RESUMO
BACKGROUND: Swallowing is a complex process that requires the coordination of muscles in the mouth, pharynx, larynx, and esophagus. Dysphagia occurs when a person has difficulty swallowing. In the case of subjects with respiratory diseases, the presence of oropharyngeal dysphagia potentially increases lung disease exacerbations, which can lead to a rapid decline in lung function. This study aimed to analyze the swallowing of patients with idiopathic pulmonary fibrosis (IPF). METHODS: Patients with IPF were evaluated using the Eating Assessment Tool (EAT-10), tongue pressure, the Timed Water Swallow Test (TWST), and the Test of Mastication and Swallowing Solids (TOMASS). The findings were related to dyspnea severity assessed by the modified Medical Research Counsil (mMRC) score; the nutritional status screened with Mini Nutritional Assessment (MNA) tool; and pulmonary function tests, specifically spirometry and measurement of the diffusing capacity for carbon monoxide (DLCO), the maximal inspiratory pressure (PImax), and the maximal expiratory pressure (PEmax). RESULTS: The sample consisted of 34 individuals with IPF. Those who exhibited swallowing modifications scored lower on the MNA than those who did not (9.6 ± 0.76 vs. 11.64 ± 0.41 points; mean difference 1.98 ± 0.81 points; p = 0.02). They also showed poorer lung function when considering the predicted force vital capacity (FVC; 81.5% ± 4.61% vs. 61.87% ± 8.48%; mean difference 19.63% ± 9.02%; p = 0.03). The speed of liquid swallowing was altered in 31of 34 of the evaluated subjects (91.1%). The number of liquid swallows correlated significantly with the forced expiratory volume in 1 s (FEV1)/FVC ratio (r = 0.3; p = 0.02). Solid eating and swallowing assessed with the TOMASS score correlated with lung function. The number of chewing cycles correlated negatively with PImax% predicted (r = -0.4; p = 0.0008) and PEmax% predicted (r = -0.3; p = 0.02). FVC% predicted correlated with increased solid swallowing time (r = -0.3; p = 0.02; power = 0.6). Swallowing solids was also impacted by dyspnea. CONCLUSION: Patients with mild-to-moderate IPF can present feeding adaptations, which can be related to the nutritional status, lung function, and the severity of dyspnea.
Assuntos
Transtornos de Deglutição , Deglutição , Fibrose Pulmonar Idiopática , Língua , Humanos , Masculino , Feminino , Idoso , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/complicações , Deglutição/fisiologia , Transtornos de Deglutição/fisiopatologia , Transtornos de Deglutição/etiologia , Pessoa de Meia-Idade , Língua/fisiopatologia , Testes de Função Respiratória , Pressão , Estado Nutricional , Pulmão/fisiopatologia , Dispneia/fisiopatologia , Dispneia/etiologia , Avaliação Nutricional , Idoso de 80 Anos ou maisRESUMO
INTRODUCTION: One of the major concerns with post-acute sequelae of COVID-19 (PASC) is the development of pulmonary fibrosis, for which no approved pharmacological treatment exists. Therefore, the primary aim of this open-label study was to evaluate the safety and the potential clinical efficacy of a prolonged-release pirfenidone formulation (PR-PFD) in patients having PASC-pulmonary fibrosis. METHODS: Patients with PASC-pulmonary fibrosis received PR-PFD 1800 mg/day (1200 mg in the morning after breakfast and 600 mg in the evening after dinner) for three months. Blood samples were taken to confirm the pharmacokinetics of PR-PFD, and adverse events (AEs) were evaluated monthly using a short questionnaire. Symptoms, dyspnea, and pulmonary function tests (spirometry, diffusing capacity for carbon monoxide, plethysmography, and 6-min walk test [6MWT]) were evaluated at baseline, and one and three months after having started the PR-PFD treatment. RESULTS: Seventy subjects with mild to moderate lung restriction were included. The most common AEs were diarrhea (23%), heartburn (23%), and headache (16%), for which no modifications in the drug study were needed. Two patients died within the first 30 days of enrolment, and three opted not to continue the study, events which were not associate with PR-PFD. Pulmonary function testing, 6MWT, dyspnea, symptoms, and CT scan significantly improved after three months of treatment with PR-PFD. CONCLUSION: In patients with PASC pulmonary fibrosis, three months' treatment with PR-PFD was safe and showed therapeutic efficacy. Still, it remains to be seen whether the pulmonary fibrotic process remains stable, becomes progressive or will improve.
Assuntos
COVID-19 , Fibrose Pulmonar Idiopática , Pneumonia , Humanos , COVID-19/complicações , Progressão da Doença , Dispneia/tratamento farmacológico , Dispneia/etiologia , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/diagnóstico , Fenótipo , Pneumonia/tratamento farmacológico , Piridonas/efeitos adversosRESUMO
Idiopathic pulmonary fibrosis (IPF) and gastroesophageal reflux disease (GERD) are undoubtedly related. Even though it is not clear yet which one is the primary disease, they certainly interact increasing each other's severity. Symptoms are unreliable to diagnose GERD in patients with IPF, and objective evaluation with pH monitoring and/or bronchoalveolar lavage analysis is mandatory. Pharmacological treatment with proton pump inhibitors (PPIs) may bring control of IPF in few patients, but PPIs do not control reflux but just change the pH of the gastric refluxate. Surgical therapy based on a fundoplication is safe and effective as it controls any type of reflux, independently from the pH of the gastric refluxate. In patients waiting for lung transplantation (if they can tolerate a laparoscopic operation under general anesthesia), a fundoplication before the operation might block the progression of IPF, while after transplantation it might prevent rejection by preventing the bronchiolitis obliterans syndrome.
Assuntos
Refluxo Gastroesofágico/complicações , Fibrose Pulmonar Idiopática/complicações , Bronquiolite Obliterante/prevenção & controle , Lavagem Broncoalveolar , Progressão da Doença , Monitoramento do pH Esofágico , Fundoplicatura/métodos , Refluxo Gastroesofágico/diagnóstico , Refluxo Gastroesofágico/tratamento farmacológico , Refluxo Gastroesofágico/cirurgia , Rejeição de Enxerto/prevenção & controle , Azia/tratamento farmacológico , Humanos , Fibrose Pulmonar Idiopática/cirurgia , Transplante de Pulmão , Inibidores da Bomba de Prótons/uso terapêutico , SíndromeRESUMO
OBJECTIVE: To investigate the reliability, internal consistency and validity of the Brazilian Portuguese version of the University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ) in patients with interstitial lung disease (ILD). METHODS: Patients with ILD completed the questionnaire at three different time points, one week apart, with the assistance of two independent assessors. Intra- and inter-rater reliability were analysed via the intraclass correlation coefficient (ICC). Internal consistency was assessed with the Cronbach's alpha coefficient. For the validity analysis, associations between variables were assessed with Spearman's or Pearson's correlation coefficient. RESULTS: Thirty patients with ILD (idiopathic pulmonary fibrosis, connective tissue disease-associated pulmonary fibrosis, sarcoidosis, asbestosis or non-specific interstitial pneumonia) were included (15 men; mean age, 59 ± 10 years; DLCO: 46 [33-64] % predicted). UCSD SOBQ scores showed excellent agreement and internal consistency in the intra-rater analysis (ICC: 0.93 [0.85-0.97]; Cronbach alpha: 0.95) and in the inter-rater analysis (ICC: 0.95 [0.89-0.97]; Cronbach alpha: 0.95), as well as correlating significantly with dyspnoea (as assessed by the Medical Research Council scale; r = 0.56); Medical Outcomes Study 36-item Short-Form Health Survey domains bodily pain, general health, vitality and physical functioning (-0.40 ≤ r ≤ -0.74); six-minute walk distance (r = -0.38); and quadriceps muscle strength (r = -0.41). CONCLUSIONS: The Brazilian Portuguese version of the UCSD SOBQ is valid, is reliable and has internal consistency in patients with ILD in Brazil.
Assuntos
Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Idoso , Brasil , Dispneia/diagnóstico , Dispneia/etiologia , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Masculino , Pessoa de Meia-Idade , Psicometria , Reprodutibilidade dos Testes , Inquéritos e QuestionáriosRESUMO
Background: Interstitial lung disease (ILD) corresponds to a heterogeneous group of pathologies that differ in etiology with common clinical and radiological manifestations. In Latin America and Mexico, reports are scarce and the need for studies to understand the scenario is emphasized. Objective: To analyze a multidimensional profile in patients with interstitial lung disease in Yucatan. Method: This is an observational, prospective, analytic, descriptive study including consecutive patients diagnosed with ILD over a 4-year period. Demographic and clinical data, lung function tests, chest imaging, serum immunological profile, and echocardiographic findings were recorded. Differences between subgroups were analyzed performing a one-way analysis of variance (ANOVA). Results: 110 patients were included. The median age was 60 years and women were most affected. The main cause of ILD was related with connective tissue diseases (CTD). A group subanalysis revealed that Idiopathic pulmonary fibrosis (IPF) was common in males with a history of smoking and an imaging pattern of usual interstitial pneumonia. Lung function tests showed a moderate-to-severe pulmonary restriction (FVC 55%p) and mild hypoxemia (PaO2 79mmHg). Positive antinuclear antibodies are less likely in cases with IPF (20 vs. 65%; p = 0.006). Conclusion: In Southeastern Mexico, ILD occurs in women in their seventh decade of life; the most common cause is related with CTD. Our results support that ILD has a heterogeneous expression and is relevant the need for subsequent studies characterizing each ILD.
Introducción: La enfermedad pulmonar intersticial (EPI) corresponde a un grupo heterogéneo de patologías que difieren en su etiología pero tienen manifestaciones clínicas y radiológicas comunes. En Latinoamérica y México los reportes son escasos, enfatizando la necesidad de estudios que permitan conocer su escenario clínico-epidemiológico. Objetivo: Realizar un análisis multidimensional y contrastado de la EPI en la población de la Península de Yucatán. Método: Estudio observacional, prospectivo, analítico y descriptivo, que incluye la totalidad de pacientes diagnosticados de EPI en un período de 4 años. Se registraron datos demográficos y clínicos, pruebas de función pulmonar, imagenología del tórax, perfil inmunológico y ecocardiografía transtorácica. Se analizaron las diferencias según la etiología mediante análisis de la varianza de una sola vía (ANOVA). Resultados: Se incluyeron 110 pacientes con una mediana de edad de 60 años y predominio del sexo femenino. La causa principal de la EPI fue relacionada con enfermedad del tejido conectivo. El subanálisis de grupos mostró que la fibrosis pulmonar idiopática (FPI) es frecuente en los varones con antecedente de tabaquismo y patrón tomográfico de neumonía intersticial usual. Las pruebas de función pulmonar demostraron restricción pulmonar moderadamente grave (FVC 55%p) e hipoxemia leve (PaO2 79 mmHg). La positividad de anticuerpos antinucleares ocurre en menor proporción en la FPI (20 vs. 65%, p = 0.006). Conclusiones: En el sureste de México, la EPI ocurre en mujeres de la séptima década de la vida y se relaciona con enfermedad del tejido conectivo. Nuestros resultados respaldan que la EPI tiene expresión heterogénea y se requieren estudios subsecuentes sobre cada tipo de enfermedad.
Assuntos
Doenças do Tecido Conjuntivo , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Doenças do Tecido Conjuntivo/complicações , Feminino , Humanos , Fibrose Pulmonar Idiopática/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/etiologia , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Estudos Prospectivos , Fumar , Tomografia Computadorizada por Raios XRESUMO
El patrón de neumonía intersticial usual (NIU) en la tomografía computada de alta resolución (TCAR) tiene un alto grado de correlación con el patrón histológico lo que permite obviar en dichos casos la necesidad de realizar biopsia pulmonar. La exactitud del diagnóstico de NIU en TCAR se basa en la detección de signos específicos de fibrosis como el panal y las densidades reticulares con bronquiolectasias por tracción. La actualización de las guías 2018 de práctica clínica de la ATS/ERS/JRS/ALAT de Fibrosis Pulmonar Idiopática (FPI) y la declaración de consenso de la Sociedad Fleischner proponen una nueva forma de clasificar los patrones tomográficos tomando en cuenta los aspectos antes considerados. Si bien la presencia de panal sigue siendo el hallazgo principal para caracterizar el patrón NIU, la distribución del reticulado intersticial y la presencia de bronquiolectasias por tracción en el contexto clínico especifico puede ser suficiente para realizar el diagnóstico de fibrosis pulmonar idiopática.
Usual interstitial pneumonia pattern (UIP) in high-resolution computed tomography (HRCT) has a high degree of correlation with the histological pattern which makes it possible to obviate in these cases the need for lung biopsy. The accuracy of a UIP diagnosis in HRCT is based on the detection of specific signs of fibrosis such as honeycomb and reticular densities with traction bronchiolectasis. The update of the 2018 Clinical Practice Guidelines of the ATS / ERS / JRS / ALAT of Idiopathic Pulmonary Fibrosis (IPF) and the Fleischner Society White Paper proposed a new way of classifying the tomographic patterns taking into account the aspects previously considered. Although the presence of honeycomb remains the main finding to characterize the UIP pattern, the distribution of interstitial reticulate and the presence of traction bronchiolectasis, in the specific clinical context, may be sufficient to make the diagnosis of idiopathic pulmonary fibrosis.
Assuntos
Humanos , Tomografia Computadorizada por Raios X/métodos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Bronquiectasia/etiologia , Bronquiectasia/diagnóstico por imagem , Fibrose Pulmonar Idiopática/complicaçõesRESUMO
La fibrosis pulmonar idiopática (FPI) se caracteriza por presentar una capacidad funcional reducida, disnea e hipoxia inducida por el ejercicio, lo que disminuye su tolerancia al esfuerzo y limita su capacidad de realizar actividades diarias. Las comorbilidades son frecuentes y su presencia contribuyen al empeoramiento de la calidad de vida y aumento de la mortalidad. Por lo anterior, es que además de las terapias antifibróticas, los pacientes con FPI se benefician de un enfoque integral de la atención que puede incluir: pesquisa, diagnóstico y tratamiento de comorbilidades, ingreso a protocolos de investigación, manejo sintomático, cuidados paliativos, oxígeno suplementario, rehabilitación pulmonar, educación y apoyo por un equipo multidisciplinario.
Idiopathic pulmonary fibrosis (IPF) is characterized by reduced functional capacity, dyspnea and exercise-induced hypoxia, which decreases tolerance to exertion and limits the ability to perform daily activities. Comorbidities are frequent and their presence contribute to worsening quality of life and increased mortality. Therefore, in addition to antifibrotic therapies, patients with IPF benefit of a comprehensive approach to care that may include: screening, diagnosis and treatment of comorbidities, admission to research protocols, symptomatic management, palliative care, supplementary oxygen, pulmonary rehabilitation, education and support by a multidisciplinary team.
Assuntos
Humanos , Fibrose Pulmonar Idiopática/terapia , Fibrose Pulmonar Idiopática/epidemiologia , Oxigenoterapia , Equipe de Assistência ao Paciente , Comorbidade , Quimioterapia Adjuvante , Tosse/etiologia , Dispneia/etiologia , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/tratamento farmacológicoRESUMO
Pulmonary fibrosis is a progressive disease. Lung transplantation is the only effective therapy for a group of patients. Objective: To evaluate results of lung transplantation for fibrosis up to a 5 years of follow up. Methodology: Retrospective review of clinical records of patients subjected to lung transplantation from Clínica Las Condes and Instituto Nacional del Tórax. Demographic data, type of transplant, baseline and post-transplant spirometry and 6 min walked distance (6MWT), early and late complications and long-term survival rate were analyzed. Results: From 1999 to 2015, 87patients with pulmonary fibrosis were transplanted, in average they were 56 years old, 56% were male, 89% of patients were subjected to a single lung transplant. 16% of them were in urgency. Baseline and 1-3-5 years for FVC were 49-73-83 and 78% of the reference values and for 6MWT were 280, 485, 531 and 468 meters respectively. Predominant complications < 1 year post-transplant were: acute rejection (30%) and infections (42%). Complications after 1 year of transplantation were chronic graft dysfunction (DCI) 57% and neoplasms (15%). The main causes of mortality > 1 year were DCI (45%) and neoplasms (11%). The estimated 1, 3 and 5 year survival rate were 84, 71 and 58% respectively. Conclusions: Lung transplantation in patients with pulmonary fibrosis improves their quality of life and survival rate. The monopulmonary technique is efficient in the long term. Acute rejection and infection were the most common early complications and chronic graft dysfunction was the prevalent long-term complication.
La Fibrosis pulmonar es una enfermedad progresiva y el trasplante constituye una terapia efectiva para un grupo de pacientes. Objetivo: Evaluar los resultados del trasplante pulmonar por fibrosis a 5 años. Metodología: Revisión retrospectiva de registros de trasplante pulmonar de la Clínica Las Condes e Instituto Nacional del Tórax. Se analizaron datos demográficos, tipo de trasplante, función pulmonar basal y post-trasplante, complicaciones precoces y tardías y sobrevida a largo plazo. Resultados: Entre 1999 y 2015 ambos centros trasplantaron 87 pacientes por fibrosis pulmonar. Los pacientes tenían una edad promedio de 56 años, 56% eran del género masculino y se usó técnica monopulmonar en 89% de ellos. 16% de los pacientes se encontraba en urgencia. Los resultados espirométricos y la distancia caminada en 6 minutos (T6 min) basales y a 1- 3 - 5 años fueron: CVF 49- 73- 83 y 78% del valor teórico y T6 min fue 280, 485, 531 y 468 metros respectivamente. Complicaciones predominantes < 1 año fueron: rechazo agudo 30% e infecciones 42%. Complicaciones > 1 año fueron: disfunción crónica del injerto (DCI) 57% y neoplasias 15%. Las causas de mortalidad > 1 año fueron DCI 45% y neoplasias 11%. La sobrevida estimada a uno, 3 y 5 años fue 84, 71 y 58% respectivamente. Conclusiones: El trasplante en pacientes con fibrosis pulmonar, permite mejorar la calidad de vida y sobrevida de estos pacientes. La técnica monopulmonar es eficiente a largo plazo. En las complicaciones precoces predominaron el rechazo agudo e infecciones y a largo plazo la DCI.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Transplante de Pulmão/métodos , Fibrose Pulmonar Idiopática/cirurgia , Fibrose Pulmonar Idiopática/complicações , Espirometria , Exercício Físico , Oxigenação por Membrana Extracorpórea , Análise de Sobrevida , Capacidade de Difusão Pulmonar , Estudos Retrospectivos , Caminhada , Dados Estatísticos , Fibrose Pulmonar Idiopática/diagnósticoRESUMO
The mucosal metastasis of adenocarcinomas located in colonic mucosa is not infrequent. We present a clinical report of a patient diagnosed with a gastric multifocal signet ring cell adenocarcinoma without any evidence of visceral dissemination with the exception of mucosal infiltration of signet ring cell adenocarcinoma in a colonic polyp and in the mucosa of previous colonic anastomosis. The histopathological study of suspect lesions in the colonic mucosa is necessary to correctly approach the treatment of these patients.
Assuntos
Anastomose Cirúrgica , Carcinoma de Células em Anel de Sinete/secundário , Pólipos do Colo/patologia , Neoplasias Colorretais/secundário , Segunda Neoplasia Primária/patologia , Neoplasias Gástricas/patologia , Adenocarcinoma/patologia , Adenocarcinoma/cirurgia , Idoso de 80 Anos ou mais , Carcinoma de Células em Anel de Sinete/complicações , Neoplasias Colorretais/complicações , Diabetes Mellitus Tipo 2/complicações , Humanos , Hipertensão Pulmonar/complicações , Fibrose Pulmonar Idiopática/complicações , Falência Renal Crônica/complicações , Masculino , Isquemia Miocárdica/complicações , Embolia Pulmonar/complicações , Neoplasias do Colo Sigmoide/patologia , Neoplasias do Colo Sigmoide/cirurgia , Neoplasias Gástricas/complicaçõesRESUMO
OBJETIVO: Determinar a prevalência da doença do refluxo gastroesofágico (DRGE) e sua apresentação clínica, além do perfil funcional do esôfago em pacientes com fibrose pulmonar idiopática (FPI). MÉTODOS: Foram avaliados prospectivamente 28 pacientes com FPI. Os pacientes foram submetidos à esofagomanometria estacionária, pHmetria de 24 h e testes de função pulmonar, assim como responderam a questionários sobre sintomas e qualidade de vida em DRGE. RESULTADOS: A prevalência de DRGE foi de 35,7 por cento. Os pacientes foram divididos em dois grupos: grupo DRGE+ (pHmetria anormal; n = 10), e grupo DRGE- (pHmetria normal; n = 18). No grupo DRGE+, 77,7 por cento dos pacientes apresentavam pelo menos um sintoma típico de DRGE. Nesse grupo de pacientes, 8 (80 por cento) apresentaram refluxo em posição supina, e 5 (50 por cento) apresentaram refluxo exclusivamente nessa posição. Nos grupos DRGE+ e DRGE-, respectivamente, 5 (50,0 por cento) e 7 (38,8 por cento) dos pacientes apresentaram hipotonia do esfíncter esofágico inferior, assim como 7 (70,0 por cento) e 10 (55,5 por cento) apresentaram hipomotilidade esofágica. Não houve diferenças significativas entre os grupos quanto a características demográficas, função pulmonar, apresentação clínica ou achados manométricos. CONCLUSÕES: A prevalência de DRGE nos pacientes com
OBJECTIVE: To determine the prevalence of gastroesophageal reflux disease (GERD) and to evaluate its clinical presentation, as well as the esophageal function profile in patients with idiopathic pulmonary fibrosis (IPF). METHODS: In this prospective study, 28 consecutive patients with IPF underwent stationary esophageal manometry, 24-h esophageal pH-metry and pulmonary function tests. All patients also completed a symptom and quality of life in GERD questionnaire. RESULTS: In the study sample, the prevalence of GERD was 35.7 percent. The patients were then divided into two groups: GERD+ (abnormal pH-metry; n = 10) and GERD- (normal pH-metry; n = 18). In the GERD+ group, 77.7 percent of the patients presented at least one typical GERD symptom. The pH-metry results showed that 8 (80 percent) of the GERD+ group patients had abnormal supine reflux, and that the reflux was exclusively in the supine position in 5 (50 percent). In the GERD+ and GERD- groups, respectively, 5 (50.0 percent) and 7 (38.8 percent) of the patients presented a hypotensive lower esophageal sphincter, 7 (70.0 percent) and 10 (55.5 percent), respectively, presenting lower esophageal dysmotility. There were no significant differences between the groups regarding demographic characteristics, pulmonary function, clinical presentation or manometric findings. CONCLUSIONS: The prevalence of GERD in the patients with IPF was high. However, the clinical and functional characteristics did not differ between the patients with GERD and those without.