Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 192
Filtrar
1.
J Thorac Imaging ; 39(1): W5-W10, 2024 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-37423612

RESUMEN

Many lung diseases, first, tumors, are smoking-related, and it is very likely to find more than one pattern in the same patient. Airspace enlargement with fibrosis (AEF) is one of them, but it has not been deeply understood or studied yet. In fact, we think that it might still be wrongly assimilated with other conditions that have different radiological features altogether and different prognoses. This pictorial essay is aimed at pointing out AEF so that radiologists and pulmonologists get acquainted with it and use the proper terminology, as AEF might not be that rare.


Asunto(s)
Enfisema , Enfisema Pulmonar , Fibrosis Pulmonar , Humanos , Enfisema Pulmonar/diagnóstico por imagen , Fibrosis Pulmonar/diagnóstico por imagen , Fumar , Pronóstico , Enfisema/patología , Pulmón/diagnóstico por imagen , Pulmón/patología
3.
Clin Exp Med ; 23(8): 4721-4728, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37803100

RESUMEN

Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs), with incidence and prevalence variously assessed in the literature but reported in up to 30% of patients, with higher frequency in rheumatoid arthritis (RA) and systemic sclerosis (SSc). Recent years have seen a growing interest in the pulmonary manifestations of ILD-CTDs, mainly due to the widening of the use of anti-fibrotic drugs initially introduced exclusively for IPF, and radiologists play a key role because the lung biopsy is very rarely used in these patients where the morphological assessment is essentially left to imaging and especially HRCT. In this narrative review we will discuss, from the radiologist's point of view, the most recent findings in the field of ILD secondary to SSc and RA, with a special focus about the progression of disease and in particular about the 'progressive pulmonary fibrosis' (PPF) phenotype, and we will try to address two main issues: How to predict a possible evolution and therefore a worse prognosis when diagnosing a new case of ILD-CTDs and how to assess the progression of an already diagnosed ILD-CTDs.


Asunto(s)
Artritis Reumatoide , Enfermedades Pulmonares Intersticiales , Fibrosis Pulmonar , Esclerodermia Sistémica , Humanos , Fibrosis Pulmonar/etiología , Fibrosis Pulmonar/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/etiología , Pulmón/diagnóstico por imagen , Pulmón/patología , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/diagnóstico por imagen , Esclerodermia Sistémica/patología , Artritis Reumatoide/complicaciones , Artritis Reumatoide/diagnóstico por imagen , Artritis Reumatoide/patología , Tomografía Computarizada por Rayos X
5.
Minerva Med ; 114(5): 608-619, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37204783

RESUMEN

BACKGROUND: During the last decade, a number of clinical scores, such as Gender-Age-Physiology (GAP) Index, TORVAN Score and Charlson Comorbidity Index (CCI), have been separately used to measure comorbidity burden in idiopathic pulmonary fibrosis (IPF). However, no previous study compared the prognostic value of these scores to assess mortality risk stratification in IPF patients with mild-to-moderate disease. METHODS: All consecutive patients with mild-to-moderate IPF who underwent high-resolution computed tomography, spirometry, transthoracic echocardiography and carotid ultrasonography at our Institution, between January 2016 and December 2018, were retrospectively analyzed. GAP Index, TORVAN Score and CCI were calculated in all patients. Primary endpoint was all-cause mortality, whereas secondary endpoint was the composite of all-cause mortality and rehospitalizations for all-causes, over medium-term follow-up. RESULTS: Seventy IPF patients (70.2±7.4 yrs, 74.3% males) were examined. At baseline, GAP Index, TORVAN Score and CCI were 3.4±1.1, 14.7±4.1 and 5.3±2.4, respectively. A strong correlation between coronary artery calcification (CAC) and common carotid artery (CCA) intima-media thickness (IMT) (r=0.88), CCI and CAC (r=0.80), CCI and CCA-IMT (r=0.81), was demonstrated in the study group. Follow-up period was 3.5±1.2 years. During follow-up, 19 patients died and 32 rehospitalizations were detected. CCI (HR 2.39, 95% CI: 1.31-4.35) and heart rate (HR 1.10, 95% CI: 1.04-1.17) were independently associated with primary endpoint. CCI (HR 1.54, 95% CI: 1.15-2.06) predicted secondary endpoint, also. A CCI ≥6 was the optimal cut-off for predicting both outcomes. CONCLUSIONS: Due to the increased atherosclerotic and comorbidity burden, IPF patients with CCI ≥6 at an early-stage disease have poor outcome over medium-term follow-up.


Asunto(s)
Enfermedad de la Arteria Coronaria , Fibrosis Pulmonar Idiopática , Masculino , Humanos , Femenino , Estudios Retrospectivos , Grosor Intima-Media Carotídeo , Comorbilidad , Fibrosis Pulmonar Idiopática/diagnóstico por imagen
6.
Intern Emerg Med ; 18(3): 755-767, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36966265

RESUMEN

During the last decade, the CHA2DS2-VASc score has been used for stratifying the mortality risk in both atrial fibrillation (AF) and non-AF patients. However, no previous study considered this score as a prognostic indicator in non-AF patients with mild-to-moderate idiopathic pulmonary fibrosis (IPF). All consecutive non-AF patients with mild-to-moderate IPF, diagnosed between January 2016 and December 2018 at our Institution, entered this study. All patients underwent physical examination, blood tests, spirometry, high-resolution computed tomography and transthoracic echocardiography. CHA2DS2-VASc score, Gender-Age-Physiology (GAP) index and Charlson Comorbidity Index (CCI) were determined in all patients. Primary endpoint was all-cause mortality, while the secondary endpoint was the composite of all-cause mortality and rehospitalizations for all causes over mid-term follow-up. 103 consecutive IPF patients (70.7 ± 7.3 yrs, 79.6% males) were retrospectively analyzed. At the basal evaluation, CHA2DS2-VASc score, GAP index and CCI were 3.7 ± 1.6, 3.6 ± 1.2 and 5.5 ± 2.3, respectively. Mean follow-up was 3.5 ± 1.3 yrs. During the follow-up period, 29 patients died and 43 were re-hospitalized (44.2% due to cardiopulmonary causes). On multivariate Cox regression analysis, CHA2DS2-VASc score (HR 2.15, 95% CI 1.59-2.91) and left ventricular ejection fraction (LVEF) (HR 0.91, 95% CI 0.86-0.97) were independently associated with all-cause mortality in IPF patients. CHA2DS2-VASc score (HR 1.66, 95% CI 1.39-1.99) and LVEF (HR 0.94, 95% CI 0.90-0.98) also predicted the secondary endpoint in the same study group. CHA2DS2-VASc score > 4 was the optimal cut-off for predicting both outcomes. At mid-term follow-up, a CHA2DS2-VASc score > 4 predicts an increased risk of all-cause mortality and rehospitalizations for all causes in non-AF patients with mild-to-moderate IPF.


Asunto(s)
Fibrilación Atrial , Fibrosis Pulmonar Idiopática , Accidente Cerebrovascular , Masculino , Humanos , Femenino , Pronóstico , Estudios Retrospectivos , Volumen Sistólico , Función Ventricular Izquierda , Medición de Riesgo/métodos , Fibrosis Pulmonar Idiopática/complicaciones , Factores de Riesgo , Accidente Cerebrovascular/etiología
7.
Sarcoidosis Vasc Diffuse Lung Dis ; 39(2): e2022021, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36118536

RESUMEN

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic disease with a peculiar (typical) HRCT pattern, but biopsy can demonstrate usual interstitial pneumonia in patients with atypical patterns. It is unknown how progression pattern varies among different radiographic presentations of IPF. We sought to investigate the longitudinal radiographic evolution and survival of typical and non-typical patterns. Materials and Methods: One-hundred-twenty-three patients diagnosed with IPF in 2 tertiary referral hospitals were included in the study. Longitudinal evolution of non-typical patterns was considered. The HRCT visual fibrosis score was used as a reliable evaluation tool of disease progression. HRCTs were scored by 2 senior chest radiologists with ILD expertise. The primary endpoint was the evolution of the presentation pattern to probable or typical. The secondary endpoint was lung transplant (LTx)-free survival from the time of diagnosis. Results: Average interval between HRCTs was 16±5 months; average follow-up after the 2nd HRCT was 17±11 months. Four out of 45 (8.9%) patients with probable pattern "evolved" to a typical pattern of IPF, while 5 out of 31 (16.1%) with indeterminate/alternative pattern "evolved" to probable pattern. An average HRCT fibrosis score increase of 9±11% was observed with typical (n=49), 6±5% with probable (n=43) and 7±8% (n=31) with indeterminate/alternative presentation pattern. LTx-free survival and lung function declines did not show any difference related to presentation HRCT patterns. Conclusions: The evolution of a non-typical UIP pattern to a typical one is infrequent. All presentation HRCT patterns of IPF evolve in similar way and are associated with comparable survival time.[/sc].

8.
Minerva Med ; 2022 Jun 07.
Artículo en Inglés | MEDLINE | ID: mdl-35671002

RESUMEN

BACKGROUND: Cardiovascular diseases are frequent in idiopathic pulmonary fibrosis (IPF) and impact on survival. We investigated the association of coronary artery calcium (CAC) score at IPF diagnosis and during mid-term follow-up, with adverse cardiovascular events and all-cause mortality. METHODS: Consecutive patients with IPF were retrospectively analyzed. Demographic data, smoking history, comorbidities and pulmonary function tests (PFTs) were recorded. All patients had at least two chest high resolution computed tomography (HRCT) performed 2 years apart. The total CAC score and visual fibrotic score were calculated and all clinically significant cardiovascular events and deaths were reported. RESULTS: The population consisted of 79 patients (57 male, mean age 74.4 ± 7.6 years); 67% of patients had a history of smoking, 48% of hypertension, 37% of dyslipidemia and 22.8% of diabetes. The visual score was 21.28 ± 7.99% at T0 and 26.54 ± 9.34% at T1, respectively (T1-T0 5.26 ± 6.13%, p< 0.001). CAC score at T0 and at T1 was 537.93 ± 839.94 and 759.98 ± 1027.6, respectively (T1-T0 224.66 ± 406.87, p< 0.001). Mean follow-up time was 2.47±1.1 years. On multivariate analysis, male sex (HR 3.58, 95% CI 1.14-11.2) and CAC score at T0 (HR 1.04, 95% CI 1.01-1.07) correlated with mortality and cardiovascular events. CAC score at T0 ≥405 showed 82% sensitivity and 100% specificity for predicting mortality and adverse cardiovascular events. CONCLUSIONS: IPF patients with a CAC score at diagnosis ≥405 have a poor prognosis over a midterm follow-up. A higher CAC score is associated with mortality and cardiovascular events.

9.
Respir Res ; 23(1): 147, 2022 Jun 07.
Artículo en Inglés | MEDLINE | ID: mdl-35672770

RESUMEN

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is associated with increased expression of cyclin-dependent kinase inhibitors such as p16 and p21, and subsequent induction of cell cycle arrest, cellular senescence, and pro-fibrotic gene expression. We sought to link p16-expression with a diagnosis of IPF or other fibrotic interstitial lung diseases (ILDs), radiographic pattern, senescent foci-specific gene expression, antifibrotic therapy response, and lung transplant (LTx)-free survival. METHODS: Eighty-six cases of fibrosing ILD were identified with surgical lung biopsy. Immunohistochemistry for p16 was performed on sections with the most active fibrosis. p16-positive foci (loose collection of p16-positive fibroblasts with overlying p16-positive epithelium) were identified on digital slides and quantified. Cases were scored as p16-low (≤ 2.1 foci per 100 mm2) or p16-high (> 2.1 foci per 100 mm2). Twenty-four areas including senescent foci, fibrotic and normal areas were characterized using in situ RNA expression analysis with digital spatial profiling (DSP) in selected cases. RESULTS: The presence of p16-positive foci was specific for the diagnosis of IPF, where 50% of cases expressed any level of p16 and 26% were p16-high. There was no relationship between radiographic pattern and p16 expression. However, there was increased expression of cyclin-dependent kinase inhibitors, collagens and matrix remodeling genes within p16-positive foci, and cases with high p16 expression had shorter LTx-free survival. On the other hand, antifibrotic therapy was significantly protective. DSP demonstrated that fibroblastic foci exhibit transcriptional features clearly distinct from that of normal-looking and even fibrotic areas. CONCLUSIONS: We demonstrated the potential clinical applicability of a standardized quantification of p16-positive fibroblastic foci. This method identifies an IPF phenotype associated with foci-specific upregulation of senescence-associated and matrix remodeling gene expression. While these patients have reduced LTx-free survival, good response to antifibrotic therapies was observed in those who were treated.


Asunto(s)
Fibrosis Pulmonar Idiopática , Enfermedades Pulmonares Intersticiales , Quinasas Ciclina-Dependientes/análisis , Quinasas Ciclina-Dependientes/genética , Quinasas Ciclina-Dependientes/metabolismo , Fibrosis , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Fibrosis Pulmonar Idiopática/genética , Pulmón/metabolismo , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/genética , Fenotipo
10.
Intern Emerg Med ; 17(7): 1907-1919, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-35753022

RESUMEN

The present study was primarily designed to validate the modified Haller index (MHI), the ratio of chest transverse diameter over the distance between sternum and spine, measured by a ruler and transthoracic echocardiography (TTE), respectively, in a cohort of subjects with obesity, but otherwise healthy, by comparing the results to the conventional Haller index (HI) measured on chest X-ray (CXR). 100 consecutive subjects with body mass index (BMI) ≥ 30 kg/m2 and 60 matched controls with BMI < 30 kg/m2, who underwent a two-plane CXR for any clinical indication, were prospectively examined over a 6-month period. All participants underwent MHI assessment, TTE and speckle-tracking analysis of left ventricular (LV) global longitudinal strain (GLS). Bland-Altman analysis was used to compare the radiological and nonradiological techniques. Second, independent predictors of subclinical myocardial dysfunction, defined as LV-GLS less negative than - 20%, were evaluated. Bland-Altman analysis revealed a bias of - 4.91 cm for latero-lateral thoracic diameter, of - 0.74 cm for antero-posterior (A-P) thoracic diameter and of - 0.22 for HI assessment, suggesting a systematic overestimation of the nonradiological methodology in comparison to that radiological. Despite normal LV systolic function on TTE, LV-GLS resulted impaired in 76% of subjects with obesity. Waist circumference (OR 1.13, 95%CI 1.04-1.22) and nonradiological A-P thoracic diameter (OR 0.51, 95%CI 0.28-0.93) were the main independent predictors of subclinical myocardial dysfunction in subjects with obesity. The impairment in LV myocardial strain detected in subjects with obesity appears to be primarily related to extrinsic abdominal and thoracic compressive phenomena, rather than intrinsic myocardial dysfunction.


Asunto(s)
Cardiomiopatías , Disfunción Ventricular Izquierda , Ecocardiografía/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Obesidad/complicaciones , Volumen Sistólico , Disfunción Ventricular Izquierda/diagnóstico por imagen
11.
Intern Emerg Med ; 17(6): 1679-1687, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35596103

RESUMEN

During the Coronavirus-19 pandemic, chest X-ray scoring system have been validated by Al-Smadi and Toussie in this group of patients and even RALE score, previously designed for ARDS, have been used to estimate correlation with mortality. The aim of this study was to evaluate the prognostic value of As-Smadi, Tuossie and RALE scores in predicting death in the same population of patients when associated to clinical data. In this retrospective clinical study, data of patients with COVID-19, admitted to our hospital from 1st October 2020 to 31st December 2020 were collected. CXR images of each patient were analyzed with the three different scores above mentioned. 144 patients (male 96 aged 68.5 years) were included in the study. 93 patients reported a least 1 comorbidity and 36 died. The association with increasing age, presence of comorbidities, and lower hemoglobin was significantly associated with risk of death for all the regression models. When considering the radiological score, a significant effect was found for the Al Smadi and RALE scores, while no evidence of association was found for the Toussie score. The fraction of new information is 16.7% for the Al Smadi score, 12.9% for the RALE and 5.1% for the Toussie score. The improvement in the prognostic usefulness with respect to the base model is particularly interesting for the Al Smadi score. The highest c-index was also obtained by the model with the Al Smadi score.


Asunto(s)
COVID-19 , Humanos , Masculino , Pronóstico , Ruidos Respiratorios , Estudios Retrospectivos , SARS-CoV-2
12.
Diagnostics (Basel) ; 12(4)2022 Apr 08.
Artículo en Inglés | MEDLINE | ID: mdl-35453983

RESUMEN

Background: Fibrosis in pulmonary Langerhans cell histiocytosis (PLCH) histologically comprises a central scar with septal strands and associated airspace enlargement that produce an octopus-like appearance. The purpose of this study was to identify the octopus sign on high-resolution computed tomography (HRCT) images to determine its frequency and distribution across stages of the disease. Methods: Fifty-seven patients with confirmed PLCH were included. Two experienced chest radiologists assessed disease stages as early, intermediate, or late, as well as the lung parenchyma for nodular, cystic, or fibrotic changes and for the presence of the octopus sign. Statistical analysis included Cohen's kappa for interrater agreement and Fisher's exact test for the frequency of the octopus sign. Results: Interobserver agreement was substantial for the octopus sign (kappa = 0.747). Significant differences in distribution of the octopus sign between stages 2 and 3 were found with more frequent octopus signs in stage 2 and fewer in stage 3. In addition, we only found the octopus sign in cases of nodular und cystic lung disease. Conclusions: The octopus sign in PLCH can be identified not only on histological images, but also on HRCT images. Its radiological presence seems to depend on the stage of PLCH.

13.
Eur J Intern Med ; 100: 110-118, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35483993

RESUMEN

RATIONALE AND OBJECTIVE: Various forms of Non-invasive respiratory support (NRS) have been used during COVID-19, to treat Hypoxemic Acute Respiratory Failure (HARF), but it has been suggested that the occurrence of strenuous inspiratory efforts may cause Self Induced Lung Injury(P-SILI). The aim of this investigation was to record esophageal pressure, when starting NRS application, so as to better understand the potential risk of the patients in terms of P-SILI and ventilator induced lung injury (VILI). METHODS AND MEASUREMENTS: 21 patients with early de-novo respiratory failure due to COVID-19, underwent three 30 min trials applied in random order: high-flow nasal cannula (HFNC), continuous positive airway pressure (CPAP), and non-invasive ventilation (NIV). After each trial, standard oxygen therapy was reinstituted using a Venturi mask (VM). 15 patients accepted a nasogastric tube placement. Esophageal Pressure (ΔPes) and dynamic transpulmonary driving pressure (ΔPLDyn), together with the breathing pattern using a bioelectrical impedance monitor were recorded. Arterial blood gases were collected in all patients. MAIN RESULTS: No statistically significant differences in breathing pattern and PaCO2 were found. PaO2/FiO2 ratio improved significantly during NIV and CPAP vs VM. NIV was the only NRS to reduce significantly ΔPes vs. VM (-10,2 ±5 cmH20 vs -3,9 ±3,4). No differences were found in ΔPLDyn between NRS (10,2±5; 9,9±3,8; 7,6±4,3; 8,8±3,6 during VM, HFNC, CPAP and NIV respectively). Minute ventilation (Ve) was directly dependent on the patient's inspiratory effort, irrespective of the NRS applied. 14% of patients were intubated, none of them showing a reduction in ΔPes during NRS. CONCLUSIONS: In the early phase of HARF due to COVID-19, the inspiratory effort may not be markedly elevated and the application of NIV and CPAP ameliorates oxygenation vs VM. NIV was superior in reducing ΔPes, maintaining ΔPLDyn within a range of potential safety.


Asunto(s)
COVID-19 , Ventilación no Invasiva , Insuficiencia Respiratoria , COVID-19/terapia , Humanos , Hipoxia/terapia , Ventilación no Invasiva/métodos , Terapia por Inhalación de Oxígeno/métodos , Insuficiencia Respiratoria/terapia
14.
Respir Res ; 23(1): 65, 2022 Mar 21.
Artículo en Inglés | MEDLINE | ID: mdl-35313890

RESUMEN

BACKGROUND: Long-term pulmonary sequelae following hospitalization for SARS-CoV-2 pneumonia is largely unclear. The aim of this study was to identify and characterise pulmonary sequelae caused by SARS-CoV-2 pneumonia at 12-month from discharge. METHODS: In this multicentre, prospective, observational study, patients hospitalised for SARS-CoV-2 pneumonia and without prior diagnosis of structural lung diseases were stratified by maximum ventilatory support ("oxygen only", "continuous positive airway pressure (CPAP)" and "invasive mechanical ventilation (IMV)") and followed up at 12 months from discharge. Pulmonary function tests and diffusion capacity for carbon monoxide (DLCO), 6 min walking test, high resolution CT (HRCT) scan, and modified Medical Research Council (mMRC) dyspnea scale were collected. RESULTS: Out of 287 patients hospitalized with SARS-CoV-2 pneumonia and followed up at 1 year, DLCO impairment, mainly of mild entity and improved with respect to the 6-month follow-up, was observed more frequently in the "oxygen only" and "IMV" group (53% and 49% of patients, respectively), compared to 29% in the "CPAP" group. Abnormalities at chest HRCT were found in 46%, 65% and 80% of cases in the "oxygen only", "CPAP" and "IMV" group, respectively. Non-fibrotic interstitial lung abnormalities, in particular reticulations and ground-glass attenuation, were the main finding, while honeycombing was found only in 1% of cases. Older patients and those requiring IMV were at higher risk of developing radiological pulmonary sequelae. Dyspnea evaluated through mMRC scale was reported by 35% of patients with no differences between groups, compared to 29% at 6-month follow-up. CONCLUSION: DLCO alteration and non-fibrotic interstitial lung abnormalities are common after 1 year from hospitalization due to SARS-CoV-2 pneumonia, particularly in older patients requiring higher ventilatory support. Studies with longer follow-ups are needed.


Asunto(s)
COVID-19/complicaciones , Enfermedades Pulmonares/diagnóstico , Enfermedades Pulmonares/virología , Anciano , COVID-19/diagnóstico , COVID-19/terapia , Femenino , Estudios de Seguimiento , Hospitalización , Humanos , Enfermedades Pulmonares/terapia , Masculino , Persona de Mediana Edad , Terapia por Inhalación de Oxígeno , Estudios Prospectivos , Respiración Artificial , Pruebas de Función Respiratoria , Factores de Tiempo
15.
SN Compr Clin Med ; 4(1): 57, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35194572

RESUMEN

The aim of the study was to evaluate the temporal evolution of fibrotic-like pulmonary interstitial abnormalities secondary to Sars-CoV-2 virus (COVID-19) pneumonia detected on chest-CTs of patients hospitalized for COVID-19 infection. We retrospectively reviewed chest-CTs obtained up to 9 months after disease onset in a group of patients with COVID-19 pneumonia and CT features suggestive of lung fibrosis at the first follow-up after hospital discharge. We observed a complete and unexpected resolution of all interstitial abnormalities, including reticulations and bronchial dilatation, in a period of about 6-9 months after discharge. Interstitial fibrotic-like changes detectable in the first months after COVID-19 pneumonia could be slowly or very slowly resolving but still completely reversible and probably secondary to an organizing pneumonia reaction.

17.
BMC Pulm Med ; 21(1): 396, 2021 Dec 04.
Artículo en Inglés | MEDLINE | ID: mdl-34863146

RESUMEN

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is characterized by a poor prognosis, with a progressive decline in lung function and considerable variability in the disease's natural history. Besides lung transplantation (LTx), the only available treatments are anti-fibrosing drugs, which have shown to slow down the disease course. Therefore, predicting the prognosis is of pivotal importance to avoid treatment delays, which may be fatal for patients with a high risk of progression. Previous studies showed that a multi-dimensional approach is practical and effective in the development of a reliable prognostic score for IPF. In the RIsk Stratification scorE (RISE), physiological parameters, an objective measure of patient-reported dyspnea and exercise capacity are combined to capture different domains of the complex pathophysiology of IPF. METHODS: This is an observational, multi-centre, prospective cohort study, designed to reflect common clinical practice in IPF. A development cohort and a validation cohort will be included. Patients newly diagnosed with IPF based on the ATS/ERS criteria and multi-disciplinary discussion will be included in the study. A panel of chest radiologists and lung pathologists will further assess eligibility. At the first visit (time of diagnosis), and every 4-months, MRC dyspnea score, pulmonary function tests (FEV1, FVC and DLCO), and 6-min walking distance will be recorded. Patients will be prospectively followed for 3 years. Comorbidities will be considered. The radiographic extent of fibrosis on HRCT will be recalculated at a 2-year interval. RISE, Gender-Age-Physiology, CPI and Mortality Risk Scoring System will be calculated at 4-month intervals. Longitudinal changes of each variable considered will be assessed. The primary endpoint is 3-year LTx-free survival from the time of diagnosis. Secondary endpoints include several, clinically-relevant information to ensure reproducibility of results across a wide range of disease severity and in concomitance of associated pulmonary hypertension or emphysema. DISCUSSION: The objective of this study is to validate RISE as a simple, straightforward, inexpensive and reproducible tool to guide clinical decision making in IPF, and potentially as an endpoint for future clinical trials. TRIAL REGISTRATION: U.S National Library of Medicine Clinicaltrials.gov, trial n. NCT02632123 "Validation of the risk stratification score in idiopathic pulmonary fibrosis". Date of registration: December 16th, 2015.


Asunto(s)
Toma de Decisiones Clínicas/métodos , Fibrosis Pulmonar Idiopática , Medición de Riesgo , Canadá/epidemiología , Humanos , Fibrosis Pulmonar Idiopática/epidemiología , Londres/epidemiología , Desarrollo de Programa , Estudios Prospectivos , Reproducibilidad de los Resultados , Medición de Riesgo/métodos , Medición de Riesgo/normas , Ciudad de Roma/epidemiología
18.
Respiration ; 100(10): 989-999, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34044410

RESUMEN

BACKGROUND: Over the last 2 decades, great progress has been made in the understanding of the clinical aspects and pathogenesis of lymphangioleiomyomatosis (LAM), leading to publication of guidelines and approval of an effective therapy. OBJECTIVES: Aim of our study was to describe how the management and the natural history of this rare disease have changed after the publication of the ERS and American Thoracic Society/Japanese Respiratory Society guidelines and the introduction of sirolimus. METHODS: We examined 162 LAM patients followed at our center between 2001 and 2017, reporting clinical characteristics and diagnostic approach. Response to sirolimus in patients undergoing long-term treatment and mortality risk, estimated in terms of cumulative incidence taking into account organ transplantation as a competing cause of the event, were evaluated. The difference in the cumulative incidence between the patients admitted to the observation before 2011 and after 2011, year of the publication of the MILES trial for the efficacy of sirolimus, has also been estimated. RESULTS: Sixty-one patients had a histological diagnosis (22 from 2010 onward). 101 patients received a radiological diagnosis according to the guidelines criteria. Pulmonary function tests remained stable over a 3-year treatment period in patients who received sirolimus for over 12 months. The cumulative incidence of mortality after 10 years in the whole population was 25.5%. The cumulative incidence of mortality after 5 years was significantly lower in patients who entered the study since 2011 (after publication of the MILES trial) than in patients who entered the study before. CONCLUSIONS: We provide the data supporting the long-term efficacy of sirolimus therapy in a large cohort of patients with functional impairment and other manifestations of the disease. Our results also suggest that the advent of sirolimus and the publication of international guidelines changed the natural history of the disease lowering the mortality and reducing the need of invasive diagnostic techniques.


Asunto(s)
Neoplasias Pulmonares , Linfangioleiomiomatosis , Estudios de Cohortes , Humanos , Neoplasias Pulmonares/tratamiento farmacológico , Linfangioleiomiomatosis/diagnóstico , Linfangioleiomiomatosis/tratamiento farmacológico , Pruebas de Función Respiratoria , Sirolimus/efectos adversos , Sirolimus/uso terapéutico
19.
Diagn Interv Radiol ; 27(3): 329-335, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-34003121

RESUMEN

PURPOSE: Mediastinal lymph node (MLN) enlargement detected on chest computed tomography (CT) is frequent in patients with interstitial lung disease (ILD) and is shown in approximately 70% of cases of idiopathic pulmonary fibrosis (IPF). We hypothesized that enlarged MLNs might be a predictor of poor prognosis, associated with lower survival and stronger disease severity. METHODS: This study included patients with idiopathic pulmonary fibrosis (IPF) or nonspecific interstitial pneumonia (NSIP) from January 2009 to December 2018. Baseline chest CT scan and one-year follow-up scan of the patients were reviewed for the extent of lung fibrosis and MLNs. Two radiologists independently assessed MLN diameter and location. Patients with drug toxicity-related ILD, sarcoidosis, chronic hypersensitivity pneumonitis and other rare idiopathic interstitial pneumonias were excluded. The primary endpoint was survival. Secondary endpoints included number of hospitalizations for respiratory causes, lung function evaluated by forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO), and lung fibrosis score determined by CT scan. RESULTS: We retrospectively reviewed the medical registries of 110 patients with chronic pulmonary fibrosis (mean age 71 years, 60.4% male). Nine participants were excluded because the CT scans were of poor diagnostic quality for the evaluation of the mediastinum or unavailable for review. The analysis of 101 patients showed that enlarged MLNs (short axis diameter ≥10 mm) were present in 50.5% (n=51) and strongly predicted survival (HR= 2.11, 95% CI 1.12-3.96, p = 0.020). Patients with MLN enlargement experienced greater number of hospitalizations for respiratory causes (mean 2.5 vs. 1.8, p = 0.010) and had significantly worse lung function parameters (FVC, 71% vs. 81%, p = 0.018 and DLCO, 40% vs. 50%, p = 0.001) and a higher lung fibrosis score (50% vs. 39%, p = 0.001). CONCLUSION: In patients with IPF and NSIP, enlarged MLNs predict survival, are associated with increased number of hospitalizations, and show signs of poorer lung function and more severe fibrosis.


Asunto(s)
Fibrosis Pulmonar Idiopática , Enfermedades Pulmonares Intersticiales , Anciano , Femenino , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Pulmón/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Ganglios Linfáticos/diagnóstico por imagen , Masculino , Pronóstico , Estudios Retrospectivos
20.
Emerg Radiol ; 28(3): 507-518, 2021 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-33646498

RESUMEN

Coronavirus disease 2019 (COVID-19) emerged in early December 2019 in China, as an acute lower respiratory tract infection and spread rapidly worldwide being declared a pandemic in March 2020. Chest-computed tomography (CT) has been utilized in different clinical settings of COVID-19 patients; however, COVID-19 imaging appearance is highly variable and nonspecific. Indeed, many pulmonary infections and non-infectious diseases can show similar CT findings and mimic COVID-19 pneumonia. In this review, we discuss clinical conditions that share a similar imaging appearance with COVID-19 pneumonia, in order to identify imaging and clinical characteristics useful in the differential diagnosis.


Asunto(s)
Neumonía/diagnóstico por imagen , Radiografía Torácica , Tomografía Computarizada por Rayos X , COVID-19/diagnóstico por imagen , Diagnóstico Diferencial , Humanos , Pandemias , SARS-CoV-2
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA