RÉSUMÉ
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)
Sujet(s)
Humains , Mâle , Femelle , Fibrose pulmonaire/épidémiologie , Maladie de Raynaud/diagnostic , Sclérodermie systémique/complications , Facteurs de risque de maladie cardiaque , Épidémiologie Descriptive , Études transversalesRÉSUMÉ
Background: Infection by SARS-CoV-2 has been associated with multiple symptoms; however, still, little is known about persistent symptoms and their probable association with the risk of developing pulmonary fibrosis in patients post-COVID-19. Methods: A longitudinal prospective study on health workers infected by SARS-CoV-2 was conducted. In this work, signs and symptoms were recorded of 149 health workers with a positive PCR test for SARS-CoV-2 at the beginning of the diagnosis, during the active infection, and during post-COVID-19 follow-up. The McNemar chi-square test was used to compare the proportions and percentages of symptoms between the baseline and each follow-up period. Results: The signs and symptoms after follow-up were cardiorespiratory, neurological, and inflammatory. Gastrointestinal symptoms were unusual at the disease onset, but unexpectedly, their frequency was higher in the post-infection stage. The multivariate analysis showed that pneumonia (HR 2.4, IC95%: 1.5−3.8, p < 0.001) and positive PCR tests still after four weeks (HR 5.3, IC95%: 2.3-12.3, p < 0.001) were factors associated with the diagnosis of post-COVID-19 pulmonary fibrosis in this study group. Conclusions: Our results showed that pneumonia and virus infection persistence were risk factors for developing pulmonary fibrosis post-COVID-19, after months of initial infection.
Sujet(s)
COVID-19 , Fibrose pulmonaire , COVID-19/complications , Humains , Patients en consultation externe , Études prospectives , Fibrose pulmonaire/épidémiologie , SARS-CoV-2RÉSUMÉ
OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.
Sujet(s)
Oxygénation extracorporelle sur oxygénateur à membrane/méthodes , Maladies pulmonaires/thérapie , Transplantation pulmonaire/méthodes , Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Brésil/épidémiologie , Dilatation des bronches/épidémiologie , Dilatation des bronches/thérapie , Femelle , Humains , Unités de soins intensifs , Durée du séjour , Maladies pulmonaires/épidémiologie , Mâle , Adulte d'âge moyen , Complications postopératoires , Études prospectives , Hypertension artérielle pulmonaire/épidémiologie , Hypertension artérielle pulmonaire/thérapie , Emphysème pulmonaire/épidémiologie , Emphysème pulmonaire/thérapie , Fibrose pulmonaire/épidémiologie , Fibrose pulmonaire/thérapie , Études rétrospectives , Facteurs temps , Résultat thérapeutiqueRÉSUMÉ
Hermansky-Pudlak syndrome (HPS) is a multisystemic autosomal recessive disorder characterized by oculocutaneous albinism, bleeding diathesis, and lethal pulmonary fibrosis (PF) in some HPS subtypes. During middle adulthood, ground-glass opacities, reticulation, and traction bronchiectasis develop with progression of PF. HPS is an orphan disease occurring in 1 in 500,000 to 1,000,000 individuals worldwide, though the prevalence is 1 in 1,800 in individuals with Puerto Rican heritage. Recessive mutations or disruptions in HPS genes alter the function of HPS proteins which are components of biogenesis of lysosome-related organelle complexes and are critical for intracellular protein trafficking. Diagnosis and management of HPS-related comorbidities represent a challenge to physicians, and a multidisciplinary clinical approach is necessary for early detection, health management, and surveillance of PF in patients with HPS types 1, 2, and 4. Treatment options for individuals with HPS-PF include pirfenidone and lung transplantation. In this article, we describe the epidemiology, genetics, clinical manifestations, and management of HPS.
Sujet(s)
Syndrome d'Hermanski-Pudlak/diagnostic , Syndrome d'Hermanski-Pudlak/thérapie , Évolution de la maladie , Syndrome d'Hermanski-Pudlak/épidémiologie , Syndrome d'Hermanski-Pudlak/génétique , Humains , Mutation , Porto Rico/ethnologie , Fibrose pulmonaire/épidémiologie , Fibrose pulmonaire/étiologieRÉSUMÉ
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) causes progressive dyspnea, hypoxemia, and death within a few years. Little is known about the effect of air pollution on disease exacerbations. RESEARCH QUESTION: Are acute increases in air pollution a risk factor for hospitalization of patients with a primary diagnosis of IPF. STUDY DESIGN AND METHODS: Hospital admissions for IPF are coded J84.1 by the International Classification of Disease, 10th Revision. Using ambient air pollution and climate data from seven air monitoring stations distributed in the seven urban centers in Santiago, Chile, along with daily patient hospitalization data from 2001 to 2012, a linear association between daily ambient air pollution and daily J84.1 hospital admissions was tested using generalized linear models. RESULTS: Average pollutant levels for all regions were as follows: carbon monoxide was 0.96 ppm, ozone was 64 ppb, nitrogen dioxide (NO2) was 43 ppb, sulfur dioxide was 9 ppb, particulate matter < 2.5 µm in diameter was 29 µg/m3 and particulate matter < 10 µm in diameter (PM10) was 67 µg/m3. For the combined Santiago area, relative risk estimates of J84.1 hospitalizations for all pollutants (except ozone), adjusted for age, sex, and weather were statistically significant. In the two-pollutant models, the significance of NO2 and PM10 persisted despite adjustments for each of the other measured pollutants. INTERPRETATION: Our findings suggest that acute increases in air pollution are a risk factor for hospitalization of patients with a primary diagnosis of IPF.
Sujet(s)
Pollution de l'air/effets indésirables , Hospitalisation/statistiques et données numériques , Fibrose pulmonaire/épidémiologie , Sujet âgé , Sujet âgé de 80 ans ou plus , Chili/épidémiologie , Femelle , Humains , Mâle , Adulte d'âge moyen , Facteurs de risque , Aggravation transitoire des symptômesRÉSUMÉ
OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.
Sujet(s)
Oxygénation extracorporelle sur oxygénateur à membrane/méthodes , Maladies pulmonaires/thérapie , Complications postopératoires , Emphysème pulmonaire/thérapie , Emphysème pulmonaire/épidémiologie , Fibrose pulmonaire/thérapie , Fibrose pulmonaire/épidémiologie , Facteurs temps , Brésil/épidémiologie , Dilatation des bronches/thérapie , Dilatation des bronches/épidémiologie , Études prospectives , Études rétrospectives , Transplantation pulmonaire/méthodes , Résultat thérapeutique , Hypertension artérielle pulmonaire primitive familiale/thérapie , Hypertension artérielle pulmonaire primitive familiale/épidémiologie , Unités de soins intensifs , Durée du séjour , Maladies pulmonaires/épidémiologieRÉSUMÉ
OBJECTIVE: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. METHODS: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. RESULTS: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. CONCLUSIONS: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.
Sujet(s)
Pneumopathies interstitielles/anatomopathologie , Fibrose pulmonaire/anatomopathologie , Adulte , Répartition par âge , Âge de début , Sujet âgé , Sujet âgé de 80 ans ou plus , Biopsie , Brésil/épidémiologie , Femelle , Humains , Pneumopathies interstitielles/imagerie diagnostique , Pneumopathies interstitielles/épidémiologie , Mâle , Adulte d'âge moyen , Fibrose pulmonaire/imagerie diagnostique , Fibrose pulmonaire/épidémiologie , Tests de la fonction respiratoire , Répartition par sexe , TomodensitométrieRÉSUMÉ
ABSTRACT Objective: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. Methods: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. Results: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. Conclusions: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.
RESUMO Objetivo: Descrever as características clínicas, funcionais e radiológicas de um grupo de casos índice diagnosticados com fibrose pulmonar familiar (FPF) no Brasil. Métodos: Trinta e cinco pacientes com FPF (18 mulheres; 51,4%), com mediana de idade de 66,0 anos (variação: 35,5-89,3 anos), responderam a um questionário padronizado e foram submetidos a testes de função pulmonar e TCAR de tórax. Tecido pulmonar foi obtido para revisão em 6 casos: a partir de biópsias cirúrgicas em 5 e de autópsia em 1. Resultados: Antecedentes de tabagismo e de exposição a aves ou mofo foram referidos por 45,7% e 80,0% dos casos, respectivamente. Tosse e dispneia significante foram referidas por 62,8% e 48,6% dos pacientes, respectivamente. Estertores finos foram detectados em 91,4% dos indivíduos. Em 4 pacientes, os achados levantaram suspeitas de doença dos telômeros. As medianas da CVF e da DLCO foram, respectivamente, de 64,9% (variação: 48,8-105,7%) e 38,9% (variação: 16,0-60,0%) em porcentagem dos valores previstos. Apesar de espirometria normal, 9 pacientes exibiram DLCO reduzida. Em relação às TCAR, padrões típicos de pneumonia intersticial usual foram encontrados em 6 pacientes (17,1%). Em 25 casos (71,5%) os achados tomográficos foram mais consistentes com um diagnóstico de não relacionado a fibrose pulmonar idiopática. Em 11 pacientes (31,4%) o padrão radiológico foi incaracterístico para doença pulmonar intersticial. Das seis amostras de tecido pulmonar analisadas, quatro mostraram pneumonias intersticiais com acentuação bronquiolocêntrica e, em função de outros dados clínicos e radiológicos, pneumonite de hipersensibilidade foi diagnosticada. Conclusões: Pacientes com FPF podem apresentar características clínicas diversas. A maioria das TCAR desses pacientes exibe padrões não típicos de pneumonia intersticial usual. A pesquisa da história clínica de outros casos de pneumopatias fibrosantes na família deve ser feita em todos os pacientes em investigação, independentemente da idade.
Sujet(s)
Humains , Mâle , Femelle , Adulte , Adulte d'âge moyen , Sujet âgé , Sujet âgé de 80 ans ou plus , Fibrose pulmonaire/anatomopathologie , Pneumopathies interstitielles/anatomopathologie , Fibrose pulmonaire/épidémiologie , Fibrose pulmonaire/imagerie diagnostique , Tests de la fonction respiratoire , Biopsie , Brésil/épidémiologie , Tomodensitométrie , Répartition par sexe , Pneumopathies interstitielles/épidémiologie , Pneumopathies interstitielles/imagerie diagnostique , Âge de début , Répartition par âgeRÉSUMÉ
Idiopathic pulmonary fibrosis (IPF) is a lethal lung disorder of unknown etiology. The disease is likely the result of complex interactions between genetic and environmental factors. Evidence suggests that certain environmental factors, such as cigarette smoking and metal dust exposures, or comorbidities like gastroesophageal reflux, and type 2 diabetes mellitus (DM2) may increase risk to develop IPF. Substantial uncertainty remains, however, regarding these and other putative risk factors for IPF. In this study we performed a case-control analysis including 100 patients with IPF and 263 controls matched for age sex and place of residence. We used a structured questionnaire to identify potential risk factors for IPF, including environmental and occupational exposures as well as the relevance of family history of pulmonary fibrosis. The multivariate analysis revealed that family history of pulmonary fibrosis [OR = 6.1, CI95% 2.3-15.9; p < 0.0001] was strongly associated with increased risk of IPF. Actually, 20% of the cases reported a parent or sibling with pulmonary fibrosis. Gastroesophageal reflux [OR = 2.9, CI: 1.3-6.6; p = 0.007], former cigarette smoking [OR = 2.5, CI: 1.4-4.6, p = 0.003], and past or current occupational exposure to dusts, smokes, gases or chemicals [OR = 2.8, CI: 1.5-5.5; p = 0.002] were also associated with the disease. Despite being a significant risk factor on univariate analysis DM2 was not significant in multivariate analysis. These findings indicate that family history of pulmonary fibrosis is a strong risk factor for IPF. Also, we confirmed that occupational exposures, gastroesophageal reflux and former smoking increase the risk for this disease.
Sujet(s)
Fibrose pulmonaire idiopathique/étiologie , Métaux/effets indésirables , Exposition professionnelle/effets indésirables , Fibrose pulmonaire/complications , Fumer/effets indésirables , Sujet âgé , Études cas-témoins , Comorbidité , Poussière , Femelle , Reflux gastro-oesophagien/complications , Reflux gastro-oesophagien/épidémiologie , Humains , Fibrose pulmonaire idiopathique/épidémiologie , Fibrose pulmonaire idiopathique/physiopathologie , Mâle , Analyse multifactorielle , Fibrose pulmonaire/épidémiologie , Fibrose pulmonaire/physiopathologie , Facteurs de risque , Enquêtes et questionnairesRÉSUMÉ
UNLABELLED: Infections are an important cause of morbidity and mortality among transplanted patients. Their pathophysiology is associated with anatomic factors, immunosuppression, and pretransplant viral exposure. The aim of this investigation was to characterize infections following lung transplantation. We retrospectively analyzed the charts of 51 lung transplant recipients, who were transplanted between 1999 and 2008. Infections were classified according to their origin, etiology, occurrence time, and risk factors. The patient mean age was 55 years (range 13-71), 65% were male, and pulmonary fibrosis was the lung disease etiology in 59% of cases. Seventy-one episodes of infection were reported in the 51 patients, including (75%) during the first year after transplantation and 30 within the first 3 months (42%). Between the 4th and 11th months the number of infections decreased to 23 (32%), and afterwards there were 18 additional cases. The original site of infection was pulmonary in 43 episodes (60%), and the etiology was bacterial in 34 (48%), with Pseudomonas in 12 instances (35% of bacterial infections). Viruses were involved in 25 episodes, especially cytomegalovirus (CMV) in seronegative patients. The nine infections of fungal etiology (13%) were all caused by Aspergillus and always associated with either an acute rejection episode or suture damage. Three cases of tuberculosis were diagnosed, including two in the late post-transplant period. Three patients died of early infections. CONCLUSIONS: The critical period for infections in lung transplantation patients is the first 3 months, especially for those of bacterial etiology. CMV diseases were more common in seronegative patients and fungal infections in airway injury cases.
Sujet(s)
Infections/épidémiologie , Transplantation pulmonaire/effets indésirables , Complications postopératoires/épidémiologie , Maladies de l'appareil respiratoire/épidémiologie , Adolescent , Adulte , Sujet âgé , Infections bactériennes/classification , Infections bactériennes/épidémiologie , Infections bactériennes/mortalité , Mucoviscidose/épidémiologie , Femelle , Humains , Transplantation pulmonaire/mortalité , Mâle , Adulte d'âge moyen , Complications postopératoires/microbiologie , Complications postopératoires/virologie , Fibrose pulmonaire/épidémiologie , Maladies de l'appareil respiratoire/classification , Études rétrospectives , Facteurs de risque , Facteurs temps , Maladies virales/épidémiologieRÉSUMÉ
Idiopathic pulmonary fibrosis is a progressive and usually fatal lung disease characterized by fibroblast proliferation and extracellular matrix remodeling, which result in irreversible distortion of the lung's architecture. Although the pathogenetic mechanisms remain to be determined, the prevailing hypothesis holds that fibrosis is preceded and provoked by a chronic inflammatory process that injures the lung and modulates lung fibrogenesis, leading to the end-stage fibrotic scar. However, there is little evidence that inflammation is prominent in early disease, and it is unclear whether inflammation is relevant to the development of the fibrotic process. Evidence suggests that inflammation does not play a pivotal role. Inflammation is not a prominent histopathologic finding, and epithelial injury in the absence of ongoing inflammation is sufficient to stimulate the development of fibrosis. In addition, the inflammatory response to a lung fibrogenic insult is not necessarily related to the fibrotic response. Clinical measurements of inflammation fail to correlate with stage or outcome, and potent anti-inflammatory therapy does not improve outcome. This review presents a growing body of evidence suggesting that idiopathic pulmonary fibrosis involves abnormal wound healing in response to multiple, microscopic sites of ongoing alveolar epithelial injury and activation associated with the formation of patchy fibroblast-myofibroblast foci, which evolve to fibrosis. Progress in understanding the fibrogenic mechanisms in the lung is likely to yield more effective therapies.
Sujet(s)
Fibrose pulmonaire/étiologie , Fibrose pulmonaire/thérapie , Anti-inflammatoires/usage thérapeutique , Évolution de la maladie , Épithélium/anatomopathologie , Femelle , Humains , Inflammation/anatomopathologie , Mâle , Fibrose pulmonaire/épidémiologie , Fibrose pulmonaire/anatomopathologie , Cicatrisation de plaie/physiologieRÉSUMÉ
To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortality rates.
Sujet(s)
Fibrose pulmonaire/diagnostic , Adolescent , Biopsie , Enfant , Enfant d'âge préscolaire , Maladie chronique , Colorado/épidémiologie , Études de suivi , Humains , Nourrisson , Poumon/imagerie diagnostique , Poumon/anatomopathologie , Prévalence , Fibrose pulmonaire/épidémiologie , Fibrose pulmonaire/thérapie , Radiographie , Tests de la fonction respiratoire , Études rétrospectives , Résultat thérapeutiqueRÉSUMÉ
Erythrocytosis, a known response to chronic hypoxemia, is considered infrequent in interstitial lung diseases. We studied the prevalence of high hematocrit (Hct) values and the relationship between Hct and SaO2 in 79 patients with chronic pigeon breeder's lung (PBL) and 34 with idiopathic pulmonary fibrosis (IPF), all of whom lived in the Mexico City metropolitan area (2,240 m above sea level). Lung biopsy was performed in 31 patients with IPF and 71 with PBL. We analyzed only one simultaneous measurement of Hct and SaO2 per patient (usually the initial measurement) before treatment. No additional cause for anemia or erythrocytosis was detected. Forty-eight percent of the patients with PBL (38/79) and 62 percent of those with IPF (21/34) had high Hct values (greater than 2 SD above mean values for Mexico City); in 14 (12.3 percent) of the 113 patients (nine with PBL and five with IPF), the Hct was above 60 percent. The Hct and SaO2 values displayed a poor correlation for the whole group: Hct = 65.7-0.16(SaO2), r = 0.24, p = 0.012. The correlation between Hct and SaO2 was nonsignificant if patients were separated by diagnosis. For an SaO2 of less than 80 percent, the slope of SaO2 vs Hct was zero. Half of our patients with PBL and IPF had Hct values that were high for the altitude. In most cases, Hct responses fell within the confidence limits reported as normal at high altitudes. We found a poor relationship between Hct and awake SaO2.
Sujet(s)
Hématocrite/statistiques et données numériques , Fibrose pulmonaire/sang , Population urbaine/statistiques et données numériques , Altitude , Biopsie , Maladie des éleveurs d'oiseaux/sang , Maladie des éleveurs d'oiseaux/épidémiologie , Maladie des éleveurs d'oiseaux/anatomopathologie , Maladie chronique , Humains , Poumon/anatomopathologie , Mexique/épidémiologie , Oxygène/sang , Prévalence , Études prospectives , Fibrose pulmonaire/épidémiologie , Fibrose pulmonaire/anatomopathologieRÉSUMÉ
A review of the relevant literature provides evidence that cigarette smoking causes microscopic diffuse interstitial pulmonary fibrosis in humans at autopsy. The association shows a dose-response relationship and the fibrosis is often severe. Experimental animal studies have confirmed these observations when the exposure to inhaled smoke was prolonged or heavy. Six published and one unpublished cross-sectional chest x-ray surveys of adults have shown low profusion of small irregular opacities related to smoking with a dose-response relationship when this has been studied. Although information on radiographic-pathologic correlation is deficient, these studies support the hypothesis that smoking causes diffuse interstitial pulmonary fibrosis which is radiologically visible with low profusion in low prevalence. The x-ray manifestations may be confused with early pneumoconiosis such as asbestosis.
Sujet(s)
Fibrose pulmonaire/étiologie , Fumer/effets indésirables , Facteurs âges , Animaux , Humains , Fibrose pulmonaire/imagerie diagnostique , Fibrose pulmonaire/épidémiologie , RadiographieSujet(s)
Fibrose pulmonaire , Adolescent , Adulte , Sujet âgé , Enfant , Femelle , Humains , Mâle , Mexique , Adulte d'âge moyen , Fibrose pulmonaire/épidémiologieSujet(s)
Nicotiana , Végétaux toxiques , Fibrose pulmonaire/épidémiologie , Fumer , Adulte , Sujet âgé , Poids , Dyspnée/étiologie , Femelle , Guyana , Défaillance cardiaque/étiologie , Humains , Mâle , Adulte d'âge moyen , Fibrose pulmonaire/imagerie diagnostique , Fibrose pulmonaire/étiologie , Coeur pulmonaire/étiologie , Radiographie , Tests de la fonction respiratoireRÉSUMÉ
The clinical features and epidemiology of diffuse pulmonary fibrosis in Guyana have been investigated. The condition is limited to East Indians and is characterised by progressive dyspnoea leading to pulmonary heart-disease and congestive heart-failure. 19 hospital patients studied, and 7 others radiographically confirmed, had all smoked "blackfat" tobacco, a variety which is not widely used.(Summary)