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1.
Rev. am. med. respir ; 19(2): 109-111, jun. 2019. ilus
Artigo em Espanhol | LILACS | ID: biblio-1041686

RESUMO

Las enfermedades del intersticio pulmonar (ILD's) comprenden más de 100 afecciones,­algunas de causas conocidas y otras no­, que comparten un cuadro clínico, radiográfico y funcional. Muchas de ellas afectan además la luz y la pared bronquiolar como sucede por ejemplo en la Neumonía Crónica Organizada (COP) y en la Bronquiolitis Respiratoria asociada a Intersticiopatía (BR-ILD)


Assuntos
Fibrose Pulmonar , Enfisema
2.
Rev. am. med. respir ; 19(2): 159-161, jun. 2019. ilus
Artigo em Espanhol | LILACS | ID: biblio-1041695

RESUMO

Caso clínico: Paciente de sexo masculino, de 72 años. Ingresa por un cuadro de disnea súbita mMRC 2-3, con progresión a disnea mMRC 4. Refiere tos crónica sin cambio en sus características. Niega fiebre. Antecedentes: Ex tabaquista (36 paquetes/año), con antecedentes de artritis reumatoidea, y fibrosis pulmonar. Examen físico: Saturación de oxígeno 88% aire ambiente, taquipneico. Con hipoventilación y crepitantes velcro hasta el tercio medio, bilaterales. Exámenes complementarios: Hemograma normal. La gasometría arterial revela alcalosis respiratoria compensada. La espirometría y los volúmenes pulmonares se encuentran dentro de los límites de referencia, se observa disminución severa de la capacidad de difusión pulmonar (DLCO). La tomografía computarizada torácica de alta resolución (TCAR) (Figuras 1 y 2) muestra enfisema en los lóbulos superiores, con fibrosis de predominio en lóbulos inferiores, no se observan signos indirectos de hipertensión pulmonar


Assuntos
Artrite Reumatoide , Fibrose Pulmonar , Enfisema
3.
J. bras. pneumol ; 45(5): e20180079, 2019. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1012575

RESUMO

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.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Fibrose Pulmonar/patologia , Doenças Pulmonares Intersticiais/patologia , Fibrose Pulmonar/epidemiologia , Fibrose Pulmonar/diagnóstico por imagem , Testes de Função Respiratória , Biópsia , Brasil/epidemiologia , Tomografia Computadorizada por Raios X , Distribuição por Sexo , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Idade de Início , Distribuição por Idade
4.
Pesqui. vet. bras ; 38(11): 2056-2064, Nov. 2018. tab, ilus
Artigo em Inglês | LILACS, VETINDEX | ID: biblio-976400

RESUMO

Respiratory diseases cause significant veterinary costs, reduce performance and require withdrawal of horses. Yet, studies of the causes of pneumonia in horses are scant. This study aimed to describe the pathological and microbiological features of lung lesions in slaughtered horses in southern Brazil. In this study, 84 samples of lungs were examined, and a conclusive diagnosis was obtained in 74 cases. These were composed of bronchopneumonia in 50 cases, followed by granulomatous eosinophilic pneumonia (9/74), recurrent airway obstruction (7/74), lung fibrosis (4/74), lung hemorrhage (3/74) and pulmonary pythiosis (1/74). Bronchopneumonia had grossly firm focally extensive yellow to dark-red areas, which consisted microscopically of multifocal to coalescing infiltrate of degenerate neutrophils. Streptococcus equi subsp. zooepidemicus was identified in 21 of the 50 cases. Granulomatous eosinophilic pneumonia had multifocal pinpoint firm-hard yellow areas, which microscopically were composed of granulomas with a mineralized center surrounded by collagen fibers and severe infiltrate of eosinophils. Recurrent airway obstruction had mild multifocal pinpoint firm white areas that consisted microscopically of large amounts of mucus inside bronchi and bronchiole. Lung fibrosis had two patterns: focally extensive areas of consolidation and firm nodular areas. Microscopically, the first pattern had interstitial to peribronchial fibrosis, while the second had, in addition to the interstitial fibrosis, a severe pneumocyte hyperplasia and an alveolar infiltrate of neutrophils and macrophages with rare intranuclear inclusion bodies (equine herpesvirus 5, EHV-5). Pulmonary pythiosis presented a focal firm nodular area, with multiple kunkers observed in the cut surface, which corresponded microscopically to areas of necrosis surrounded by a mixed inflammatory infiltrate. At the periphery of the necrotic areas, multiple negatively stained hyphae were observed, which were evidenced through Grocott's stain and immunohistochemistry anti-Pythium insidiosum.(AU)


Doenças respiratórias causam em equinos custos significativos com tratamento veterinário, redução de performance e descarte de animais. No entanto, estudos que abordem as causas de pneumonia em equinos são escassos. O objetivo deste estudo foi descrever os aspectos patológicos e microbiológicos de lesões pulmonares em equinos abatidos em matadouro-frigorífico no Sul do Brasil. Neste estudo, 84 amostras de pulmões foram examinadas, e o diagnóstico conclusivo das condições foi obtido em 74 casos. Esses foram compostos por broncopneumonia em 50 casos, seguido por pneumonia granulomatosa eosinofílica (9/74), obstrução aérea recorrente (7/74), fibrose pulmonar (4/74), hemorragia pulmonar (3/74) e pitiose pulmonar (1/74). A broncopneumonia era caracterizada macroscopicamente por áreas focalmente extensas firmes de coloração amarelada a vermelho-escuras, as quais consistiam microscopicamente em infiltrado multifocal a coalescente de neutrófilos degenerados. Streptococcus equi subsp. zooepidemicus foi identificado em 21 dos 50 casos. A pneumonia eosinofílica granulomatosa era caracterizada por áreas multifocais puntiformes firmes a duras e amareladas, que microscopicamente eram compostas por granulomas com área central mineralizada circundados por fibras de colágeno e infiltrado acentuado de eosinófilos. A obstrução aérea recorrente era caracterizada por discretas áreas puntiformes firmes e brancacentas que consistiam microscopicamente em grande quantidade de muco no interior de brônquios e bronquíolos. A fibrose pulmonar exibia dois padrões: áreas de consolidação focalmente extensas e áreas nodulares firmes. Microscopicamente, o primeiro padrão exibia fibrose intersticial a peribronquial, enquanto no segundo padrão havia, além da fibrose intersticial, intensa hiperplasia de pneumócitos e infiltrado alveolar de neutrófilos e macrófagos com raros corpúsculos de inclusão intranucleares (herpesvírus equino 5, EHV-5). A pitiose pulmonar exibia uma área nodular firme focal com múltiplos kunkers ao corte, os quais correspondiam microscopicamente a áreas de necrose circundadas por infiltrado inflamatório misto. À periferia das áreas necróticas, múltiplas imagens negativas de hifas eram observadas, as quais foram evidenciadas através da coloração de Grocott e imuno-histoquímica anti-Pythium insidiosum.(AU)


Assuntos
Animais , Pneumonia/veterinária , Fibrose Pulmonar/veterinária , Infecções Estreptocócicas/veterinária , Streptococcus equi , Lesão Pulmonar/veterinária , Pitiose , Doenças dos Cavalos/microbiologia , Doenças dos Cavalos/patologia , Doenças dos Cavalos/epidemiologia
5.
Rev. bras. med. trab ; 16(3): 378-386, out.2018.
Artigo em Inglês, Português | LILACS | ID: biblio-966086

RESUMO

Introdução: A relação entre a sílica e o tabaco como potencializadores na geração de fibrose pulmonar não foi ainda bem estabelecida, embora tenham sido postulados alguns mecanismos fisiopatológicos para embasá-la. É necessário reconhecer o papel das diversas células envolvidas na resposta inflamatória, assim como as diversas vias biológicas que participam na gênese. Esses fatores nos motivaram a desenvolver a presente revisão descritiva. Resultados: Cada vez mais evidências sugerem que a inflamação local produzida por exposição à sílica e à fumaça do tabaco pode ser modulada por fatores genéticos, mecanismos epigenéticos, reações autoimunes e hipóxia local, levando à transição epitélio-mesênquima e ao acúmulo de material necrótico no pulmão, o que contribui à perpetuação da inflamação e a uma resposta imunológica inata exagerada nos trabalhadores com silicose fumantes. Conclusão: Comparações diretas de diferentes estudos de mensuração de biomarcadores inflamatórios associados à silicose e ao tabagismo devem ser realizadas com cautela, devido a uma série de possíveis fatores de confusão, como compartimentalização ou interação com as diversas vias biológicas e tipos celulares envolvidos. Convém destacar que para se evitar a ocorrência de dano pulmonar nos trabalhadores expostos à sílica, devem-se melhorar os sistemas de ventilação e reduzir sua exposição. No contexto da cessação do tabagismo, é necessário o uso de componentes psicoterapêuticos, com o fim de evitar o dano pulmonar precocemente


Background: The relationship between silica dust and tobacco smoking as enhancers of pulmonary fibrosis development has not yet been well established. Some pathophysiological mechanisms which might support this relationship were postulated. The role of different cells involved in the inflammatory response, and of different biological pathways needs to be recognized. These facts encouraged us to perform the present descriptive review. Results: Growing evidence suggests that local inflammation induced by exposure to silica dust and tobacco smoking might be modulated by genetic factors, epigenetic mechanisms, autoimmune reactions and local hypoxia, giving rise to the epithelial­mesenchymal transition. These phenomena lead to accumulation of necrotic material in the lungs, which contributes to inflammation's perpetuation and to an exaggerated innate immunological response among workers with silicosis who smoke. Conclusion: Direct comparisons of different measurement studies of inflammatory biomarkers associated with silicosis and tobacco smoking should be performed cautiously due to several possible confounding factors, such as compartmentalization or interaction among the various biological pathways and cell types involved. Ventilation systems should be improved and exposure reduced to prevent lung damage in workers exposed to silica. In regard to smoking cessation, psychotherapy approaches are needed for early prevention of lung damage


Assuntos
Fibrose Pulmonar/prevenção & controle , Silicose , Tabagismo , Biomarcadores , Dióxido de Silício/efeitos adversos
7.
Rev. méd. Minas Gerais ; 28: [1-7], jan.-dez. 2018.
Artigo em Português | LILACS | ID: biblio-967797

RESUMO

A pneumonia de hipersensibilidade (PH) constitui síndrome inflamatória causada pela resposta imune exagerada a partículas antigênicas inaladas. Objetivo: descrever características clínicas, radiológicas, funcionais esobrevida de pacientes com PH. Metodologia: estudo retrospectivo, envolvendo pacientes em acompanhamento no ambulatório de doenças pulmonares intersticiais do HC-UFMG no período de 2011 a 2015. Analisados dados clínicos, radiológicos, funcionais e morfológicos coletados através de um protocolo padronizado. Resultados: Dentre 139 pacientes com doenças intersticiais pulmonares, 34 (24%) tinham PH, com idade de 60,7 ± 18,1 anos. Exposição a mofo foi a etiologia mais frequente (19;55%). A forma crônica foi predominante (100% doas casos), assim como a presença de padrão reticular na tomografia de tórax (100%), com ou sem faveolamento. Observou-se distúrbio restritivo leve (CVF=69,00 ± 17,62L) associado a redução moderada da difusão de monóxido de carbono (59,20 ± 16,99%). O lavado broncoalveolar não auxiliou no diagnóstico e a biópsia transbrônquica foi diagnóstica em 5 (55%) casos e a biópsia cirúrgica foi conclusiva em 6 (85,7%). A sobrevida mediana foi de 75 meses. Conclusão: Nesta amostra de um ambulatório de referência, a PH respondeu por cerca de um quarto dos pacientes com doença intersticial pulmonar. A exposição a mofo foi a principal etiologia e a mortalidade significativa, o que reforça a importância da adoção de medidas preventivas através do reconhecimento precoce da exposição aos fatores de risco. (AU)


Hypersensitivity pneumonitis (HP) inflammatory is a syndrome caused by an excessive immune response to inhaled antigen particles. Objective:To describe clinical, radiological and functional characteristics and survival in patients with HP. Methodology: Retrospective study involving patients followed up at the pulmonary interstitial diseases clinic of the HC-UFMG from 2011 to 2015. Clinical, radiological, functional and morphological data were analysed from a standardized protocol. Results: Among 139 patients with interstitial lung disease, 34 (24%) had HP, aged 60.7 ± 18.1 years. Mold exposure was the most frequent cause (19; 55%). The chronic form predominated (100% donate cases), as well as the presence of reticular pattern on the chest tomography (100%), with or without honeycombing. There was mild restrictive lung disease (FVC = 69.00 ± 17,62L) associated with moderate reduction in carbon monoxide diffusion (59.20 ± 16.99%). Bronchoalveolar lavage did not help in the diagnosis and transbronchial biopsy was diagnostic in 5 (55%) cases; surgical biopsy was conclusive in 6 (85.7%). Median survival was 60 months. Conclusion: In this sample of a reference clinic, HP accounted for about a quarter of the patients with interstitial lung disease. The mold exposure was the main etiology and the mortality rate was significant, what reinforces the importance of adopting preventive measures through early recognition of exposure to risk factors. (AU)


Assuntos
Humanos , Masculino , Feminino , Alveolite Alérgica Extrínseca , Fibrose Pulmonar , Doenças Pulmonares Intersticiais
9.
Pesqui. vet. bras ; 37(11): 1247-1252, Nov. 2017. graf
Artigo em Inglês | LILACS, VETINDEX | ID: biblio-895358

RESUMO

Equine multinodular pulmonary fibrosis (EMPF) diagnosed at the Laboratório Regional de Diagnóstico of Faculdade de Medicina Veterinária, Universidade Federal de Pelotas (LRD/UFPel), is described. Differential aspects of other pulmonary diseases in horses with pneumonia and interstitial fibrosis were discussed. The disease occurred in a 15-year-old equine that presented with clinical signs of respiratory distress, intermittent fever, anorexia, and dyspnea. Macroscopically, there was enlargement of the lungs with whitish, pale, firm and well-delimited nodules, approximately 7-10 cm in diameter, distributed throughout the parenchyma. Histologically, the lung nodules had alveolar spaces with walls covered by cuboidal epithelium containing macrophages, neutrophils, lymphocytes, hyperplasia of type II pneumocytes and, eventually, multinucleated giant cells. The interstitium was markedly thickened by mature fibrous connective tissue and collagen. There were intranuclear inclusion bodies in the macrophages. The PCR technique for detecting the EHV-5 DNA was positive. In a retrospective study of pneumonia cases in horses with interstitial fibrosis diagnosed in the LRD/UFPel, two animals had macroscopic and histological lesions similar to those with EMPF, but they were negative for EHV-5 in PCR. Four cases diagnosed with pneumonia and interstitial tissue fibrosis had a histological pattern that was different from that observed in the EMPF animal, thus eliminating the possibility of EMPF. It is concluded that EMPF is a sporadic disease that should be considered in cases of respiratory disease in horses. Reports of such cases are important to alert technicians about the occurrence of rare diseases in Brazil. It is also necessary to establish the true role of EHV-5 in the pathogenesis of EMPF. Cases of pulmonary fibrosis such as EMPF, in which the virus is not present, should be studied to establish whether it could be an idiopathic form of the disease.(AU)


Descreve-se a fibrose multinodular pulmonar equina (EMPF) diagnosticado no Laboratório Regional de Diagnóstico da Faculdade de Veterinária da Universidade Federal de Pelotas. Foram discutidos a patologia da doença e os aspectos diferenciais de outras enfermidades pulmonares de equinos que cursam com pneumonia e fibrose intersticial. A doença ocorreu em um equino sem raça definida de 15 anos de idade que apresentou sinais clínicos de dificuldade respiratória febre intermitente, anorexia e dispneia, com evolução de aproximadamente 10 dias. Macroscopicamente havia aumento de volume dos pulmões e nódulos esbranquiçados, pálidos, firmes e bem delimitados, de aproximadamente 7-10 cm de diâmetro, distribuídos pelo parênquima. Histologicamente, o tecido pulmonar apresentava nódulos caracterizados pela presença de espaços alveolares, com paredes revestidas por epitélio cuboidal achatado, contendo macrófagos e neutrófilos e havia, também, linfócitos e hiperplasia de pneumócitos tipo II e eventualmente células gigantes multinuacleadas. O interstício estava acentuadamente espessado por tecido conjuntivo fibroso maduro e por colágeno. Havia corpúsculos de inclusão intranucleares em macrófagos. A técnica de PCR para detecção do DNA de herpes vírus equino-5 (EHV-5) resultou positiva. Em um estudo retrospectivo de casos de pneumonia com fibrose intersticial diagnosticados no LRD entre 2000 e 2015, dois equinos apresentaram lesões macroscópicas e histológicas similares às de EMPF, porém resultaram negativos na PCR para detecção de EHV-5. Quatro casos de pneumonia com fibrose do tecido intersticial apresentaram padrão histológico diverso da EMPF descartando-se a possibilidade de tratar-se da doença. Conclui-se que EMPF é uma enfermidade esporádica, no entanto deve ser levada em consideração em casos de doença respiratória em equinos. A descrição dos casos diagnosticados é importante para alertar técnicos sobre a ocorrência da mesma no Brasil. É necessário estabelecer o real papel do EHV-5 na patogenia da doença. Casos de fibrose pulmonar semelhantes à EMPF em que não esteja presente o vírus, devem ser estudados a fim de ficar estabelecido se poderia ser uma forma idiopática da mesma doença.(AU)


Assuntos
Animais , Fibrose Pulmonar/patologia , Fibrose Pulmonar/veterinária , Doenças dos Cavalos , Estudos Retrospectivos , Diagnóstico Diferencial
10.
Autops. Case Rep ; 7(2): 15-26, Apr.-June 2017. ilus
Artigo em Inglês | LILACS | ID: biblio-905200

RESUMO

In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Hipertensão Pulmonar/patologia , Doença Pulmonar Obstrutiva Crônica/patologia , Enfisema Pulmonar/patologia , Fibrose Pulmonar/patologia , Autopsia , Dispneia/diagnóstico , Edema/diagnóstico , Evolução Fatal , Pulmão/patologia , Doença Cardiopulmonar/diagnóstico , Fumar Tabaco/efeitos adversos
11.
Rev. chil. enferm. respir ; 33(1): 31-36, mar. 2017. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-844394

RESUMO

Diffuse Lung Disease (DLD) is an important cause of morbidity and mortality, however in Chile we are lacking of epidemiological data on this condition. Our aim is communicate the first report of a prospective registry of DLD patients treated at bronchopulmonary unit of DIPRECA hospital. Methods: Cross-sectional study in patients referred to our bronchopulmonary unit under suspicion of DLD. Diagnosis was confirmed by chest computed tomography and informed consent was approved by patients. Data regarding clinical, serological, pulmonary function tests and echocardiography were collected from 2014 up to date. Results: 30 patients were analysed, their median of age was 76.5 years-old (Interquartile Range 68-80), 56.7% were women, median duration of disease: 4 years (IQR 1-10.6) and 43% has smoking history Most frequent signs and symptoms were crackles (97%), dyspnoea (90%) and cough (57%). Comorbidities: 3% had asthma, 3% chronic obstructive pulmonary disease and 6.7% connective tissue diseases. Radiological findings: 20% had DLD with usual interstitial pneumonia pattern (UIP), 23% DLD possible UIP, 30% DLD inconsistent with UIP, 14% chronic hypersensitivity pneumonitis and 13% nonspecific interstitial pneumonia. Serology: 18% had positive rheumatoid factor of which only one case had rheumatoid arthritis, 67% had positive antinuclear antibodies (ANA), 17% ANCA positive of which only one case of clinical vasculitis. Spirometry was mainly normal (52%) or restrictive (45%). Echocardiography showed pulmonary hypertension mainly mild in 52% of patients. No significant association was found between titles of ANA ≥ 1/320 and gender, smoking or radiological pattern. Conclusions: Our demographic and radiological findings are similar to those reported in literature; however, the highlights in our cohort are the increased frequency of female gender and positive ANA without history or clinical manifestation of connective tissue diseases.


La enfermedad pulmonar difusa (EPD) es causa importante de morbimortalidad; a pesar de esto no tenemos datos epidemiológicos en Chile. Nuestro objetivo es comunicar el primer reporte del registro prospectivo de pacientes con EPD atendidos en la unidad de broncopulmonar del hospital DIPRECA. Métodos: Estudio de corte transversal en pacientes derivados alpoliclínico broncopulmonar del hospital DIPRECA por sospecha de EPD. En caso de confirmación diagnóstica por tomografía computada de tórax y consentimiento informado aprobado por los pacientes, se compilaron datos clínicos, serológicos, pruebas de función pulmonar y ecocardiografía, desde 2014 hasta la fecha. Resultados: Se analizaron 30 pacientes la mediana de su edad fue 76,5 años (rango intercuartílico 68-80), 56,7% eran mujeres, duración mediana de la enfermedad: 4 años (RIC 1-10,6)y 43% con antecedentes de tabaquismo. Los síntomas y signos más frecuente fueron crujidos (97%), disnea (90%) y tos (57%). Comorbilidades: 3% tenía asma, 3% enfermedad pulmonar obstructiva crónica y 6,7% enfermedades del tejido conectivo. Hallazgos radiológicos: 20% tenía EPD con patrón de neumonía intersticial usual (UIP), 23% EPD posible UIP, 30% EPD inconsistente con UIP, 14% neumonitis por hipersensibilidad crónica y 13% neumonía intersticial no específica. Serología: 18% tenía factor reumatoide positivo de ellos sólo uno de los casos tenía artritis reumatoide, el 67% tenía anticuerpos antinucleares (ANA) positivos, 17% ANCA positivo de ellos sólo un caso tenía historia de vasculitis clínica. La espirometría fue mayoritariamente normal (52%) o restrictiva (45%). Ecocardiografía detectó hipertensión pulmonar mayoritariamente leve en 52% de los pacientes. No se encontró asociación significativa entre los títulos de ANA ≥ 1/320 en relación a género, tabaquismo o patrón radiológico. Conclusiones: Nuestros hallazgos demográficos y radiológicos son similares a los de la literatura; sin embargo, destaca en nuestra cohorte la mayor frecuencia de género femenino y ANA positivos sin historia o manifestación clínica de enfermedades del tejido conectivo.


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Doenças Pulmonares Intersticiais/patologia , Fibrose Pulmonar/patologia , Anticorpos Antinucleares , Autoimunidade , Estudos Transversais , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/imunologia , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/imunologia , Fatores Sexuais , Tomografia Computadorizada por Raios X
12.
Rev. am. med. respir ; 17(1): 102-108, mar. 2017. ilus
Artigo em Espanhol | LILACS | ID: biblio-843042

RESUMO

Se presenta un paciente masculino, de 67 años, residente en la provincia de La Rioja, quien concurre al Servicio de Neumonología del Hospital Vera Barros. Ex fumador importante (60 p/y hasta hace 5 años), trabajó en curtiembre y con forrajes. Consultó por disnea de esfuerzo grado III en la escala modificada del Medical Researh Council (mMRC) y tos seca de 3 o 4 años de evolución. Estos síntomas se agudizaban 3 o 4 veces al año y requerían medicación que no podía precisar


Assuntos
Enfisema Pulmonar , Fibrose Pulmonar
13.
Rev. am. med. respir ; 16(3): 288-289, set. 2016.
Artigo em Espanhol | LILACS | ID: biblio-843005

RESUMO

El Ateneo Multicéntrico "EPID asociada a ANCA" publicado en el último número de la RAMR comenta el caso de un paciente con dicha asociación. Las patologías que padece el enfermo son comentadas con muy buen criterio clínico y estoy de acuerdo en todo lo que se dice. Sin embargo, me permito agregar y enfatizar algunos puntos. La combinación de Fibrosis Pulmonar (FP) y Vasculitis-ANCA (VAA) fue descripta por primera vez por Nada y col. en 1990 al reportar dos casos de FP y ANCA-P1 .


Assuntos
Fibrose Pulmonar , Vasculite
14.
Rev. chil. enferm. respir ; 32(1): 13-17, mar. 2016. graf, tab
Artigo em Espanhol | LILACS | ID: lil-784857

RESUMO

Introduction: The presence ofpulmonary hypertension (PH) in patients with pulmonary fibrosis is a predictor of severity andpoor survival in patients awaiting lung transplantation. Little is known about the impact of PH on survival after lung transplantation. Objective: To evaluate the effect of PH in pulmonary fibrosis patient survival after lung transplantation. Methods: Retrospective study ofpatients diagnosed with pulmonary fibrosis subjected to lung transplantation at the Instituto Nacional del Tórax during the period of August 2010 to June 2015. Thresholds of > 25 and > 35 mm Hg were chosen for mean pulmonary artery pressure (PAmean) and systolic pulmonary artery pressure (PAsystolic), respectively as indicators of PH. Results: Out of a total of 63 patients undergoing lung transplantation during the 2010-2015 period, 42 patients were diagnosed with pulmonary fibrosis. 35 of these patients had histologic diagnosis of usual interstitial pneumonia (UIP) and 7 of extrinsic allergic alveolitis in fibrotic stage. Of the total 25 patients with pulmonary fibrosis (60 percent) had PH in the pre-transplant period. A total of 15 patients died during the follow-up. There was no significant difference in survival between patients with and without PH (p = 0.74). Conclusions: Similar to international studies, we observed that the presence of PH in patients with pulmonary fibrosis did not increase risk of death in post-transplant period.


Introducción: La presencia de hipertensión pulmonar (HTP) en pacientes con fibrosis pulmonar es un predictor de gravedad y pobre sobrevida en pacientes en espera de trasplante pulmonar. Poco se sabe del impacto de la HTP en la sobrevida de los pacientes en el período post trasplante. Objetivo: Evaluar el efecto de la HTP en la sobrevida de los pacientes con fibrosis pulmonar sometidos a trasplante pulmonar. Material y Método: Estudio retrospectivo de pacientes con diagnóstico de fibrosis pulmonar sometidos a trasplante pulmonar en el Instituto Nacional de Tórax durante el período de agosto de 2010 a junio de 2015. Los criterios diagnósticos de hipertensión pulmonar fueron: presión de arteria pulmonar media mayor o igual a 25 mmHg y/o presión sistólica de arteria pulmonar mayor o igual a 35 mmHg. Resultados: De un total de 63 pacientes sometidos a trasplante pulmonar durante el período 2010-2015 en el Hospital del Tórax, 42 pacientes tenían diagnóstico de fibrosis pulmonar. De estos, 35 pacientes tenían diagnóstico histológico de neumonía intersticial usual (UIP) y 7 de alveolitis alérgica extrínseca en etapa fibrótica. Del total de pacientes con fibrosis pulmonar, 25 (60 por ciento) presentaba HTP en el período pre trasplante. Un total de 15 pacientes fallecieron durante el seguimiento. Al comparar la sobrevida post trasplante de pacientes con HTP vs sin HTP no se observó diferencia significativa (p = 0,74). Conclusiones: Al igual que en estudios internacionales, no observamos que la presencia de HTP en pacientes con fibrosis pulmonar aumente el riesgo de muerte en el período post-trasplante.


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Hipertensão Pulmonar/epidemiologia , Transplante de Pulmão , Fibrose Pulmonar , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/terapia
15.
Univ. med ; 57(4): 508-516, oct. - dic. 2016. ilus
Artigo em Espanhol | LILACS, COLNAL | ID: biblio-1007423

RESUMO

En la última década, la radioterapia estereotáctica corporal se ha implementado como tratamiento a pacientes con cáncer de pulmón estadio I, quienes son considerados inoperables o quienes rechazan el procedimiento quirúrgico. El artículo presenta el caso de uno de los primeros pacientes tratados con esta tecnología en un centro oncológico: un hombre de 72 años de edad con cáncer de pulmón estadio I, quien rechazó el manejo quirúrgico y se le realizó el tratamiento con radiocirugía, y con dos años de seguimiento, presentó cambios radiográficos, que inicialmente se interpretaron como progresión de la enfermedad. Además, se revisa la literatura relacionada con la interpretación de imágenes de seguimiento después del tratamiento.


Stereotactic radiotherapy body has been implemented in the last decade as a treatment for patients with stage I lung cancer, who are considered inoperable or who reject the surgical pro- cedure. For one of the first patients treated with this technology in our Cancer Center presented; 72 year old man with lung cancer stage I reject the surgical management and underwent radio- surgery treatment and for 2 years follow-up, presented radiographic changes, which were initially interpreted as disease progression. Additionally, a review of the literature related to image interpretation follow-up after treatment is done


Assuntos
Humanos , Radioterapia , Fibrose Pulmonar , Carcinoma de Pequenas Células do Pulmão
16.
Rev. am. med. respir ; 15(3): 171-189, set. 2015. graf, tab
Artigo em Espanhol | LILACS | ID: biblio-842921

RESUMO

Introducción: Las enfermedades pulmonares intersticiales difusas (EPID) son un grupo de enfermedades raras que, si bien comparten ciertas características clínicas, tienen un pronóstico muy diferente. La fibrosis pulmonar idiopática (FPI) es la más prevalente en muchos países y su diagnóstico puede ser dificultoso. Luego de los resultados expuestos en el consenso sobre diagnóstico y manejo de la FPI, y la llegada de nuevas drogas como la pirfenidona, se ha modifcado el enfoque de esta enfermedad. Se realizó una encuesta a neumonólogos argentinos, con el fin de evaluar la aceptabilidad e implementación de estas guías en Argentina. Materiales y métodos: Se diseñó una encuesta con 24 preguntas. Entre los datos que se recolectaron en el cuestionario estaban demografía de los encuestados, lugar de trabajo (instituciones públicas, privadas, grandes o pequeños centros o instituciones), frecuencia con la que evaluaban pacientes con FPI, disponibilidad de pruebas diagnósticas y estrategias diagnósticas empleadas para pacientes con EPID. Por último, la encuesta se focalizó en las recomendaciones terapéuticas en los pacientes diagnosticados como FPI. Dicha encuesta fue completada durante el Congreso Argentino de Medicina Respiratoria que se realizó en el 2013 en la ciudad de Mendoza. La misma metodología y cuestionario fueron utilizados previamente en el Congreso Argentino de Medicina Respiratoria del 2011. Resultados: Un total de 252 médicos respondieron la encuesta en el 2013, lo que representó alrededor del 20% de los concurrentes al congreso. El método complementario de mayor disponibilidad fue la prueba de marcha de 6 minutos (PM6M). El método complementario más utilizado fue la tomografía computada de tórax (86.9% de los encuestados la realizaban ante la sospecha de EPID) y solo el 44.4% de los encuestados realizaban difusión de monóxido de carbono (DLCO) en todos sus pacientes. Cerca del 50% de los encuestados consultaban a centros de referencia en menos del 30% de sus casos con sospecha de EPID. Menos del 20% de los respondedores consideraban que llegaban a un diagnóstico defnitivo de EPID en más del 60% de sus pacientes. La distribución final de los diagnósticos fue heterogénea. Notablemente, casi el 50% de los encuestados consideraba que la FPI había sido el diagnóstico final en menos de 30% de sus pacientes. Solo el 30% de los encuestados prescribieron pirfenidona como tratamiento de elección en la FPI y más del 60% todavía continuaban prescribiendo tratamientos que incluían diferentes combinaciones de corticoides e inmunosupresores. Conclusiones: Nuestra encuesta sugiere que existen dificultades en el abordaje diagnóstico de estas entidades, que existe un bajo porcentaje de pacientes que son evaluados en centros de referencia y que hay una baja proporción de estos que reciben tratamiento específico.


Background: Diffuse interstitial (or parenchymal) lung diseases (ILDs) are a very large group of diseases that although they share certain clinical features, have a very different prognosis. Idiopathic pulmonary fibrosis (IPF) is the most prevalent in many countries and its diagnosis can be difficult. After the results shown in the consensus on diagnosis and management of IPF, and the arrival of new drugs such as pirfenidone, the approach to this disease have changed. A survey was performed to argentine pulmonologists in order to evaluate the acceptability and implementation of these guidelines in Argentina. Material and Methods: A survey of 24 questions was designed. Among the data collected in the questionnaire were demographics of respondents, workplace (public or private healthcare facilities, referral center, large or small healthcare centers or institutions), frequency at which IPF patients were examined, availability of diagnostic tests, and diagnostic strategies used with ILD patients. Finally, the survey focused on therapeutic recommendations for patients diagnosed with IPF. The survey was completed during the Argentine Congress of Respiratory Medicine held in 2013 in the city of Mendoza. The same methodology and questionnaire were previously used in the Argentine Congress of Respiratory Medicine in 2011. Results: In 2013, a total of 252 physicians completed the survey, which represented approximately 20% of Congress attendees. The complementary test of higher availability was the the six minutes walk test (6MWT). The most widely used supplementary method was thoracic computed tomography (CT) as 86.9% of the responders used it if they suspected ILD, and only 44.4% of the responders used diffusing capacity of the lungs for carbon monoxide (DLCO) with all their patients. Almost 50% of the responders consulted referral centers for less than 30% of patients with suspected ILD. Less than 20% of the responders considered that they reached a final diagnosis of ILD in over 60% of their patients. Final distribution of diagnosis was heterogeneous. Interestingly, almost 50% of the responders considered IPF as the fnal diagnosis in less than 30% of their patients. Approximately 50% of the responders answered that less than 20% of their IPF patients received specifc treatment for the disease. Conclusions: Our survey suggests that there are difficulties in the diagnostic approach of ILDs, there is a low percentage of patients that are evaluated in referral centers and there is a low proportion of IPF patients receiving specific treatment


Assuntos
Fibrose Pulmonar , Pneumopatias , Doença Mista do Tecido Conjuntivo
17.
Rev. bras. epidemiol ; 18(2): 413-424, Apr.-Jun. 2015. tab
Artigo em Inglês | LILACS | ID: lil-755179

RESUMO

OBJECTIVE:

To evaluate the access to drugs for hypertension and diabetes and the direct cost of buying them among users of the Family Health Strategy (FHS) in the state of Pernambuco, Brazil.

METHODS:

Population-based, cross-sectional study of a systematic random sample of 785 patients with hypertension and 823 patients with diabetes mellitus who were registered in 208 randomly selected FHS teams in 35 municipalities of the state of Pernambuco. The selected municipalities were classified into three levels with probability proportional to municipality size (LS, large-sized; MS, medium-sized; SS, small-sized). To verify differences between the cities, we used the χ2 test.

RESULTS:

Pharmacological treatment was used by 91.2% patients with hypertension whereas 85.6% patients with diabetes mellitus used oral antidiabetic drugs (OADs), and 15.4% used insulin. The FHS team itself provided antihypertensive medications to 69.0% patients with hypertension, OADs to 75.0% patients with diabetes mellitus, and insulin treatment to 65.4%. The 36.9% patients with hypertension and 29.8% with diabetes mellitus that had to buy all or part of their medications reported median monthly cost of R$ 18.30, R$ 14.00, and R$ 27.61 for antihypertensive drugs, OADs, and insulin, respectively.

CONCLUSION:

It is necessary to increase efforts to ensure access to these drugs in the primary health care network.

.

OBJETIVO:

Avaliar o acesso a medicamentos para hipertensão e diabetes e o gasto direto relacionado à aquisição destes insumos entre os usuários da Estratégia Saúde da Família (ESF), no estado de Pernambuco.

MÉTODOS:

Estudo transversal, de base populacional, numa amostra aleatória sistemática de 785 pacientes hipertensos e 823 diabéticos cadastrados em 208 equipes da ESF sorteadas em 35 municípios do estado de Pernambuco. Os municípios selecionados foram classificados em três estratos com probabilidade proporcional ao tamanho do município (GP: grande porte; MP: médio porte; PP: pequeno porte). A fim de verificar diferenças entre os municípios, foi utilizado o teste χ2.

RESULTADOS:

Dos 785 hipertensos, 91,2% referiram o uso de anti-hipertensivos e dos 823 diabéticos, 85,6% utilizavam antidiabéticos orais (ADO), e 15,4%, insulina. Os anti-hipertensivos eram fornecidos pelas equipes da ESF para 69,0% dos hipertensos, os ADO, para 75,0% dos diabéticos, e a insulina e insumos, para 65,4%. Os hipertensos (36,9%) e os diabéticos (29,8%) que precisavam comprar os medicamentos referiram um gasto mediano mensal de R$ 18,30, R$ 14,00 e R$ 27,61 para anti-hipertensivos, ADO e insulina, respectivamente.

CONCLUSÃO:

É necessário ampliar os esforços para assegurar o acesso aos medicamentos na rede de atenção primária de saúde.

.


Assuntos
Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Transplante de Pulmão , Pneumopatias/cirurgia , Fatores Etários , Bronquiectasia/mortalidade , Bronquiectasia/cirurgia , Irã (Geográfico) , Estimativa de Kaplan-Meier , Pneumopatias/mortalidade , Transplante de Pulmão/efeitos adversos , Transplante de Pulmão/mortalidade , Análise Multivariada , Fibrose Pulmonar/mortalidade , Fibrose Pulmonar/cirurgia , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Fatores de Tempo , Doadores de Tecidos , Resultado do Tratamento
18.
Braz. j. med. biol. res ; 48(6): 545-552, 06/2015. tab, graf
Artigo em Inglês | LILACS | ID: lil-748222

RESUMO

Abnormal high mobility group protein B1 (HMGB1) activation is involved in the pathogenesis of pulmonary fibrosis. Pulmonary rehabilitation mixture (PRM), which combines extracts from eight traditional Chinese medicines, has very good lung protection in clinical use. However, it is not known if PRM has anti-fibrotic activity. In this study, we investigated the effects of PRM on transforming growth factor-β1 (TGF-β1)-mediated and bleomycin (BLM)-induced pulmonary fibrosis in vitro and in vivo. The effects of PRM on TGF-β1-mediated epithelial-mesenchymal transition (EMT) in A549 cells, on the proliferation of human lung fibroblasts (HLF-1) in vitro, and on BLM-induced pulmonary fibrosis in vivo were investigated. PRM treatment resulted in a reduction of EMT in A549 cells that was associated with attenuating an increase of vimentin and a decrease of E-cadherin. PRM inhibited the proliferation of HLF-1 at an IC50 of 0.51 µg/mL. PRM ameliorated BLM-induced pulmonary fibrosis in rats, with reduction of histopathological scores and collagen deposition, and a decrease in α-smooth muscle actin (α-SMA) and HMGB1 expression. An increase in receptor for advanced glycation end-product (RAGE) expression was found in BLM-instilled lungs. PRM significantly decreased EMT and prevented pulmonary fibrosis through decreasing HMGB1 and regulating RAGE in vitro and in vivo. PRM inhibited TGF-β1-induced EMT via decreased HMGB1 and vimentin and increased RAGE and E-cadherin levels. In summary, PRM prevented experimental pulmonary fibrosis by modulating the HMGB1/RAGE pathway.


Assuntos
Animais , Humanos , Masculino , Medicamentos de Ervas Chinesas/farmacologia , Fibrose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/prevenção & controle , Antibióticos Antineoplásicos , Receptor para Produtos Finais de Glicação Avançada/efeitos dos fármacos , Apoptose/efeitos dos fármacos , Bleomicina , Western Blotting , Células Cultivadas , Colágeno/efeitos dos fármacos , Misturas Complexas/farmacologia , Medicamentos de Ervas Chinesas/uso terapêutico , Transição Epitelial-Mesenquimal/efeitos dos fármacos , Fibroblastos/efeitos dos fármacos , Proteína HMGB1/efeitos dos fármacos , Hidroxiprolina/análise , Imuno-Histoquímica , Pulmão/efeitos dos fármacos , Pulmão/patologia , Fator de Crescimento Derivado de Plaquetas/efeitos dos fármacos , Fibrose Pulmonar/patologia , Distribuição Aleatória , Ratos Sprague-Dawley , Reprodutibilidade dos Testes , Fator de Crescimento Transformador beta1/efeitos dos fármacos
19.
Braz. j. med. biol. res ; 48(3): 267-272, 03/2015. tab, graf
Artigo em Inglês | LILACS | ID: lil-741252

RESUMO

18F-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) is widely used to diagnose and stage non-small cell lung cancer (NSCLC). The aim of this retrospective study was to evaluate the predictive ability of different FDG standardized uptake values (SUVs) in 74 patients with newly diagnosed NSCLC. 18F-FDG PET/CT scans were performed and different SUV parameters (SUVmax, SUVavg, SUVT/L, and SUVT/A) obtained, and their relationship with clinical characteristics were investigated. Meanwhile, correlation and multiple stepwise regression analyses were performed to determine the primary predictor of SUVs for NSCLC. Age, gender, and tumor size significantly affected SUV parameters. The mean SUVs of squamous cell carcinoma were higher than those of adenocarcinoma. Poorly differentiated tumors exhibited higher SUVs than well-differentiated ones. Further analyses based on the pathologic type revealed that the SUVmax, SUVavg, and SUVT/L of poorly differentiated adenocarcinoma tumors were higher than those of moderately or well-differentiated tumors. Among these four SUV parameters, SUVT/L was the primary predictor for tumor differentiation. However, in adenocarcinoma, SUVmax was the determining factor for tumor differentiation. Our results showed that these four SUV parameters had predictive significance related to NSCLC tumor differentiation; SUVT/L appeared to be most useful overall, but SUVmax was the best index for adenocarcinoma tumor differentiation.


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Silicatos de Alumínio/toxicidade , Doenças Profissionais/induzido quimicamente , Exposição Ocupacional/efeitos adversos , Fibrose Pulmonar/induzido quimicamente , Biópsia , Evolução Fatal , Testes de Função Respiratória
20.
Rev. am. med. respir ; 15(1): 36-50, mar. 2015. graf, tab
Artigo em Espanhol | LILACS | ID: biblio-842896

RESUMO

Introducción: La neumonía intersticial usual (NIU) es un patrón histológico que conlleva mal pronóstico. Sin embargo, en los últimos años se ha sugerido que la NIU asociada a las enfermedades del tejido conectivo (NIU-ETC) puede tener un comportamiento diferente a la asociada a la fibrosis pulmonar idiopática (FPI). Objetivos: Conocer si existen diferencias en la severidad y supervivencia entre los pacientes con NIU asociada a FPI y los pacientes con NIU en contexto de ETC, incluyendo esclerosis sistémica, artritis reumatoidea, polidermatomiositis y enfermedad mixta del tejido conectivo. Materiales y métodos: Fueron evaluadas las características clínicas y la supervivencia de 102 pacientes (81 con FPI y 21 con NIU-ETC) diagnosticados en base a biopsia quirúrgica o una tomografía computada de alta resolución (TCAR) con NIU definida. Resultados: La media de seguimiento fue de 24 meses (0 a 146 meses). Cuarenta y cuatro pacientes murieron durante el seguimiento, una proporción significativamente mayor entre los pacientes con FPI que entre los pacientes con NIU-ETC (49.4 vs 19.0%, p = 0.014) y la supervivencia a 3 y 5 años fue mayor en pacientes con NIU asociada a ETC que en pacientes con FPI. Los pacientes con NIU-ETC tuvieron una tasa de mortalidad a los 3 y 5 años de 19.5% y 20.0%, respectivamente, comparado con pacientes con FPI que tuvieron una tasa de mortalidad a 3 y 5 años de 35.0%, y 65.9% respectivamente (p = 0,014). Los pacientes con FPI fueron mayores que los pacientes con NIU-ETC (edad 67.95 ± 9.4 vs 57.78 ± 14.5, p = 0.021), con una proporción mayor de pacientes de sexo masculino (67.9% vs 33.3%, p = 0.006). No hubo diferencias significativas en la función pulmonar basal, la cantidad de pacientes con disnea en el momento del diagnóstico, el tiempo de inicio de síntomas al diagnóstico o en número de pacientes biopsiados entre ambos grupos. En el análisis multivariado, la DLCO y el diagnóstico de FPI fueron los únicos factores pronósticos independientes. Conclusiones: Nuestro estudio sugiere que los pacientes con NIU-ETC se asocian con una mejor supervivencia que aquellos pacientes con FPI, a pesar de presentar la misma severidad de enfermedad al momento del diagnóstico.


Background: Usual interstitial pneumonia (UIP) is a histologic pattern that implies poor prognosis. However, some studies have suggested that UIP associated to connective tissue diseases (CTD-UIP) may have a different outcome than that associated with idiopathic pulmonary fibrosis (IPF). Objectives: To compare disease severity and survival between IPF and UIP associated to connective tissue diseases including scleroderma, rheumatoid arthritis, polymyositis and mixed CTD. Methods: The study included the analysis of clinical features and survival of 102 patients (81 with IPF and 21with CTD-UIP) diagnosed through surgical biopsy or high resolution computed tomography (HRCT) in patients with definitive UIP. Results: Median follow-up was 24 months (0 to 146 months). Forty-four patients died during the follow-up; the proportion of deaths was significantly higher amongst patients with IPF than amongst patients with CTD-UIP (49.4 vs 19.0%, p = 0.014). The 3 and 5 year survival was higher in patients with UIP secondary to CTD than in patients with IPF. Patients with CTD-UIP showed 3 and 5-year case fatality rate of 19.5% and 20.0% respectively, compared to 3 and 5-year case fatality rate of 35.0%, and 65.9% respectively in patients with IPF (p = 0.014). Patients with IPF were older than patients with CTD-UIP (age 67.95 ±9.4 vs 57.78 ±14.5, p = 0.021) and were more likely to be male (67.9% vs 33.3%, p = 0.006). There were no significant differences among baseline lung function, time between onset of symptoms and diagnosis, number of patients biopsied and the proportion of patients with dyspnea at the time of diagnosis between IPF and CTD-UIP patients. By multivariate analysis, the diffusing capacity of the lung for carbon monoxide (DLCO) and the presence of IPF were independent prognostic factors. Conclusions: Our data suggest that patients with UIP associated to CTD have a better survival than patients with IPF related UIP despite similar disease severity at the time of the diagnosis.


Assuntos
Pneumonia , Fibrose Pulmonar , Doenças Pulmonares Intersticiais , Doença Mista do Tecido Conjuntivo
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