RESUMEN
OBJECTIVE: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. METHODS: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. RESULTS: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (c2 = 312.4; p < 0.001). CONCLUSIONS: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.
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Alveolitis Alérgica Extrínseca , Enfermedades del Tejido Conjuntivo , Fibrosis Pulmonar Idiopática , Enfermedades Pulmonares Intersticiales , Humanos , Femenino , Persona de Mediana Edad , Anciano , Masculino , Estudios Retrospectivos , Incidencia , Brasil/epidemiología , Enfermedades Pulmonares Intersticiales/epidemiología , Alveolitis Alérgica Extrínseca/diagnóstico , Alveolitis Alérgica Extrínseca/epidemiología , Enfermedades del Tejido Conjuntivo/complicacionesRESUMEN
ABSTRACT Objective: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. Methods: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. Results: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (c2 = 312.4; p < 0.001). Conclusions: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.
RESUMO Objetivo: Avaliar a frequência relativa de casos incidentes de doenças pulmonares intersticiais (DPI) no Brasil. Métodos: Levantamento retrospectivo de casos novos de DPI em seis centros de referência entre janeiro de 2013 e janeiro de 2020. O diagnóstico de DPI seguiu os critérios sugeridos por órgãos internacionais ou foi feito por meio de discussão multidisciplinar (DMD). A condição foi caracterizada como DPI não classificável quando não houve um diagnóstico final específico após a DMD ou houve discordância entre dados clínicos, radiológicos ou histológicos. Resultados: A amostra foi composta por 1.406 pacientes (média de idade = 61 ± 14 anos), sendo 764 (54%) do sexo feminino. Dos 747 casos expostos a antígenos para pneumonite de hipersensibilidade (PH), 327 (44%) tiveram diagnóstico final de PH. Houve relato de história familiar de DPI em 8% dos casos. Os achados de TCAR foram indicativos de fibrose em 74% dos casos, incluindo faveolamento, em 21%. Autoanticorpos relevantes foram detectados em 33% dos casos. Biópsia transbrônquica foi realizada em 23% dos pacientes, e biópsia pulmonar cirúrgica, em 17%. Os diagnósticos finais foram: DPI associada à doença do tecido conjuntivo (em 27%), PH (em 23%), fibrose pulmonar idiopática (em 14%), DPI não classificável (em 10%) e sarcoidose (em 6%). Os diagnósticos variaram significativamente entre os centros (c2 = 312,4; p < 0,001). Conclusões: Nossos achados mostram que DPI associada à doença do tecido conjuntivo é a DPI mais comum no Brasil, seguida pela PH. Esses resultados destacam a necessidade de uma estreita colaboração entre pneumologistas e reumatologistas, a importância de fazer perguntas detalhadas aos pacientes a respeito da potencial exposição a antígenos e a necessidade de campanhas de saúde pública destinadas a enfatizar a importância de evitar essa exposição.
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Advances in the understanding that severe asthma is a complex and heterogeneous disease and in the knowledge of the pathophysiology of asthma, with the identification of different phenotypes and endotypes, have allowed new approaches for the diagnosis and characterization of the disease and have resulted in relevant changes in pharmacological management. In this context, the definition of severe asthma has been established, being differentiated from difficult-to-control asthma. These recommendations address this topic and review advances in phenotyping, use of biomarkers, and new treatments for severe asthma. Emphasis is given to topics regarding personalized management of the patient and selection of biologicals, as well as the importance of evaluating the response to treatment. These recommendations apply to adults and children with severe asthma and are targeted at physicians involved in asthma treatment. A panel of 17 Brazilian pulmonologists was invited to review recent evidence on the diagnosis and management of severe asthma, adapting it to the Brazilian reality. Each of the experts was responsible for reviewing a topic or question relevant to the topic. In a second phase, four experts discussed and structured the texts produced, and, in the last phase, all experts reviewed and approved the present manuscript and its recommendations.
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Asma , Asma/diagnóstico , Asma/tratamiento farmacológico , Biomarcadores , Brasil , Humanos , FenotipoRESUMEN
The pharmacological management of asthma has changed considerably in recent decades, as it has come to be understood that it is a complex, heterogeneous disease with different phenotypes and endotypes. It is now clear that the goal of asthma treatment should be to achieve and maintain control of the disease, as well as to minimize the risks (of exacerbations, disease instability, accelerated loss of lung function, and adverse treatment effects). That requires an approach that is personalized in terms of the pharmacological treatment, patient education, written action plan, training in correct inhaler use, and review of the inhaler technique at each office visit. A panel of 22 pulmonologists was invited to perform a critical review of recent evidence of pharmacological treatment of asthma and to prepare this set of recommendations, a treatment guide tailored to use in Brazil. The topics or questions related to the most significant changes in concepts, and consequently in the management of asthma in clinical practice, were chosen by a panel of experts. To formulate these recommendations, we asked each expert to perform a critical review of a topic or to respond to a question, on the basis of evidence in the literature. In a second phase, three experts discussed and structured all texts submitted by the others. That was followed by a third phase, in which all of the experts reviewed and discussed each recommendation. These recommendations, which are intended for physicians involved in the treatment of asthma, apply to asthma patients of all ages.
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Corticoesteroides/administración & dosificación , Antiasmáticos/administración & dosificación , Asma/tratamiento farmacológico , Manejo de la Enfermedad , Administración por Inhalación , Factores de Edad , Brasil , Humanos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Brote de los SíntomasRESUMEN
ABSTRACT The pharmacological management of asthma has changed considerably in recent decades, as it has come to be understood that it is a complex, heterogeneous disease with different phenotypes and endotypes. It is now clear that the goal of asthma treatment should be to achieve and maintain control of the disease, as well as to minimize the risks (of exacerbations, disease instability, accelerated loss of lung function, and adverse treatment effects). That requires an approach that is personalized in terms of the pharmacological treatment, patient education, written action plan, training in correct inhaler use, and review of the inhaler technique at each office visit. A panel of 22 pulmonologists was invited to perform a critical review of recent evidence of pharmacological treatment of asthma and to prepare this set of recommendations, a treatment guide tailored to use in Brazil. The topics or questions related to the most significant changes in concepts, and consequently in the management of asthma in clinical practice, were chosen by a panel of experts. To formulate these recommendations, we asked each expert to perform a critical review of a topic or to respond to a question, on the basis of evidence in the literature. In a second phase, three experts discussed and structured all texts submitted by the others. That was followed by a third phase, in which all of the experts reviewed and discussed each recommendation. These recommendations, which are intended for physicians involved in the treatment of asthma, apply to asthma patients of all ages.
RESUMO O manejo farmacológico da asma mudou consideravelmente nas últimas décadas, com base no entendimento de que a asma é uma doença heterogênea e complexa, com diferentes fenótipos e endótipos. Agora está claro que o objetivo do tratamento da asma deve ser alcançar e manter o controle da doença e evitar riscos futuros (exacerbações, instabilidade da doença, perda acelerada da função pulmonar e efeitos adversos do tratamento). Isso implica em uma abordagem personalizada, incluindo tratamento farmacológico, educação do paciente, plano de ação por escrito, treinamento para uso do dispositivo inalatório e revisão da técnica inalatória a cada visita ao consultório. Um painel de 22 pneumologistas brasileiros foi convidado a revisar criticamente evidências recentes de tratamento farmacológico da asma e a preparar esta recomendação, um guia de tratamento adaptado à nossa realidade. A escolha dos tópicos ou questões relacionadas às mudanças mais significativas nos conceitos e, consequentemente, no manejo da asma na prática clínica foi realizada por um painel de especialistas. Foi solicitado a cada especialista que revisasse criticamente um tópico ou respondesse a uma pergunta, com base em evidências, para estas recomendações. Numa segunda fase, três especialistas discutiram e estruturaram todos os textos submetidos pelos demais e, na última fase, todos revisaram e discutiram cada recomendação. As presentes recomendações se aplicam a adultos e crianças com asma e destinam-se a médicos envolvidos no tratamento da doença.
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Humanos , Asma/tratamiento farmacológico , Corticoesteroides/administración & dosificación , Antiasmáticos/administración & dosificación , Manejo de la Enfermedad , Índice de Severidad de la Enfermedad , Administración por Inhalación , Brasil , Factores de Riesgo , Factores de Edad , Brote de los SíntomasRESUMEN
Este documento é uma revisão do protocolo de asma grave da SMPCT de 2015, que se fez necessária devido à atualização de avanços em pesquisas, principalmente em fenotipagem/genotipagem e terapêutica da asma grave, além de asma grave na pediatria. A maioria da publicações relata que 5% a 10% dos asmáticos podem apresentar asma grave. Porém, levantamento na Holanda encontrou uma prevalência menor, de 3,6% ou 10,4/10000 habitantes, que parece ser mais próximo da realidade. Este protocolo tem como população alvo os pacientes com asma grave, adultos e pediátricos, conforme definições de asma grave da"International ERS/ATS guidelines on definition, evaluation and treatment of severe asthma" de 2014 e GINA 2018.1,3 Seus potenciais utilizadores são especialistas em doenças respiratórias que lidam com asma grave, e que devem ser os responsáveis pela aplicação do protocolo, e também clínicos gerais, pediatras, médicos de cuidados primários, enfermeiros, fisioterapeutas e outros profissionais da saúde. É aconselhável consulta com um especialista em asma nos seguintes casos: asma de difícil diagnóstico, suspeita de asma ocupacional, asma persistente não controlada com exacerbações frequentes, asma com risco de morte, eventos adversos significativos ou suspeita de subtipos de asma grave.4 Este documento não tem a intenção de instituir um tratamento padronizado, mas estabelecer bases racionais para decisões em pacientes com asma grave, pois as recomendações não conseguem abranger toda a complexidade do julgamento clínico em casos individuais. Os autores recomendam sua revisão e atualização no período máximo de 3 anos, ou, se necessário, em tempo menor.