Abstract Introduction: patients with granulomatosis with polyangiitis (GPA) may present upper airway (UA) and lower airway (LA) involvement. Objectives: we describe the endoscopic manifestations of the airways, histological findings from biopsied tissue and antineutrophilic cytoplasm antibody (ANCA) in a group of patients with GPA. Methods: retrospective study of medical records of patients with GPA undergoing bronchoscopy between 2012 and 2019. Airway findings, results of biopsies performed and ANCA results were analyzed. Results: 40 patients were included, with a mean age of 46.92±17.61 years and predominantly female (67.5%). Airway involvement was observed in 90% (n=36). The C-ANCA was reactive in 63.9%, P-ANCA in 25%, doubly reactive ANCA in 8.33% and non-reactive in 20%. The findings in upper airway (UA) were: chronic sinusitis (41.7%), destruction of the nasal septum (16.7%); and in lower airway (LA) were: tracheobronchial stenosis (38.9%) and tracheobronchitis (25%). The pathological findings most common of bronchoscopic biopsies were: polymorphonuclear inflammatory process (61.9%) and geographic necrosis (47.6%). Conclusion: the airway is involved in up to 90% of patients with GPA. Non-reactive ANCA does not rule out this possibility. Chronic sinusitis and tracheobronchial fibrostenotic processes were the most common endoscopic findings. Vasculitis in biopsies was found in a minority of cases.

Resumen Introducción: los pacientes con granulomatosis con poliangitis (GPA) pueden presentar compromiso de la vía aérea superior (VAS) o inferior (VAI). Objetivos: describimos las manifestaciones endoscópicas de las vías respiratorias, los hallazgos histológicos y los anticuerpos anticitoplasma de neutrófilos (ANCA) en un grupo de pacientes con GPA. Métodos: estudio retrospectivo de historias clínicas de pacientes con GPA sometidos a broncoscopia entre 2012 y 2019. Se analizaron hallazgos de la vía aérea, biopsias y ANCA. Resultados: se incluyeron 40 pacientes, con una edad media de 46,92±17,61 años, predominantemente del sexo femenino (67,5%). Se observó afectación de la vía aérea en el 90% (n=36). El C-ANCA fue reactivo en el 63,9%, P-ANCA en el 25%, ANCA doblemente reactivo en el 8,33% y no reactivo en el 20%. Los hallazgos comunes en la vía aérea superior (VS) fueron sinusitis crónica (41,7%), destrucción del tabique nasal (16,7%); y en la vía aérea inferior (AI): estenosis traqueobronquial (38,9%), traqueobronquitis (25%). Los hallazgos más frecuentes de las biopsias broncoscópicas fueron proceso inflamatorio polimorfonuclear (61,9%) y necrosis geográfica (47,6%). Conclusión: la vía aérea está comprometida hasta en un 90% de los pacientes con GPA. ANCA no reactivos no descartan esta posibilidad. La sinusitis crónica y los procesos fibroestenóticos traqueobronquiales fueron los hallazgos endoscópicos más comunes. La vasculitis en biopsias se encontró en una minoría de casos.

RESUMO OBJETIVOS A embolia pulmonar aguda (EAP) é uma causa importante de mortalidade cardiovascular ao causar instabilidade hemodinâmica. Nesses casos, a recomendação é a realização de algum procedimento de reperfusão, sendo a trombólise sistêmica a principal terapia utilizada. No entanto, dados nacionais avaliando a eficácia e a segurança da trombólise são escassos. MÉTODO Análise retrospectiva de uma série de casos. Foram incluídos 13 pacientes com o diagnóstico de EAP de alto risco e quatro pacientes de risco intermediário-alto, de um único centro, e que foram tratados com alteplase 100 mg. RESULTADOS A média de idade dos pacientes foi 55 anos, sendo a maioria do gênero feminino (76,4%). Dos fatores de risco para TEV, estavam presentes a imobilização (41,17%), o uso de anticonceptivos (35,29%), câncer (17,63%) e história prévia de TVP (11,76%). As manifestações clínicas mais frequentes da EAP foram dispneia (88,23%), hipóxia (82,35%), hipotensão (82,35%) e taquicardia (64,70%); 82,35% dos pacientes apresentaram sinais ecocardiográficos de disfunção ventricular direita e 52,94% apresentaram aumento da troponina e BNP. Sangramento grave associado à trombólise ocorreu em 17,54% dos casos. Nenhum paciente faleceu em decorrência de sangramento. Houve oito mortes por insuficiência ventricular direita (47%): seis nos casos de paciente que se apresentaram como EAP de alto risco (35,3%) e duas nos casos de risco intermediário-alto (11,8%). CONCLUSÃO A trombólise nos pacientes com EAP de alto risco ou risco intermediário-alto apresentou uma taxa de sangramento grave de 17,6%. No entanto, a alta mortalidade dessa população (47%) por insuficiência ventricular direita justifica o uso desta modalidade terapêutica.

SUMMARY OBJECTIVES Acute pulmonary embolism (APE) is an important cause of cardiovascular mortality, due mainly to hemodynamic instability. In these cases, the recommendation is to perform some reperfusion procedure, with systemic thrombolysis being the main therapy used. However, national data evaluating the efficacy and safety of thrombolysis are scarce. METHODS Retrospective analysis of a case series. We included 13 patients diagnosed with high-risk APE and 4 patients with intermediate-high risk from a single-center, who were treated with alteplase 100mg. RESULTS The mean age of the patients was 55 years, most of them female (76.4%). Among the risk factors for VTE were immobilization (41.17%), contraceptive use (35.29%), cancer (17.63%), and previous history of DVT (11.76%). The most frequent clinical manifestations of APE were dyspnea (88.23%), hypoxia (82.35%), hypotension (82.35%), and tachycardia (64.70%). 82.35% of the patients had echocardiographic signs of right ventricular dysfunction, and 52.94% had increased troponin and BNP. Severe bleeding associated with thrombolysis occurred in 17.54% of cases. No patient died due to bleeding. There were 8 deaths from right ventricular failure (47%), 6 in the cases of patients presenting as high-risk APE (35.3%), and 2 in the cases of intermediate-high risk (11.8%). CONCLUSION Thrombolysis in patients with high-risk APE or intermediate-high risk had a severe bleeding rate of 17.6%. However, the high mortality of this population (47%) due to right ventricular failure justifies the use of this therapeutic modality.

RESUMO Inicialmente os cuidados paliativos foram desenvolvidos para pacientes com câncer avançado. Hoje este conceito evoluiu e engloba qualquer doença crônica que ameace a vida. Estudos realizados para comparar os sintomas de fim de vida mostraram que os pacientes pneumológicos apresentavam a mesma prevalência de sintomas, como dor e dispneia, porém recebiam menos tratamento paliativo que os pacientes oncológicos. É preciso desmitificar a ideia de que cuidados paliativos só devem ser adotados quando não há mais possibilidade de tratamento curativo. O cuidado paliativo deve ser associado ao tratamento curativo no momento do diagnóstico, por meio de decisão compartilhada, isto é, paciente e médico planejam as decisões sobre a terapêutica a ser tomada, buscam a melhora na qualidade de vida e a redução do sofrimento, tanto espiritual como físico e psicológico.

ABSTRACT Palliative care was initially developed for patients with advanced cancer. The concept has evolved and now encompasses any life-threatening chronic disease. Studies carried out to compare end-of-life symptoms have shown that although symptoms such as pain and dyspnea are as prevalent in patients with lung disease as in patients with cancer, the former receive less palliative treatment than do the latter. There is a need to refute the idea that palliative care should be adopted only when curative treatment is no longer possible. Palliative care should be provided in conjunction with curative treatment at the time of diagnosis, by means of a joint decision-making process; that is, the patient and the physician should work together to plan the therapy, seeking to improve quality of life while reducing physical, psychological, and spiritual suffering.

OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients. METHODS: A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016. RESULTS: Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified. CONCLUSION: Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.

RESUMO Objetivo: Apresentar uma série de casos de malformações arteriovenosas pulmonares (MAVP) e descrever os principais achados clínicos, a quantidade e localização das MAVP, as complicações clínicas e os tratamentos realizados. Métodos: Estudo retrospectivo observacional que avaliou pacientes com MAVP divididos em dois grupos: telangiectasia hemorrágica hereditária (THH) e MAVP idiopática (MAVPi). Resultados: Foram avaliados 41 pacientes, sendo 33 pacientes portadores de MAVP. Após a avaliação clínica, 27 e 6 foram diagnosticados com THH e MAVPi, respectivamente. No grupo THH a média de idade foi de 49,6 anos e 88,9% eram do sexo feminino. Desses pacientes, 4 tinham SpO2 < 90% e o achado clínico mais frequente era epistaxe. No grupo MAVPi a média de idade foi de 48,1 anos, sendo que 83,3% eram do sexo feminino. Desses, 3 tinham SpO2 < 90%. Após a realização de angiotomografia de tórax observou-se que a maior parte das MAVP se situava nos lobos inferiores, totalizando 56,4% e 85,7% nos grupos THH e MAVPi, respectivamente. O tratamento por embolização foi realizado em 23 pacientes nos dois grupos, enquanto 10 aguardavam o procedimento até o momento da escrita deste estudo. Um paciente submetido à embolização foi encaminhado para ressecção pulmonar. Conclusões: Em ambos os grupos de pacientes com MAVP observou-se uma predominância de mulheres e de fístulas localizadas nos lobos inferiores. A maioria era assintomático respiratório com SpO2 > 90%. O tratamento de escolha para todos foi a embolização percutânea por cateter.

ABSTRACT Objective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.