difficulty in the recognition and excessive delay in the diagnosis of IPF in Latin America. Most of the patients in REFIPI received antifibrotics; these were well tolerated and associated with fewer adverse events than those reported in clinical trials.
Subject(s)
Idiopathic Pulmonary Fibrosis , Humans , Male , United States , Middle Aged , Aged , Aged, 80 and over , Female , Idiopathic Pulmonary Fibrosis/diagnosis , Idiopathic Pulmonary Fibrosis/drug therapy , Idiopathic Pulmonary Fibrosis/epidemiology , Latin America/epidemiology , Pyridones/therapeutic use , Registries , Europe , Tomography, X-Ray Computed/methods , Treatment OutcomeABSTRACT
El Coronavirus SARS-CoV-2 produce la enfermedad COVID-19, cuya manifestación más grave y potencialmente letal es la neumonía. En este artículo revisaremos las manifestaciones clínicas del COVID-19, la fisiopatología de la neumonía, el manejo intrahospitalario previo al ingreso a Unidades de Cuidados Intensivos, la embolia pulmonar que es una complicación muy frecuente de esta enfermedad y el seguimiento de los pacientes posterior al alta. Para esta publicación nos hemos basado en publicaciones médicas y en estudios que hemos hecho durante esta pandemia en nuestro Centro de Enfermedades Respiratorias. i:es
The SARS-CoV-2 Coronavirus causes the COVID-19 disease, the most severe and potentially fatal manifestation of which is pneumonia. In this article, we will review the clinical manifestations of COVID-19, the pathophysiology of pneumonia, in-hospital management prior to admission to Intensive Care Units, pulmonary embolism, which is a very frequent complication of this disease, and the follow-up of patients after hospitalization. For this publication we have relied on medical publications and studies that we have done during this pandemic at our Center for Respiratory Diseases. i:en
Subject(s)
Humans , Pneumonia/physiopathology , Pneumonia/therapy , COVID-19/physiopathology , COVID-19/therapy , Oxygen Inhalation Therapy , Pneumonia/etiology , Pulmonary Embolism , Risk Factors , Adrenal Cortex Hormones/therapeutic use , Noninvasive Ventilation , SARS-CoV-2/pathogenicity , COVID-19/complications , COVID-19/diagnosisABSTRACT
Interstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.
Subject(s)
Lung Diseases, Interstitial/classification , Lung Diseases, Interstitial/diagnosis , Prognosis , Connective Tissue Diseases/diagnosis , Diagnosis, Differential , Idiopathic Pulmonary Fibrosis/diagnosis , Alveolitis, Extrinsic Allergic/diagnosisABSTRACT
Interstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.
Subject(s)
Lung Diseases, Interstitial/classification , Lung Diseases, Interstitial/diagnosis , Alveolitis, Extrinsic Allergic/diagnosis , Connective Tissue Diseases/diagnosis , Diagnosis, Differential , Idiopathic Pulmonary Fibrosis/diagnosis , PrognosisABSTRACT
Epithelioid hemangioendothelioma is a multifocal tumor that rarely metastasizes. It is difficult to diagnose, most often it is an incidental finding in young asymptomatic women. The radiologic pattern is heterogeneous. Histologic confirmation of Weibel-Palade bodies or immunohistochemistry based on specific tumor markers such as factor VIII and CD34 are the most important finding to confirm the diagnosis. We report a 21 years old woman Presenting with cough and dyspnea. A chest X ray was suggestive of tuberculosis. Sputum smears were negative for acid fat bacilli and the tuberculin test was negative. A chest CAT scan showed multiple nodular lesions. A surgical biopsy of the lesions confirmed the presence of a hemangioendothelioma. The patient was initially treated with prednisone and azathioprine without response. Thereafter, the patient is without treatment and without evidence of disease progression.
Subject(s)
Hemangioendothelioma, Epithelioid/diagnosis , Lung Neoplasms/diagnosis , Antimetabolites, Antineoplastic , Azathioprine/therapeutic use , Female , Hemangioendothelioma, Epithelioid/drug therapy , Humans , Lung Neoplasms/drug therapy , Prednisone/therapeutic use , Young AdultABSTRACT
Epithelioid hemangioendothelioma is a multifocal tumor that rarely metastasizes. It is difficult to diagnose, most often it is an incidental finding in young asymptomatic women. The radiologic pattern is heterogeneous. Histologic confirmation of Weibel-Palade bodies or immunohistochemistry based on specific tumor markers such as factor VIII and CD34 are the most important finding to confirm the diagnosis. We report a 21 years old woman Presenting with cough and dyspnea. A chest X ray was suggestive of tuberculosis. Sputum smears were negative for acid fat bacilli and the tuberculin test was negative. A chest CAT scan showed multiple nodular lesions. A surgical biopsy of the lesions confirmed the presence of a hemangioendothelioma. The patient was initially treated with prednisone and azathioprine without response. Thereafter, the patient is without treatment and without evidence of disease progression.
Subject(s)
Humans , Female , Young Adult , Hemangioendothelioma, Epithelioid/diagnosis , Lung Neoplasms/diagnosis , Azathioprine/therapeutic use , Prednisone/therapeutic use , Hemangioendothelioma, Epithelioid/drug therapy , Lung Neoplasms/drug therapy , Antimetabolites, AntineoplasticABSTRACT
BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is the most prevalent of all interstitial lung diseases. The usual underlying pathological picture is an interstitial pneumonia (UIP). AIM: To describe the evolution of a Chilean cohort of patients with IPF. MATERIAL AND METHODS: Patients with the disease were identified at the pathology registry of National Institute of Thoracic Diseases, Santiago, Chile. Patients were included if they had surgical biopsy of UIP and compatible clinical and radiological characteristics. The medical records of included patients were reviewed, recording clinical information and lung function test results. Survival was analyzed obtaining death records from the Chilean National Identification Service. RESULTS: Data from 142 patients with a mean age of 58 years (42% men), were analyzed. Mean initial lung function showed a forced vital capacity (FVC) of 73%, carbon monoxide diffusing capacity (DLCO) of 57% and a distance covered in 6-minute walk (6MWT) of 95% of expected normal values. The median survival was 80 months. Predictors of survival were a DLCO of less than 40% and an oxygen saturation at the end of the 6MWT of less than 89%. CONCLUSIONS: Survival in this group of patients was higher than the figures reported elsewhere. DLCO and the fall of oxygen saturation after walking were predictors of mortality, as previously described in other populations.
Subject(s)
Idiopathic Pulmonary Fibrosis/mortality , Biopsy , Cohort Studies , Female , Humans , Idiopathic Pulmonary Fibrosis/pathology , Lung/pathology , Male , Middle Aged , Respiratory Function Tests , Retrospective Studies , Survival AnalysisABSTRACT
Background: Idiopathic Pulmonary Fibrosis (IPF) is the most prevalent of all interstitial lung diseases. The usual underlying pathological picture is an interstitial pneumonia (UIP). Aim: To describe the evolution of a Chilean cohort of patients with IPF. Material and Methods: Patients with the disease were identified at the pathology registry of National Institute of Thoracic Diseases, Santiago, Chile. Patients were included if they had surgical biopsy of UIP and compatible clinical and radiological characteristics. The medical records of included patients were reviewed, recording clinical information and lung function test results. Survival was analyzed obtaining death records from the Chilean National Identification Service. Results: Data from 142 patients with a mean age of 58 years (42% men), were analyzed. Mean initial lung function showed a forced vital capacity (FVC) of 73%, carbon monoxide diffusing capacity (DLCO) of 57% and a distance covered in 6-minute walk (6MWT) of 95% of expected normal values. The median survival was 80 months. Predictors of survival were a DLCO of less than 40% and an oxygen saturation at the end of the 6MWT of less than 89%. Conclusions: Survival in this group of patients was higher than the figures reported elsewhere. DLCO and the fall of oxygen saturation after walking were predictors of mortality, as previously described in other populations.
Subject(s)
Female , Humans , Male , Middle Aged , Idiopathic Pulmonary Fibrosis/mortality , Biopsy , Cohort Studies , Idiopathic Pulmonary Fibrosis/pathology , Lung/pathology , Respiratory Function Tests , Retrospective Studies , Survival AnalysisABSTRACT
Se evalúa el efecto de una teofilina anhidra de liberación sostenida sobre los índices espirométricos y flujométricos y su relación con los niveles plasmáticos de teofilina en 12 pacientes asmáticos. La teofilina se administró en forma doble ciego en tres períodos consecutivos de cuatro días cada uno. Los pacientes recibieron placebo, teofilina 250 mg. c/12 (T1) y teofilina 375 mg c/12 horas (T2). Al término de cada período se repitió la espsirometría, se midió el flujo espiratorio máximo (FEM), capacidad vital forzada (CVF) y e volumen espiratorio forzada del 1er segundo (VEF). Las teofilinemias fueron en promedio 10 microgramos/ml con dosis T1 y 15 microgramos/ml con T2. En comparación con placebo los pacientes en tratamiento T1 y T2 tuvieron una mejoría del fEM (349 vs 391 y 413 respectivamente (p < 0.005); de la CVF (-15 vs - 0,23 y + 3,9% respectivamente (p < 0.05). Se concluye que los dos esquemas de tratamiento con teofilina T1 y T2 evaluados tienen un efecto beneficioso sobre los índices espirométricos y flujométricos, con teofilinemias promedio de 10 y 20 µg/ml
