2022 Brazilian Thoracic Association recommendations for long-term home oxygen therapy.

Some chronic respiratory diseases can cause hypoxemia and, in such cases, long-term home oxygen therapy (LTOT) is indicated as a treatment option primarily to improve patient quality of life and life expectancy. Home oxygen has been used for more than 70 years, and support for LTOT is based on two studies from the 1980s that demonstrated that oxygen use improves survival in patients with COPD. There is evidence that LTOT has other beneficial effects such as improved cognitive function, improved exercise capacity, and reduced hospitalizations. LTOT is indicated in other respiratory diseases that cause hypoxemia, on the basis of the same criteria as those used for COPD. There has been an increase in the use of LTOT, probably because of increased life expectancy and a higher prevalence of chronic respiratory diseases, as well as greater availability of LTOT in the health care system. The first Brazilian Thoracic Association consensus statement on LTOT was published in 2000. Twenty-two years later, we present this updated version. This document is a nonsystematic review of the literature, conducted by pulmonologists who evaluated scientific evidence and international guidelines on LTOT in the various diseases that cause hypoxemia and in specific situations (i.e., exercise, sleep, and air travel). These recommendations, produced with a view to clinical practice, contain several charts with information on indications for LTOT, oxygen sources, accessories, strategies for improved efficiency and effectiveness, and recommendations for the safe use of LTOT, as well as a LTOT prescribing model.

Persistence of symptoms and return to work after hospitalization for COVID-19.

Many patients hospitalized with COVID-19 were unable to return to work or their return was delayed due to their health condition. The aim of this observational study was to evaluate the impact of moderate-to-severe and critical COVID-19 infection on persistence of symptoms and return to work after hospital discharge. In this study, two thirds of hospitalized patients with pulmonary involvement reported persistence of symptoms six months after COVID-19 infection, such as memory loss (45.5%), myalgia (43.9%), fatigue (39.4%), and dyspnea (25.8%), and 50% slowly returned to work, with repercussions due to fatigue and/or loss of energy.

Clinical, radiological, and transbronchial biopsy findings in patients with long COVID-19: a case series.

This brief communication demonstrates the correlation of persistent respiratory symptoms with functional, tomographic, and transbronchial pulmonary biopsy findings in patients with COVID-19 who had a long follow-up period. We report a series of six COVID-19 patients with pulmonary involvement who presented with persistent dyspnea within 4-15 months of discharge. We performed transbronchial biopsies, and the histopathological pattern consistently demonstrated peribronchial remodeling with interstitial pulmonary fibrosis. Therefore, lung biopsy may be useful in the approach of patients with long COVID-19, although the type of procedure, its precise indication, and the moment to perform it are yet to be clarified. (Brazilian Registry of Clinical Trials-ReBEC; identifier: RBR-8j9kqy [http://www.ensaiosclinicos.gov.br]).

2022 Brazilian Thoracic Association recommendations for long-term home oxygen therapy / Recomendações para oxigenoterapia domiciliar prolongada da Sociedade Brasileira de Pneumologia e Tisiologia (2022)

ABSTRACT Some chronic respiratory diseases can cause hypoxemia and, in such cases, long-term home oxygen therapy (LTOT) is indicated as a treatment option primarily to improve patient quality of life and life expectancy. Home oxygen has been used for more than 70 years, and support for LTOT is based on two studies from the 1980s that demonstrated that oxygen use improves survival in patients with COPD. There is evidence that LTOT has other beneficial effects such as improved cognitive function, improved exercise capacity, and reduced hospitalizations. LTOT is indicated in other respiratory diseases that cause hypoxemia, on the basis of the same criteria as those used for COPD. There has been an increase in the use of LTOT, probably because of increased life expectancy and a higher prevalence of chronic respiratory diseases, as well as greater availability of LTOT in the health care system. The first Brazilian Thoracic Association consensus statement on LTOT was published in 2000. Twenty-two years later, we present this updated version. This document is a nonsystematic review of the literature, conducted by pulmonologists who evaluated scientific evidence and international guidelines on LTOT in the various diseases that cause hypoxemia and in specific situations (i.e., exercise, sleep, and air travel). These recommendations, produced with a view to clinical practice, contain several charts with information on indications for LTOT, oxygen sources, accessories, strategies for improved efficiency and effectiveness, and recommendations for the safe use of LTOT, as well as a LTOT prescribing model.

RESUMO Algumas doenças respiratórias crônicas podem evoluir com hipoxemia e, nessas situações, a oxigenoterapia domiciliar prolongada (ODP) está indicada como opção terapêutica com o objetivo principal de melhorar a qualidade e a expectativa de vida desses pacientes. O oxigênio domiciliar é usado há mais de 70 anos, e a ODP tem como base dois estudos da década de oitenta que demonstraram que o uso de oxigênio melhora a sobrevida de pacientes com DPOC. Existem evidências de que a ODP tem outros efeitos benéficos como melhora da função cognitiva e da capacidade de exercício e redução de hospitalizações. A ODP está indicada para outras doenças respiratórias que cursam com hipoxemia, segundo os mesmos critérios estabelecidos para a DPOC. Tem sido observado aumento no uso da ODP provavelmente pela maior expectativa de vida, maior prevalência de doenças respiratórias crônicas e maior disponibilidade de ODP no sistema de saúde. O primeiro consenso sobre ODP da Sociedade Brasileira de Pneumologia e Tisiologia foi publicado em 2000; após 22 anos, apresentamos esta versão atualizada. Este documento é uma revisão não sistemática da literatura, realizada por pneumologistas que avaliaram evidências científicas e diretrizes internacionais sobre ODP nas diversas doenças que cursam com hipoxemia e em situações específicas (exercício, sono e viagens aéreas). Estas recomendações, tendo em vista a prática clínica, oferecem diversos quadros com informações sobre indicações, fontes de oxigênio, acessórios e estratégias para melhor eficiência, efetividade e uso seguro da ODP, assim como um modelo para sua prescrição.

Persistence of symptoms and return to work after hospitalization for COVID-19 / Persistência de sintomas e retorno ao trabalho após hospitalização por COVID-19

ABSTRACT Many patients hospitalized with COVID-19 were unable to return to work or their return was delayed due to their health condition. The aim of this observational study was to evaluate the impact of moderate-to-severe and critical COVID-19 infection on persistence of symptoms and return to work after hospital discharge. In this study, two thirds of hospitalized patients with pulmonary involvement reported persistence of symptoms six months after COVID-19 infection, such as memory loss (45.5%), myalgia (43.9%), fatigue (39.4%), and dyspnea (25.8%), and 50% slowly returned to work, with repercussions due to fatigue and/or loss of energy.

RESUMO Muitos pacientes hospitalizados com COVID-19 não puderam retornar ao trabalho ou seu retorno foi atrasado devido ao seu estado de saúde. O objetivo deste estudo observacional foi avaliar o impacto da infecção moderada a grave ou crítica por COVID-19 na persistência dos sintomas e no retorno ao trabalho após a alta hospitalar. Neste estudo, dois terços dos pacientes hospitalizados com acometimento pulmonar relataram persistência dos sintomas seis meses após a infecção por COVID-19, como perda de memória (45,5%), mialgia (43,9%), fadiga (39,4%) e dispneia (25,8%), e 50% retornaram lentamente ao trabalho, com repercussões devido à fadiga e/ou perda de energia.

Clinical, radiological, and transbronchial biopsy findings in patients with long COVID-19: a case series / Achados clínicos, radiológicos e de biópsia transbrônquica em pacientes com COVID-19 prolongada: série de casos

ABSTRACT This brief communication demonstrates the correlation of persistent respiratory symptoms with functional, tomographic, and transbronchial pulmonary biopsy findings in patients with COVID-19 who had a long follow-up period. We report a series of six COVID-19 patients with pulmonary involvement who presented with persistent dyspnea within 4-15 months of discharge. We performed transbronchial biopsies, and the histopathological pattern consistently demonstrated peribronchial remodeling with interstitial pulmonary fibrosis. Therefore, lung biopsy may be useful in the approach of patients with long COVID-19, although the type of procedure, its precise indication, and the moment to perform it are yet to be clarified. (Brazilian Registry of Clinical Trials-ReBEC; identifier: RBR-8j9kqy [http://www.ensaiosclinicos.gov.br])

RESUMO Esta comunicação breve demonstra a correlação de sintomas respiratórios persistentes com achados funcionais, tomográficos e de biópsia pulmonar transbrônquica em pacientes com COVID-19 que tiveram um longo período de acompanhamento. Relatamos uma série de seis pacientes com COVID-19 com acometimento pulmonar que apresentavam dispneia persistente após 4-15 meses da alta. Realizamos biópsias transbrônquicas, e o padrão histopatológico consistentemente demonstrou remodelação peribrônquica com fibrose pulmonar intersticial. Portanto, a biópsia pulmonar pode ser útil na abordagem de pacientes com COVID-19 prolongada, embora o tipo de procedimento, suas indicações precisas e o momento de sua realização ainda não estejam esclarecidos. (Registro Brasileiro de Ensaios Clínicos - ReBEC; número de identificação: RBR-8j9kqy [http://www.ensaiosclinicos.gov.br])

Pulmonary fibrosis secondary to COVID-19: a narrative review.

Introduction: Coronavirus disease 2019 (COVID-19) is still increasing worldwide, and as a result, the number of patients with pulmonary fibrosis secondary to COVID-19 will expand over time. Risk factors, histopathological characterization, pathophysiology, prevalence, and management of post-COVID-19 pulmonary fibrosis are poorly understood, and few studies have addressed these issues.Areas covered:This article reviews the current evidence regarding post-COVID-19 pulmonary fibrosis, with an emphasis on the potential risk factors, histopathology, pathophysiology, functional and tomographic features, and potential therapeutic modalities. A search on the issue was performed in the MEDLINE, Embase, and SciELO databases and the Cochrane library between 1 December 2019, and 25 January 2021. Studies were reviewed and relevant topics were incorporated into this narrative review. Expert opinion: Pulmonary sequelae may occur secondary to COVID-19, which needs to be included as a potential etiology in the current differential diagnosis of pulmonary fibrosis. Therefore, serial clinical, tomographic, and functional screening for pulmonary fibrosis is recommended after COVID-19, mainly in patients with pulmonary involvement in the acute phase of the disease. Further studies are necessary to determine the risk factors, markers, pathophysiology, and appropriate management of post-COVID-19 pulmonary fibrosis.

Nailfold capillaroscopy as a risk factor for pulmonary arterial hypertension in systemic lupus erythematosus patients.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare and severe complication of systemic lupus erythematosus (SLE). This study aimed to evaluate clinical and laboratory risk factors associated with PAH in SLE patients. METHODS: This was a retrospective case-control study in which patients with SLE with PAH (SLE-PAH) confirmed by right heart catheterization (RHC) were compared with SLE patients without PAH. Clinical and demographic variables related to SLE and PAH and nailfold capillaroscopy were evaluated by reviewing the medical records of the patients. RESULTS: Twenty-one patients with SLE-PAH and 44 patients with SLE without PAH matched for sex and disease duration were included. The scleroderma (SD) pattern on nailfold capillaroscopy was more frequently found in patients with SLE-PAH than in those without PAH (56.3% versus 15.9%, respectively, p = 0.002). By univariate analysis, Raynaud's phenomenon, history of abortion, and SD pattern on capillaroscopy were associated with PAH. Arthritis was a protective factor for PAH development. Multivariate analysis showed that the SD pattern on capillaroscopy was the only variable associated with a significantly higher risk of PAH, with an odds ratio of 6.393 (95% confidence interval, 1.530-26.716; p = 0.011). CONCLUSION: In this study, SD pattern was associated with a 6.3-fold increased risk for PAH development in SLE patients, suggesting that nailfold capillaroscopy might be useful as a screening method to identify SLE patients with a high risk of developing this severe complication.

Nailfold capillaroscopy as a risk factor for pulmonary arterial hypertension in systemic lupus erythematosus patients

Abstract Background: Pulmonary arterial hypertension (PAH) is a rare and severe complication of systemic lupus erythematosus (SLE). This study aimed to evaluate clinical and laboratory risk factors associated with PAH in SLE patients. Methods: This was a retrospective case-control study in which patients with SLE with PAH (SLE-PAH) confirmed by right heart catheterization (RHC) were compared with SLE patients without PAH. Clinical and demographic variables related to SLE and PAH and nailfold capillaroscopy were evaluated by reviewing the medical records of the patients. Results: Twenty-one patients with SLE-PAH and 44 patients with SLE without PAH matched for sex and disease duration were included. The scleroderma (SD) pattern on nailfold capillaroscopy was more frequently found in patients with SLE-PAH than in those without PAH (56.3% versus 15.9%, respectively, p = 0.002). By univariate analysis, Raynaud's phenomenon, history of abortion, and SD pattern on capillaroscopy were associated with PAH. Arthritis was a protective factor for PAH development. Multivariate analysis showed that the SD pattern on capillaroscopy was the only variable associated with a significantly higher risk of PAH, with an odds ratio of 6.393 (95% confidence interval, 1.530-26.716; p = 0.011). Conclusion: In this study, SD pattern was associated with a 6.3-fold increased risk for PAH development in SLE patients, suggesting that nailfold capillaroscopy might be useful as a screening method to identify SLE patients with a high risk of developing this severe complication.

Estratégias do tratamento da hipertensão arterial pulmonar / Strategies treatment of pulmonary arterial

Grandes avanços têm sido observados no conhecimento da patogenia da Hipertensão Arterial Pulmonar. A vasoconstrição, a disfunção endotelial, a proliferação da camada íntima e a hiperplasia e/ou hipertrofia da camada média muscular, assim como a trombose in situ, são os principais mecanismos aceitos na gênese da HAP. Novas drogas surgiram nos últimos anos, mudando a evolução natural desta condição. Atualmente, existem 10 medicamentos autorizados pelas agências regulatórias mundiais de saúde para o tratamento da HAP: os derivados da prostaciclina (beraprost, epoprostenol, iloprost e treprostinil); os antagonistas dos receptores da endotelina (ambrisentana, bosentana e macitentan); os inibidores da fosfodiesterase-5 (sildenafil e tadalafila) e o estimulador da guanilato ciclase solúvel (riociguate). Resultados de metanálises e de estudos clínicos controlados e randomizados sugerem que estes medicamentos reduziram a mortalidade em aproximadamente 43% nos grupos tratados em relação ao placebo. Porém, numa coorte de pacientes com HAP incluídos no Registro Francês de Hipertensão Pulmonar no período de 2002 a 2003 e acompanhados por 3 anos, observou-se que a doença ainda é progressiva, ressaltando as limitações existentes no manejo atual do paciente com hipertensão pulmonar...

Great advances has been observed in understanding the pathogenesis of Pulmonary Arterial Hypertension. Vasoconstriction, endothelial dysfunction, proliferation and intimal hyperplasia and / or hypertrophy of muscle middle layer, and in situ thrombosis are major mechanisms in the accepted genesis of HAP. New drugs have emerged in recent years, changing the natural history of this condition. Currently, there are 10 drugs approved by worldwide regulatory health agencies for the treatment of PAH: derived prostacyclin (beraprost, epoprostenol, iloprost and treprostinil); antagonists of endothelin receptors (ambrisentan, bosentan and macitentan); phosphodiesterase-5 inhibitors (sildenafil and tadalafil) and the stimulator of soluble guanylate cyclase (riociguate). Results of metanalysis and randomized controlled trials suggest that these drugs have reduced mortality in about 43% in the treated groups compared to placebo groups. But a cohort of patients with PAH included in the French Registration Pulmonary Hypertension between 2002 and 2003 followed for 3 years, showed that the disease still is progressive, emphasizing the limitations in the current management of the patient with PAH....

Contrasting cardiopulmonary responses to incremental exercise in patients with schistosomiasis-associated and idiopathic pulmonary arterial hypertension with similar resting hemodynamic impairment.

It has been reported that schistosomiasis-associated PAH (Sch-PAH) has a more benign clinical course compared with idiopathic PAH (IPAH). We therefore hypothesized that Sch-PAH subjects would present with less impaired cardiopulmonary and metabolic responses to exercise than IPAH patients, even with similar resting pulmonary hemodynamic abnormalities. The aim of this study was to contrast physiologic responses to incremental exercise on cycle ergometer between subjects with Sch-PAH and IPAH. We performed incremental cardiopulmonary exercise tests (CPET) in subjects newly diagnosed with IPAH (n = 9) and Sch-PAH (n = 8), within 1 month of the hemodynamic study and before the initiation of specific therapy for PAH. There were no significant between-group differences in cardiac index, pulmonary vascular resistance or mean pulmonary artery pressure. However, mean peak oxygen uptake (VO2) was greater in Sch-PAH than IPAH patients (75.5±21.4 vs 54.1±16.1% predicted, p = 0.016), as well as the ratio of increase in VO2 to work rate (8.2±1.0 vs 6.8±1.8 mL/min/W, p = 0.03). Additionally, the slope of the ventilatory response as a function of CO2 output was lower in Sch-PAH (40.3±3.9 vs 55.6±19.8; p = 0.04), and the heart rate response for a given change in VO2 was also diminished in Sch-PAH compared to IPAH (80.1±20.6 vs 123.0±39.2 beats/L/min; p = 0.02). In conclusion, Sch-PAH patients had less impaired physiological responses to exercise than IPAH subjects with similar resting hemodynamic dysfunction. Our data suggest a more preserved cardiopulmonary response to exercise in Sch-PAH which might be related to its better clinical course compared to IPAH.

Benefícios e limitações do tratamento atual da hipertensao arterial pulmonar / Benefits and limitations about today's treatment of pulmonary arterial hypertension

Hipertensão arterial pulmonar (HAP) é uma doença rara causada pela proliferação vascular e remodelamento, resultando no aumento progressivo da resistência vascular pulmonar e disfunção ventricular direita. Apesar de recentes avanços terapêuticos, essa doença é ainda grave e rapidamente progressiva. Existem, atualmente, três classes principais de drogas que podem ser utilizadas para o tratamento da HAP: prostanoides, antagonistas dos receptores de endotelina e inibidores da fosfodiesterase-5. Nessa revisão, discutiremos o tratamento de suporte nessa população de doentes, assim como as drogas específicas atualmente disponíveis.

Recomendações para o manejo da tromboembolia pulmonar, 2010 / Recommendations for the management of pulmonary thromboembolism, 2010

A tromboembolia pulmonar constitui, juntamente com a trombose venosa profunda, a condição denominada tromboembolismo venoso. Apesar dos avanços, a morbidade e a mortalidade atribuídas a essa doença ainda são elevadas, pois os pacientes apresentam doenças mais complexas, são submetidos a um maior número de procedimentos invasivos e sobrevivem por mais tempo. Embora existam inúmeras diretrizes internacionais disponíveis, optou-se por redigir estas recomendações para sua aplicação na prática médica nacional, embasadas nas melhores evidências na literatura e na opinião do grupo de consultores. Este documento é apenas uma ferramenta para o atendimento dos pacientes, e, embora possa ser aplicado na maioria das situações, o médico deve adaptar as informações a sua realidade local e ao caso específico. O diagnóstico de tromboembolia pulmonar é realizado através da combinação da probabilidade clínica pré-teste (escores) com o resultado dos exames de imagem, sendo atualmente o método de eleição a angiotomografia computadorizada. É fundamental a estratificação do risco de desfecho desfavorável, sendo a instabilidade hemodinâmica o preditor mais importante. Pacientes de baixo risco devem ser tratados com heparina, comumente as de baixo peso molecular. Pacientes de alto risco requerem vigilância intensiva e uso de trombolíticos em alguns casos. A longo prazo, os pacientes devem receber anticoagulantes por no mínimo três meses, sendo sua manutenção decidida pela presença de fatores de risco para a recorrência e a probabilidade de sangramento. A profilaxia é altamente eficaz e deve ser amplamente utilizada, tanto em pacientes clínicos como cirúrgicos, conforme os grupos de risco. Finalmente, são feitas recomendações relacionadas ao diagnóstico, tratamento e prevenção da tromboembolia pulmonar.

Pulmonary thromboembolism and deep vein thrombosis together constitute a condition designated venous thromboembolism. Despite the advances, the morbidity and the mortality attributed to this condition are still high, because the patients present with more complex diseases, are submitted to a greater number of invasive procedures and survive longer. Although there are various international guidelines available, we decided to write these recommendations for their application in medical practice in Brazil. These recommendations are based on the best evidence in the literature and the opinion of the advisory committee. This document is only a tool for use in the management of patients. Although the recommendations it contains can be applied to most situations, physicians should adapt its content depending on their local context and on a case-by-case basis. Pulmonary thromboembolism is diagnosed by evaluating pre-test clinical probability (scores) together with the results of imaging studies, the current method of choice being CT angiography. Stratification of the risk for an unfavorable outcome is fundamental. Hemodynamic instability is the most important predictor. Low-risk patients should be treated with heparin, commonly low-molecular-weight heparins. High-risk patients require intensive monitoring and, in some cases, thrombolytic therapy. In the long term, patients should receive anticoagulants for at least three months. The decision to prolong this treatment is made based on the presence of risk factors for the recurrence of the condition and the probability of bleeding. Prophylaxis is highly effective and should be widely used in clinical and surgical patients alike, according to their risk group. Finally, we include recommendations regarding the prevention, diagnosis and treatment of pulmonary thromboembolism.

[Clinical characteristics of pulmonary hypertension patients in two reference centers in the city of Sao Paulo]. / Características clínicas dos pacientes com hipertensão pulmonar em dois centros de referência em São Paulo.

OBJECTIVES: Describe the clinical profile of PH patients from two pulmonary hypertension centers. METHODS: Retrospective chart analysis. RESULTS: One hundred and twenty three PH patients were included in the study; 62% of these presented functional class III or IV (NYHA). Mean right ventricle systolic pressure (RVSP) was 83.48+/-24.61 mmHg. There was no correlation between functional class and RVSP. About 50% of the patients were diagnosed as IPAH; 30% as pulmonary hypertension associated to schistosomiasis; 10% as PH associated to connective tissue diseases. Mean time of dyspnea until diagnosis was variable with no correlation with functional class (p>0.05). No difference was found in the clinical presentation in spite of the diverse etiologies. CONCLUSION: Based upon our findings, we stressed the need for an active investigation of PH patients prior to administration of any therapeutic alternative. We emphasized that a better understanding of PH related to schistosomiasis is needed due to the high prevalence of this condition among PH patients as shown in the Brazilian population.