RESUMEN
Idiopathic inflammatory myopathies, especially antisynthetase syndrome, often appear outside of the muscles as interstitial lung disease (ILD). Another typical finding is the presence of mechanic's hands. The aim of the present study was to describe the clinical, functional, tomographic, and serological data of patients with ILD and mechanic's hands and their response to treatment and survival rates. This is a retrospective study of ILD with concurrent myopathy. Among the 119 patients initially selected, 51 had mechanic's hands. All the patients were screened for anti-Jo-1 antibodies. An expanded panel of myopathy autoantibodies was also performed in 27 individuals. Of the 51 patients, 35 had 1 or more antibodies. The most common were anti-Jo-1, anti-PL-7, and anti-PL-12, while of the associated antibodies, anti-Ro52 was present in 70% of the 27 tested individuals. A significant response to treatment was characterized by an increase in predicted forced vital capacity (FVC) of at least 5% in the last evaluation done after 6 to 24 months of treatment. A decrease in predicted FVC of at least 5%, the need for oxygen therapy, or death were all considered treatment failures. All patients were treated with corticosteroids, and 71% with mycophenolate. After 24 months, 18 patients had an increase in FVC, 11 had a decrease, and 22 remained stable. After a median follow-up of 58 months, 48 patients remained alive and three died. Patients with honeycombing on high-resolution chest tomography (log-rankâ =â 34.65; Pâ <â .001) and a decrease in FVCâ ≥5% (log-rankâ =â 18.28, Pâ <â .001) had a poorer survival rate. Patients with ILD and mechanic's hands respond well to immunosuppressive treatment.
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Enfermedades Pulmonares Intersticiales , Miositis , Humanos , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/terapia , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/fisiopatología , Estudios Retrospectivos , Masculino , Femenino , Persona de Mediana Edad , Miositis/terapia , Miositis/mortalidad , Miositis/tratamiento farmacológico , Miositis/complicaciones , Anciano , Resultado del Tratamiento , Adulto , Autoanticuerpos/sangre , Pacientes Ambulatorios/estadística & datos numéricos , Corticoesteroides/uso terapéutico , Capacidad VitalRESUMEN
BACKGROUND: Continuous monitoring of pulse oximetry (SpO2 ) is recommended during the 6-min walk test (6MWT) to ensure that the lowest SpO2 is recorded. In this case, severe exercise-induced desaturation (EID; SpO2 < 80%) triggers walking interruption by the examiner. Our main objective was to assess the impact of this approach on 6MWT distance in patients with chronic respiratory diseases and, second, to evaluate the safety of the test without interruption due to severe EID. METHODS: 6MWTs with continuous monitoring of SpO2 were prospectively performed in subjects with chronic respiratory disease. The participants were randomly allocated to walk with or without SpO2 real-time assessment. SpO2 visualization during the test execution was available only in the first group, and walking interruption was requested by the examiner if SpO2 < 80%. RESULTS: One hundred forty-five participants were included in each group (68.6% females, 62 [52-69] y old) without differences in demographic and resting lung function parameters between them. The main respiratory conditions were COPD (n = 101), asthma (n = 73), pulmonary hypertension (n = 47), and interstitial lung disease (n = 39). The walked distance was similar comparing groups (349.5 ± 117.5 m vs 351.2 ± 105.4 m). Twenty-five subjects presented with severe EID in the group with real-time SpO2 assessment, and 20 subjects had severe EID in the group without real-time assessment respectively (overall prevalence of 15.5%). The 23 participants who had their test interrupted by the examiner due to severe EID in the first group (2 subjects stopped by themselves due to excessive symptoms) walked a shorter distance compared to the 11 subjects with severe EID without test interruption in the second group (9 subjects stopped by themselves due to excessive symptoms): 240.6 ± 100.2 m versus 345.9 ± 73.4 m. No exercise-related serious adverse events were observed. CONCLUSIONS: Interruption driven by severe EID reduced the walked distance during the 6MWT. No serious adverse event, in turn, was observed in subjects with severe desaturation without real-time SpO2 assessment.
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Oximetría , Enfermedad Pulmonar Obstructiva Crónica , Prueba de Paso , Humanos , Oximetría/métodos , Femenino , Masculino , Prueba de Paso/métodos , Persona de Mediana Edad , Anciano , Estudios Prospectivos , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Caminata/fisiología , Asma/fisiopatología , Enfermedad Crónica , Hipertensión Pulmonar/fisiopatología , Enfermedades Pulmonares Intersticiales/fisiopatología , Saturación de Oxígeno/fisiologíaRESUMEN
OBJECTIVES: To investigate muscle strength and the prevalence of muscle weakness in adults with interstitial lung diseases (ILDs) compared to healthy subjects. DESIGN: Cross-sectional (description of clinical features). SETTING: Public referral center (University Hospital). PARTICIPANTS: One hundred and twelve adults with ILD (n=48, 60±10yr, 68% female) and healthy counterparts (control group, n=64, 57±10yr, 58% female) (N=112). INTERVENTION: Not applicable. MAIN OUTCOME MEASURE(S): Muscle strength and prevalence of muscle weakness in adults with ILD. Muscle strength was assessed via maximal isometric voluntary contraction of dominant upper and lower limb muscle groups. Data from the control group were used to generate reference equations. Muscle weakness was defined as a muscle strength value below the lower limit of normal calculated using data from the control group. Data were expressed as mean ± SD or median [interquartile range] according to the data distribution. RESULTS: Compared to the control group, adults with ILD had lower muscle strength for all muscle groups assessed (values presented as %predicted: pectoralis major 75[57-86]%; quadriceps 72[58-87]%; latissimus dorsi 76[57-103]%; deltoid 74[64-98]%; biceps brachii 78[64-91]%; triceps brachii 84[62-101]%; P≤.001 for all). Prevalence of muscle weakness in people with ILD was 40% for pectoralis major, 25% for latissimus dorsi, 16% for triceps brachii, 20% for biceps brachii, 27% for deltoid and 46% for quadriceps. CONCLUSIONS: Adults with ILD present a generalised reduction in peripheral Muscle strength, ranging between 20% to 46% of people depending on the muscle group assessed. and it was more prevalent in lower limb muscles.
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Enfermedades Pulmonares Intersticiales , Fuerza Muscular , Debilidad Muscular , Humanos , Femenino , Masculino , Enfermedades Pulmonares Intersticiales/fisiopatología , Estudios Transversales , Persona de Mediana Edad , Fuerza Muscular/fisiología , Debilidad Muscular/fisiopatología , Anciano , Contracción Isométrica/fisiología , Músculo Esquelético/fisiopatología , Estudios de Casos y Controles , PrevalenciaRESUMEN
ANTECEDENTES: La prueba de capacidad de difusión de monóxido de carbono (DLCO) es una evaluación de función pulmonar rutinaria y no invasiva clínicamente útil para determinar el estado de la función pulmonar en pacientes con trastornos crónicos como la enfermedad pulmonar intersticial difusa (EPID). OBJETIVO: Describir el perfil sociodemográfico y clínico de usuarios de la prueba DLCO en Valdivia, Chile. Materiales y Métodos: Estudio observacional, retrospectivo, de base documental. A partir de registros de 490 pacientes que se realizaron la prueba DLCO entre 2017 y 2019, se describen características sociodemográficas, clínicas y reporte de consumo de cigarrillo como cigarrillos/d e índice paquetes/año (IPA), comparando por sexo. RESULTADOS: La mayor proporción de evaluados fueron mujeres (61%), con una edad mediana de 65 años, mayor en mujeres (66 vs 64 años; p = 0,0361). La mayoría consultaron por EPID (54,5%). Según estado nutricional, 38% presentó preobesidad y 24,7% obesidad I (24,7%), destacando que 40,5% de las mujeres y 33% de los hombres se encontraron en alguna categoría de obesidad. Entre quienes reportaron información de consumo de cigarrillos (n = 346, 70,6%), 14,7% (n = 51) eran consumidores actuales, con un consumo mediano de 10 cigarrillos/d, sin diferencias por sexo. Entre exfumadores (n = 144; 50% hombres/mujeres) hubo significativamente menor consumo diario (5 vs 15; p = 0,0300) y de IPA (7 vs 18; p = 0,0083) en mujeres. CONCLUSIONES: En usuarios de DLCO el principal diagnóstico de consulta fue EPID. Destacó alta frecuencia de obesidad y tabaquismo, sin diferencia de consumo por sexo en fumadores actuales, pero sí en exfumadores.
BACKGROUND: The carbon monoxide diffusion capacity test (DLCO) is a clinically useful, routine, non-invasive lung function assessment to determine the status of lung function in patients with chronic disorders such as interstitial lung disease (ILD). AIM: To describe the sociodemographic and clinical profile of users of the DLCO test in Valdivia, Chile. MATERIALS AND METHODS: Observational, retrospective, documentary-based study. From the records of 490 patients who underwent the DLCO test between 2017 and 2019, sociodemographic and clinical characteristics and reports of cigarette consumption are described, such as cigarettes/d and pack-year index (PYI), comparing by sex. Results: The highest proportion of those evaluated were women (61%), with a median age of 65 years, higher in women (66 vs. 64 years; p = 0.0361). The majority consulted for ILD (54.5%). According to nutritional status, 38% presented pre-obesity and 24.7% obesity I (24.7%), highlighting that 40.5% of women and 33% of men were in some category of obesity. Among those who reported information on cigarette consumption (n = 346, 70.6%), 14.7% (n = 51) were current consumers, with a median consumption of 10 cigarettes/d, without differences by sex. Among exsmokers (n = 144; 50% men/women) there was significantly less daily (5 vs 15; p = 0.0300) and IPA (7 vs 18; p = 0.0083) consumption in women. CONCLUSIONS: In DLCO users, the main consultation diagnosis was ILD. High frequency of obesity and smoking stood out, with no difference in consumption by sex in current smokers, but yes in ex-smokers.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano de 80 o más Años , Monóxido de Carbono/análisis , Capacidad de Difusión Pulmonar/fisiología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Pruebas de Función Respiratoria , Factores Socioeconómicos , Fumar/epidemiología , Chile , Factores Sexuales , Estudios RetrospectivosRESUMEN
BACKGROUND: The carbon monoxide diffusion capacity test (DLCO) is a clinically useful, routine, non-invasive lung function assessment to determine the status of lung function in patients with chronic disorders such as interstitial lung disease (ILD). AIM: To describe the sociodemographic and clinical profile of users of the DLCO test in Valdivia, Chile. MATERIALS AND METHODS: Observational, retrospective, documentary-based study. From the records of 490 patients who underwent the DLCO test between 2017 and 2019, sociodemographic and clinical characteristics and reports of cigarette consumption are described, such as cigarettes/d and pack-year index (PYI), comparing by sex. RESULTS: The highest proportion of those evaluated were women (61%), with a median age of 65 years, higher in women (66 vs. 64 years; p = 0.0361). The majority consulted for ILD (54.5%). According to nutritional status, 38% presented pre-obesity and 24.7% obesity I (24.7%), highlighting that 40.5% of women and 33% of men were in some category of obesity. Among those who reported information on cigarette consumption (n = 346, 70.6%), 14.7% (n = 51) were current consumers, with a median consumption of 10 cigarettes/d, without differences by sex. Among exsmokers (n = 144; 50% men/women) there was significantly less daily (5 vs 15; p = 0.0300) and IPA (7 vs 18; p = 0.0083) consumption in women. CONCLUSIONS: In DLCO users, the main consultation diagnosis was ILD. High frequency of obesity and smoking stood out, with no difference in consumption by sex in current smokers, but yes in ex-smokers.
Asunto(s)
Monóxido de Carbono , Enfermedades Pulmonares Intersticiales , Capacidad de Difusión Pulmonar , Humanos , Masculino , Femenino , Chile , Persona de Mediana Edad , Estudios Retrospectivos , Anciano , Monóxido de Carbono/análisis , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Capacidad de Difusión Pulmonar/fisiología , Adulto , Fumar/epidemiología , Pruebas de Función Respiratoria , Factores Sexuales , Factores SocioeconómicosRESUMEN
Las enfermedades restrictivas comprenden un grupo heterogéneo de trastornos que se caracterizan por una alteración en la distensibilidad pulmonar, generada por enfermedades del parénquima o intersticio pulmonar o por problemas extrapulmonares (alteraciones de la caja torácica o enfermedades neuromusculares). Presentan un patrón característico en las pruebas de función pulmonar, relación VEF1/ FVC normal o aumentada con CVF disminuida, VEF1 disminuido leve o normal y capacidad pulmonar total disminuida (CPT). Su manejo es complejo debido a la dificultad para establecer el diagnóstico diferencial, por lo que se recomienda una derivación precoz a un especialista en enfermedades respiratorias y el enfrentamiento por un equipo multidisciplinario.
Restrictive diseases comprise a heterogeneous group of disorders characterized by an alteration in lung compliance, generated by diseases of the lung parenchyma or interstitium, as well as by extrapulmonary problems (abnormalities of the rib cage or neuromuscular diseases). They present a characteristic pattern in pulmonary function tests, with decreased FVC (forced vital capacity), slightly decreased or normal FEV1 (forced expiratory volume in 1 second), normal or increased FEV1/FVC ratio, and decreased total lung capacity. Its management is complex due to the difficulty in establishing the differential diagnosis, so early referral to a specialist in respiratory diseases and confrontation by a multidisciplinary team is recommended.
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Humanos , Niño , Enfermedades Pulmonares Intersticiales/fisiopatología , Pruebas de Función Respiratoria , Rendimiento Pulmonar , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/terapiaRESUMEN
Lung diseases have high mortality and morbidity, with an important impact on quality of life. Hypoxemic patients are advised to use oxygen therapy to prolong their survival, but high oxygen saturation (SpO2) levels can also have negative effects. Pulse oximeters are the most common way to assess oxygen levels and guide medical treatment. This study aims to assess whether wearable devices can provide precise SpO2 measurements when compared to commercial pulse oximeters. This is a cross-section study with 100 patients with chronic obstructive pulmonary disease and interstitial lung disease from an outpatient pneumology clinic. SpO2 and heart rate data were collected with an Apple Watch Series 6 (Apple) and compared to two commercial pulse oximeters. The Bland-Altman method and interclass correlation coefficient were used to compare their values. We observed strong positive correlations between the Apple Watch device and commercial oximeters when evaluating heart rate measurements (r = 0.995, p < 0.001) and oximetry measurements (r = 0.81, p < 0.001). There was no statistical difference in the evaluation of skin color, wrist circumference, presence of wrist hair, and enamel nail for SpO2 and heart rate measurements in Apple Watch or commercial oximeter devices (p > 0.05). Apple Watch 6 is a reliable way to obtain heart rate and SpO2 in patients with lung diseases in a controlled environment.
Asunto(s)
Enfermedades Pulmonares Intersticiales/diagnóstico , Oximetría/instrumentación , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Dispositivos Electrónicos Vestibles , Anciano , Estudios Transversales , Femenino , Voluntarios Sanos , Frecuencia Cardíaca/fisiología , Humanos , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Persona de Mediana Edad , Saturación de Oxígeno/fisiología , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Reproducibilidad de los Resultados , MuñecaRESUMEN
BACKGROUND: Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. METHODS: A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. RESULTS: Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20- < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. CONCLUSIONS: Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. TRIAL REGISTRATION: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.
Asunto(s)
Hemodinámica , Hipertensión Pulmonar/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Hipertensión Arterial Pulmonar/diagnóstico , Esclerodermia Sistémica/fisiopatología , Adulto , Brasil , Cateterismo Cardíaco , Femenino , Estudios de Seguimiento , Humanos , Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar/fisiopatología , Enfermedades Pulmonares Intersticiales/complicaciones , Masculino , Persona de Mediana Edad , Pronóstico , Hipertensión Arterial Pulmonar/complicaciones , Hipertensión Arterial Pulmonar/fisiopatología , Estudios Retrospectivos , Esclerodermia Sistémica/complicaciones , Resistencia VascularRESUMEN
This study aimed to investigate the walking economy and possible factors influencing self-selected walking speed (SSWS) in patients with fibrotic interstitial lung disease (ILD) compared to controls. In this study, 10 patients with ILD (mean age: 63.8 ± 9.2 years, forced expiratory volume in the first second: 56 ± 7% of predicted) and 10 healthy controls underwent resting pulmonary function tests, cardiopulmonary exercise, and submaximal treadmill walking tests at different speeds. The walking economy was assessed by calculating the cost-of-transport (CoT). Dynamic stability was assessed by stride-to-stride fluctuations using video recordings. Patients with ILD showed reduced peak oxygen uptake with a tachypneic breathing pattern and significant oxygen desaturation during exercise. The CoT did not differ between the groups (p = 0.680), but dyspnea and SpO2 were higher and lower, respectively, in patients with ILD at the same relative speeds. SSWS was reduced in ILD patients (2.6 ± 0.9 vs. 4.2 ± 0.4 km h-1 p = 0.001) and did not correspond to the energetically optimal walking speed. Dynamic stability was significantly lower in patients with ILD than in healthy controls, mainly at lower speeds. Patients with ILD presented a similar cost of transport compared to healthy controls; however, they chose lower SSWS despite higher walking energy expenditure. Although walking stability and dyspnea were negatively affected, these factors were not associated with the slower walking speed chosen by individuals with ILD.
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Disnea/fisiopatología , Metabolismo Energético/fisiología , Enfermedades Pulmonares Intersticiales/complicaciones , Velocidad al Caminar/fisiología , Anciano , Estudios de Casos y Controles , Disnea/etiología , Prueba de Esfuerzo , Voluntarios Sanos , Humanos , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Persona de Mediana Edad , Consumo de Oxígeno/fisiología , Pruebas de Función RespiratoriaRESUMEN
BACKGROUND: There is limited information regarding impairment in functional performance tests and their measurement properties in ILD. The present study aimed to verify the impairment and measurement properties of functional performance tests in ILD. METHODS: ILD and healthy individuals underwent assessments of pulmonary function, peripheral muscle strength (handgrip force and maximum isometric contraction of quadriceps femoris - MIVCq) and exercise capacity (6-min walk test - 6MWT). Functional performance was assessed by timed-up-and-go with usual (TUGu) and fast (TUGf) gait speeds, 4-m gait speed (4MGS), sit-to-stand in 30 s (30sec-STS), 1 min (1min-STS) and with 5 repetition (5rep-STS) and Short Physical Performance Battery (SPPB). Functional performance was compared between groups, validity (correlation with 6MWT and MIVCq) and reliability of tests were checked in subjects with ILD (intra- and inter-rater agreement analysis). RESULTS: Seventy-six participants (40 ILD [25 women, 61 ± 11 years, FVC 75 ± 17 %pred] and 36 healthy [22 women, 61 ± 9 years, FVC 97 ± 11 %pred]) were included. Functional performance in ILD was worse than in healthy individuals in all tests, except for the 30sec-STS (p = 0.13). Pre-specified validity criteria were reached for TUGu, TUGf, 4MGS and 5rep-STS (-0.69 < r < 0.55; p < 0.05 for all). Except for 4MGS and SPPB, all tests showed good to excellent inter-rater (0.85 < ICC<0.93; p < 0.05 for all) and all tests showed good to excellent intra-rater (0.83 < ICC< 0.94; p < 0.05 for all) reliability. CONCLUSIONS: Subjects with ILD present worse functional performance than healthy individuals. According to reliability and validity results, TUGu, TUGf and 5rep-STS seem to be the most appropriate tests to evaluate functional performance in ILD.
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Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Pruebas de Función Respiratoria/métodos , Anciano , Femenino , Marcha , Voluntarios Sanos , Humanos , Masculino , Persona de Mediana Edad , Fuerza Muscular , Reproducibilidad de los Resultados , Prueba de PasoRESUMEN
BACKGROUND: In the past 20 years, hematopoietic stem cell transplantation (HSCT) has been investigated as treatment for systemic sclerosis (SSc). The goal of HSCT is to eradicate the autoreactive immune system, which is replaced by a new immune repertoire with long-lasting regulation and tolerance to autoantigens. Here, we describe the clinical outcomes of severe and refractory SSc patients that underwent HSCT at a single Brazilian center. PATIENTS AND METHODS: This is a longitudinal and retrospective study, including 70 adult SSc patients, with an established diagnosis of SSc, and who underwent autologous HSCT from 2009 to 2016. The procedure included harvesting and cryopreservation of autologous hematopoietic progenitor cells, followed by administration of an immunoablative regimen and subsequent infusion of the previously collected cells. Patients were evaluated immediately before transplantation, at 6 months and then yearly until at least 5-years of post-transplantation follow-up. At each evaluation time point, patients underwent clinical examination, including modified Rodnan's skin score (mRSS) assessment, echocardiography, high-resolution computed tomography of the lungs and pulmonary function. RESULTS: Median (range) age was 35.9 (19-59), with 57 (81.4%) female and median (range) non-Raynaud's disease duration of 2 (1-7) years. Before transplantation, 96% of the patients had diffuse skin involvement, 84.2%, interstitial lung disease and 67%, positive anti-topoisomerase I antibodies. Skin involvement significantly improved, with a decline in mRSS at all post-transplantation time points until at least 5-years of follow-up. When patients with pre-HSCT interstitial lung disease were analyzed, there was an improvement in pulmonary function (forced vital capacity and diffusing capacity of lung for carbon monoxide) over the 5-year follow-up. Overall survival was 81% and progression-free survival was 70.5% at 8-years after HSCT. Three patients died due to transplant-related toxicity, 9 patients died over follow-up due to disease reactivation and one patient died due to thrombotic thrombocytopenic purpura. CONCLUSIONS: Autologous hematopoietic progenitor cell transplantation improves skin and interstitial lung involvement. These results are in line with the international experience and support HSCT as a viable therapeutic alternative for patients with severe and progressive systemic sclerosis.
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Trasplante de Células Madre Hematopoyéticas , Enfermedades Pulmonares Intersticiales/terapia , Esclerodermia Sistémica/terapia , Adulto , Brasil , Causas de Muerte , Femenino , Trasplante de Células Madre Hematopoyéticas/métodos , Trasplante de Células Madre Hematopoyéticas/mortalidad , Humanos , Estudios Longitudinales , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Esclerodermia Sistémica/inmunología , Esclerodermia Sistémica/mortalidad , Resultado del Tratamiento , Adulto JovenRESUMEN
INTRODUCTION: Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES: To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS: This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS: 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION: The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.
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Trastornos de la Motilidad Esofágica/fisiopatología , Esófago/fisiopatología , Enfermedades Pulmonares Intersticiales/fisiopatología , Manometría/métodos , Esclerodermia Sistémica/fisiopatología , Adulto , Anciano , Estudios Transversales , Ensayo de Inmunoadsorción Enzimática , Trastornos de la Motilidad Esofágica/complicaciones , Trastornos de la Motilidad Esofágica/diagnóstico por imagen , Esfínter Esofágico Inferior/patología , Esfínter Esofágico Inferior/fisiopatología , Esófago/diagnóstico por imagen , Esófago/patología , Femenino , Hemaglutinación , Humanos , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodosRESUMEN
BACKGROUND: Pulmonary hypertension (PH) adversely affects patient's exercise capacity in interstitial lung disease (ILD). The impact of pulmonary vascular and right ventricular (RV) dysfunction, however, has traditionally been believed to be mild and clinically relevant principally in advanced lung disease states. RESEARCH QUESTION: The aim of this study was to evaluate the relative contributions of pulmonary mechanics, pulmonary vascular function, and RV function to the ILD exercise limit. STUDY DESIGN AND METHODS: Forty-nine patients with ILD who underwent resting right heart catheterization followed by invasive exercise testing were evaluated. Patients with PH at rest (ILD + rPH) and with PH diagnosed exclusively during exercise (ILD + ePH) were contrasted with ILD patients without PH (ILD non-PH). RESULTS: Peak oxygen consumption was reduced in ILD + rPH (61 ± 10% predicted) and ILD + ePH (67 ± 13% predicted) compared with ILD non-PH (81 ± 16% predicted; P < .001 and P = .016, respectively). Each ILD hemodynamic phenotype presented distinct patterns of dynamic changes of pulmonary vascular compliance relative to pulmonary vascular resistance from rest to peak exercise. Peak RV stroke work index was increased in ILD + ePH (24.7 ± 8.2 g/m2 per beat) and ILD + rPH (30.9 ± 6.1 g/m2 per beat) compared with ILD non-PH (18.3 ± 6.4 g/m2 per beat; P = .020 and P = .014). Ventilatory reserve was reduced in ILD + rPH compared with the other groups at the anaerobic threshold, but it was similar between ILD + ePH and ILD non-PH at the anaerobic threshold (0.32 ± 0.13 vs 0.30 ± 0.11; P = .921) and at peak exercise (0.70 ± 0.17 vs 0.73 ± 0.24; P = .872). INTERPRETATION: ILD with resting and exercise PH is associated with increased exercise RV work, reduced pulmonary vascular reserve, and reduced peak oxygen consumption. The findings highlight the role of pulmonary vascular and RV burden to ILD exercise limit.
Asunto(s)
Tolerancia al Ejercicio/fisiología , Hipertensión Pulmonar/complicaciones , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/fisiopatología , Disfunción Ventricular Derecha/complicaciones , Adulto , Anciano , Estudios de Casos y Controles , Ejercicio Físico , Prueba de Esfuerzo , Femenino , Humanos , Hipertensión Pulmonar/fisiopatología , Masculino , Persona de Mediana Edad , Consumo de Oxígeno , Mecánica Respiratoria , Resistencia Vascular , Disfunción Ventricular Derecha/fisiopatologíaRESUMEN
SUMMARY INTRODUCTION Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.
RESUMO INTRODUÇÃO A esclerose sistêmica (ES) é uma doença autoimune que afeta vários órgãos de etiologia desconhecida, caracterizada por dano vascular e fibrose da pele e órgãos. Entre os órgãos envolvidos estão o esôfago e o pulmão. OBJETIVOS Relacionar o perfil das alterações na eletromanometria (ME), o perfil de acometimento da pele, a pneumopatia intersticial (PI) e os sintomas esofágicos em pacientes com ES. MÉTODO Trata-se de um estudo observacional, transversal, realizado no ambulatório de SSC do Hospital das Clínicas da Universidade Federal de Uberlândia. Após aprovação pelo Comitê de Ética e assinatura dos termos de consentimento, 50 pacientes foram inicialmente convidados, de 04/12/2014 a 25/06/2015. Eles foram submetidos às investigações usuais de acordo com o quadro clínico. A análise estatística foi descritiva em porcentagem, média e desvio padrão. O teste Qui-quadrado foi utilizado para avaliar a relação entre ME, tomografia de alta resolução e sintomas esofágicos. RESULTADOS 91,9% dos pacientes apresentaram alterações manométricas. 37,8% tinham envolvimento do corpo esofágico e do esfíncter esofágico inferior. 37,8% tinham IP. 24,3% apresentaram a forma difusa da ES. Não há associação entre alterações manométricas e manifestações clínicas (sintomas cutâneos, pulmonares e gastrointestinais). CONCLUSÃO O presente estudo confirma que as alterações da motilidade esofágica detectadas pela EM são frequentes em pacientes com SSC, mas podem não estar relacionadas ao envolvimento cutâneo, à de DPI ou às queixas gastrointestinais dos pacientes.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Anciano , Esclerodermia Sistémica/fisiopatología , Trastornos de la Motilidad Esofágica/fisiopatología , Enfermedades Pulmonares Intersticiales/fisiopatología , Esófago/fisiopatología , Manometría/métodos , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/diagnóstico por imagen , Ensayo de Inmunoadsorción Enzimática , Trastornos de la Motilidad Esofágica/complicaciones , Trastornos de la Motilidad Esofágica/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Estudios Transversales , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Esfínter Esofágico Inferior/fisiopatología , Esfínter Esofágico Inferior/patología , Esófago/patología , Esófago/diagnóstico por imagen , Hemaglutinación , Persona de Mediana EdadRESUMEN
Coronavirus disease 2019 (COVID-19) is an infection caused by SARS-CoV-2 that has caused an unprecedented pandemic with a high rate of morbidity and mortality worldwide. Although most cases are mild, there are a considerable number of patients who develop pneumonia or even acute respiratory distress syndrome (ARDS). After having recovered from the initial disease, many patients continue with various symptoms (fatigue, dry cough, fever, dyspnea, anosmia, and chest pain, among others.), which has led to consider the possible existence of "post-COVID-19 syndrome". Although the definition and validity of this syndrome are not clear yet, several studies report that individuals who have recovered from COVID-19 may have persistent symptoms, radiological abnormalities, and compromised respiratory function. Current evidence suggests that there is a large number of pulmonary sequelae after COVID-19 pneumonia (interstitial thickening, ground glass opacities, crazy paving pattern, and bronchiectasis, among others.). Likewise, it seems that pulmonary function tests (spirometry, DLCO, 6MWT, and measurement of maximum respiratory pressures), in addition to high-resolution computed axial tomographies (CAT scan), are useful for the assessment of these post-COVID-19 pulmonary sequelae. This review aims to describe the possible pulmonary sequelae after COVID-19 pneumonia, as well as to suggest diagnostic procedures for their correct assessment and follow-up; thus, allowing proper management by a multidisciplinary medical team.
COVID-19 es la enfermedad causada por el virus SARS-CoV-2, la cual ha ocasionado una pandemia sin precedentes, con gran cantidad de infectados y muertos en el mundo. Aunque la mayoría de los casos son leves, existe una cantidad considerable de pacientes que desarrollan neumonía o, incluso, síndrome de distrés respiratorio agudo (SDRA). Luego de recuperarse del cuadro inicial, muchos pacientes continúan con diversos síntomas (fatiga, tos seca, fiebre, disnea, anosmia, dolor torácico, entre otras), lo que ha llevado a considerar la posible existencia del "síndrome pos-COVID-19". Aunque la definición y validez de este síndrome aún no son claras, varios estudios reportan que los individuos recuperados de la COVID-19 pueden tener persistencia de síntomas, anormalidades radiológicas y compromiso en la función respiratoria. La evidencia actual sugiere que existe gran cantidad de secuelas pulmonares despues de una neumonía por COVID-19 (engrosamiento intersticial, infiltrado en vidrio esmerilado, patrón en empedrado, bronquiectasias, entre otras.). De igual forma, parece ser que las pruebas de función pulmonar (espirometría, prueba de difusión pulmonar de monóxido de carbono, prueba de caminata de seis minutos y la medición de las presiones respiratorias máximas), además de la tomografía axial computarizada de alta resolución, son útiles para evaluar las secuelas pulmonares pos-COVID-19. En esta revisión se pretende describir las posibles secuelas a nivel pulmonar posteriores a neumonía por COVID-19, así como sugerir procedimientos diagnósticos para su correcta evaluación y seguimiento, que permitan el manejo adecuado por parte de un equipo médico multidisciplinario.
Asunto(s)
COVID-19/complicaciones , Convalecencia , Enfermedades Pulmonares/etiología , Síndrome de Dificultad Respiratoria/etiología , Bronquiectasia/diagnóstico por imagen , Bronquiectasia/etiología , Bronquiectasia/fisiopatología , Progresión de la Enfermedad , Estudios de Seguimiento , Humanos , Hipoxia/sangre , Hipoxia/etiología , Hipoxia/fisiopatología , Enfermedades Pulmonares/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Trastornos Mentales/etiología , Trastornos Mentales/fisiopatología , Oxígeno/sangre , Embolia Pulmonar/diagnóstico por imagen , Embolia Pulmonar/etiología , Embolia Pulmonar/fisiopatología , Síndrome de Dificultad Respiratoria/fisiopatología , Pruebas de Función Respiratoria , Espirometría , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Fibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL). Respiratory muscle dysfunction is among the underlying mechanisms of dyspnea and exercise intolerance in FILD but may be difficult to diagnose. Using ultrasound, we compared diaphragmatic mobility and thickening in FILD cases and healthy controls and correlated these findings with dyspnea, exercise tolerance, HRQoL and lung function. METHODS: We measured diaphragmatic mobility and thickness during quiet (QB) and deep breathing (DB) and calculated thickening fraction (TF) in 30 FILD cases and 30 healthy controls. We correlated FILD cases' diaphragmatic findings with dyspnea, exercise tolerance (six-minute walk test), lung function and HRQoL (St. George's Respiratory Questionnaire). RESULTS: Diaphragmatic mobility was similar between groups during QB but was lower in FILD cases during DB when compared to healthy controls (3.99 cm vs 7.02 cm; p < 0.01). FILD cases showed higher diaphragm thickness during QB but TF was lower in FILD when compared to healthy controls (70% vs 188%, p < 0.01). During DB, diaphragmatic mobility and thickness correlated with lung function, exercise tolerance and HRQoL, but inversely correlated with dyspnea. Most FILD cases (70%) presented reduced TF, and these patients had higher dyspnea and exercise desaturation, lower HRQoL and lung function. CONCLUSION: Compared to healthy controls, FILD cases present with lower diaphragmatic mobility and thickening during DB that correlate to increased dyspnea, decreased exercise tolerance, worse HRQoL and worse lung function. FILD cases with reduced diaphragmatic thickening are more dyspneic and exercise-intolerant, have lower HRQoL and lung function.
Asunto(s)
Diafragma , Disnea , Enfermedades Pulmonares Intersticiales , Calidad de Vida , Pruebas de Función Respiratoria , Ultrasonografía , Brasil/epidemiología , Diafragma/diagnóstico por imagen , Diafragma/patología , Diafragma/fisiopatología , Disnea/etiología , Disnea/fisiopatología , Tolerancia al Ejercicio , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Enfermedades Pulmonares Intersticiales/psicología , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria/métodos , Pruebas de Función Respiratoria/estadística & datos numéricos , Ultrasonografía/métodos , Ultrasonografía/estadística & datos numéricos , Prueba de Paso/métodosRESUMEN
OBJECTIVE: To translate the King's Brief Interstitial Lung Disease (K-BILD) questionnaire to Portuguese and culturally adapt it for use in Brazil. The K-BILD quantifies the health status of patients with ILD. METHODS: The process involved the following steps: authorization from the author of the original (English-language) questionnaire; translation of the questionnaire to Portuguese by three translators, working independently; merging of the translations by a committee of specialists; back-translation of the questionnaire to English; revision and readjustment of the back-translation by the committee of specialists; evaluation by the original author; revision of the back-translation; cognitive debriefing (verification of the clarity and acceptability of the Portuguese-language version in the target population-i.e., patients with ILD); and finalization of the Portuguese-language version. RESULTS: In the cognitive debriefing step, 20 patients with ILD were interviewed. After the interviews, the clarity and acceptability index of each question was ≥ 0.8, which is considered acceptable. CONCLUSIONS: The Portuguese-language version of K-BILD appears to be easily administered to and understood by patients with ILD in Brazil. To our knowledge, this is the only instrument in Brazilian Portuguese that is designed to evaluate the impact that ILD has on the various aspects of the lives of those it affects.
Asunto(s)
Estado de Salud , Enfermedades Pulmonares Intersticiales/fisiopatología , Encuestas y Cuestionarios/normas , Traducciones , Adulto , Anciano , Brasil , Comparación Transcultural , Femenino , Humanos , Lenguaje , Masculino , Persona de Mediana Edad , Calidad de Vida , Reproducibilidad de los Resultados , TraducciónRESUMEN
Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=-0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 of these patients.
Asunto(s)
Tolerancia al Ejercicio/fisiología , Hipertensión Pulmonar/fisiopatología , Enfermedades Pulmonares Intersticiales/fisiopatología , Pulmón/fisiología , Pruebas de Función Respiratoria/métodos , Esclerodermia Sistémica/complicaciones , Adulto , Estudios de Casos y Controles , Femenino , Humanos , Hipertensión Pulmonar/etiología , Pulmón/fisiopatología , Enfermedades Pulmonares Intersticiales/etiología , Mediciones del Volumen Pulmonar/métodos , Persona de Mediana Edad , Ventilación Pulmonar , Enfermedad de Raynaud/complicaciones , Tomografía Computarizada por Rayos X/métodos , Capacidad Vital/fisiología , Prueba de Paso/métodosRESUMEN
Interstitial lung abnormalities (ILA) represent aging-associated bilateral interstitial abnormalities in nondependent areas of the lung. However, the aging mechanisms associated with ILA remain uncertain. α-Klotho is an anti-aging molecule that decreases progressively with age, and abnormally low circulating levels of this protein have been revealed in several chronic-degenerative diseases. In this study, we evaluated α-Klotho serum concentrations in individuals with ILA, and examined whether its levels were associated with pulmonary function decline. α-Klotho was measured by ELISA in 50 respiratory asymptomatic adults with ILA and 150 healthy individuals over 60 years. Compared with controls, ILA subjects were predominantly older males, and showed lower lung diffusing capacity (DLCO), higher desaturation after exercise, and higher concentrations of serum matrix metalloprotease-7 (6.24 ± 4.1 versus 4.3 ± 1.7 ng/ml; p = 0.002). No differences were found in serum concentrations of α-Klotho. However, lower levels of this protein in ILA significantly correlated with lower values of forced vital capacity (Rho = 0.39; p = 0.005), forced expiratory volume in one second (Rho = 0.39; p = 0.005), and DLCO (Rho = 0.29, p = 0.04). These findings suggest that decreased concentrations of α-Klotho may be a predictive biomarker of accelerated decline of lung function in individuals with ILA.
Asunto(s)
Glucuronidasa/sangre , Enfermedades Pulmonares Intersticiales/sangre , Anciano , Biomarcadores/sangre , Femenino , Humanos , Proteínas Klotho , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Metaloproteinasa 7 de la Matriz/sangre , Persona de Mediana EdadRESUMEN
BACKGROUND: Patients with systemic sclerosis (SSc) undergo chest radiographs and high-resolution computed tomography (HRCT) of the thorax both for interstitial lung disease (ILD) detection and for disease progression monitoring. OBJECTIVE: The aim of this study was to assess whether lung ultrasound (LUS) is a useful screening tool for ILD in patients with SSc in comparison with HRCT. METHODS: This was a longitudinal cohort study carried out from December 2015 to April 2016. An LUS was performed to examine B-lines in 67 consecutive patients who met the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for SSc and had a previous HRCT. B-lines were quantified and classified according to the score modified from Picano. Severity and extent of lung involvement on the HRCT were determined by means of the Warrick score. RESULTS: Twenty-nine patients had both abnormal HRCT (Warrick score >7) and abnormal LUS, 2 had a mild score (6-15 B-lines), and 27 had either moderate or severe scores (≥16 B-lines). Of the 38 patients with negative HRCT, 25 presented some degree of lung involvement on the LUS. Thus, LUS has a sensitivity of 100% and a specificity of 34%. Receiver operating characteristic curve analysis showed the analytic relation between the number of B-lines and the presence of ILD on the HRCT (area under the curve, 0.80; 95% confidence interval, 0.69-0.90). CONCLUSIONS: Lung ultrasound may be a method to detect abnormal lung findings in a noninvasive manner in patients with SSc. Because of its high sensitivity, a low score almost rules out the need for an HRCT.