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Introduction: Transbronchial lung cryobiopsy (TBLC) is increasingly used in the diagnosis of diffuse lung disease (DLD), but no data have yet been published on the learning curve associated with this technique. Aim: To evaluate diagnostic yield, lung tissue sample length and area, and procedure-related complications in a cohort of TBLC procedures to define the learning curve and threshold for proficiency. Methods: Retrospective analysis of the first 100 TBLCs performed in different segments of the same lobe in patients with suspected DLD. We compared diagnostic yield, sample length and area, and complications between consecutive groups of patients. Results: The overall diagnostic yield for TBLC was 82%. Median sample length was 5.4 mm (IQR, 5–6) and median area was 19.5 mm2 (IQR, 13.3–25). Pneumothorax was the most common complication (18%). On comparing the two groups of 50 consecutive patients, a significant difference was found for diagnostic yield (74% vs 90%; p = 0.04), sample length (5.0 mm [2.5–16] vs 6.0 mm [4–12;] p

Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Epidemiological studies of different populations are essential because clinical presentation, organ involvement, disease severity, and prognosis vary significantly according to region and population. The aim of this study was to assess epidemiological and clinical characteristics, staging factors, and clinical course in patients with sarcoidosis from a tertiary hospital in Oporto, Portugal. Methods: A retrospective analysis of patients with sarcoidosis and at least 2 years of follow-up evaluated at the Centro Hospitalar de São João between 2000 and 2014. Results: We identified 409 patients with sarcoidosis (females, 58.9%; mean age at diagnosis, 38.9 ± 13.4 years; smokers, 14.4%]. All the patients were diagnosed according to the ERS/ATS/WASOG consensus statement and 64.1% had evidence of noncaseating epithelioid cell granulomas in biopsy specimens. Bronchoalveolar lavage was performed as part of the diagnostic work-up in 289 patients and 90.2% had lymphocytosis (CD4/CD8 ratio â¥Â 3.5 in 60.9% of cases). Exertion dyspnea, cough, and constitutional symptoms were the most common presenting symptoms; 10.1% of patients were asymptomatic, 22.8% had Löfgren syndrome, and 50.5% had extrathoracic involvement. Radiographic stages of disease according to the Scadding criteria were as follows: stage 0 (5.2%), stage I (33.7%), stage II (47.0%), stage III (8.4%), and stage IV (5.7%). Impaired respiratory function was observed in 45.6% patients and was mostly mild. Systemic treatment was administered in 58.6% of cases. Overall, 45.3% of patients experienced disease resolution. Conclusion: The epidemiological and clinical characteristics of this cohort of patients with sarcoidosis from the Oporto region in northern Portugal revealed epidemiological and clinical characteristics that were generally similar to those described in other Western Europe populations and in the US ACCESS study. However, we found a higher proportion of patients who progressed to chronic forms. Keywords: Sarcoidosis, Oporto, Löfgren syndrome, Extrathoracic involvement, Scadding criteria

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described rare entity, characterized by pleural and subpleural parenchymal fibrosis and elastosis mainly in the upper lobes. The etiology and pathophysiology are unknown. The prognosis is poor, with no effective therapies other than lung transplantation. IPPFE should be properly identified so that it can be approached correctly. This report describes two clinical cases with clinical imaging and histological features compatible with IPPFE. Keywords: Interstitial lung disease, Pleuroparenchymal fibrosis, Fibroelastosis

Resumo: A incidência de neoplasia associada à gravidez é relativamente baixa, podendo atingir cerca de 0,02-0,1% de todas as gestações. Os autores descrevem o caso de uma gestante de 36 anos de idade, fumadora, admitida no hospital às 27 semanas de gestação com sintomas respiratórios desde o segundo trimestre. A telerradiografia do tórax mostrou opacidade total do pulmão esquerdo, com desvio contralateral do mediastino, sugestiva de derrame pleural e a biópsia pleural revelou invasão por adenocarcinoma pulmonar. A pesquisa da mutação EGFR foi negativa. Após reunião multidisciplinar, decidiu-se iniciar a maturação pulmonar fetal e cesariana às 29 semanas de gestação. A doente realizou 2 linhas de quimioterapia bem como radioterapia óssea paliativa, verificando-se progressão da doença. A translocação EML4-ALK foi identificada num teste genético adicional. Foi iniciado crizotinib 250 mg 2 x dia. A doente apresentou uma sobrevida livre de progressão de 9 meses e faleceu 19 meses após o diagnóstico. Abstract: The incidence of pregnancy-associated cancer is relatively low, complicating only 0.02-0.1% of all pregnancies. The authors describe a case of a 36-year-old woman, a light smoker, who was admitted to the hospital at 27 weeks of pregnancy, with respiratory symptoms since second trimester. Chest-X ray showed total left lung opacity with contralateral mediastinal deviation, suggestive of pleural effusion, and the pleural biopsy revealed invasion by lung adenocarcinoma. EGFR mutation test was negative. After a multidisciplinary meeting, it was decided to start fetal lung maturation and cesarean section at 29 weeks gestation. The patient received two lines of chemotherapy and bone metastasis radiotherapy, but there was progression of the disease. An EML4-ALK translocation was identified in an additional genetic test. Crizotinib 250 mg BID was started. The patient showed a progression-free survival of 9 months and died 19 months after lung adenocarcinoma was diagnosed. Palavras-chave: Gravidez, Cancro do pulmão, Tratamento, EML4-AKL, Crizotinib, Keywords: Pregnancy, Lung cancer, Treatment, EML4-AKL, Crizotinib

Resumo: Introdução: Os agentes sedativos têm vindo a ser cada vez mais utilizados na broncofibroscopia (BF) para melhorar o conforto do doente. Devido à sua rápida ação, propriedades ansiolíticas e amnésicas, o midazolam é um dos sedativos mais frequentemente usados. Objetivos: Avaliar o efeito da sedação com midazolam na BF, incluindo a tolerância do doente, complicações e a sua potencial aplicação na prática clínica diária. Material e métodos: Estudo multicêntrico, prospetivo, randomizado, controlado com placebo, com inclusão de 100 indivíduos submetidos a BF em 2 Serviços de Pneumologia. Doentes do Grupo 1 receberam midazolam (0,05 mg/kg) e doentes do Grupo 2 receberam placebo (0,9% NaCl), 5 minutos antes do procedimento. A escala de ansiedade «The Hospital Anxiety and Depression Scale» (HADS-A) foi aplicada para determinar o nível de ansiedade basal do doente. Questionários subjetivos acerca dos principais receios e queixas relativamente à BF foram aplicados antes e depois do exame. Resultados: Média de idades de 56,0 ± 14,1 anos; 66% do sexo masculino. A maioria (65%) dos doentes apresentava baixa pontuação ( 0,05) e à dor (4 vs 12%; p > 0,05). Foi demonstrada recetibilidade em repetir o exame por todos os doentes do Grupo 1 e em 82% dos doentes do Grupo 2 (p = 0,003). Conclusão: A sedação com midazolam na BF aumentou o conforto e diminuiu queixas dos doentes, não se verificando alterações hemodinâmicas significativas. Deve ser pois oferecida, de forma regular, ao doente submetido a BF. Abstract: Introduction: Sedatives have been increasingly used to improve patient comfort during flexible bronchoscopy (FOB). Due to its rapid-onset, anxiolytic and amnestic properties, midazolam is one of the most commonly used sedatives. Objectives: To evaluate the effect of sedation with midazolam, including patient tolerance, complications and its potential use on a daily routine basis. Material and methods: A multi-centre, prospective, randomized, placebo-controlled study was made on 100 patients submitted to FOB in two Pulmonology Departments. Midazolam (0.05 mg/kg) was administered to patients in Group 1 and saline solution (0,9% NaCl) to patients in Group 2, five minutes before the procedure. The Hospital Anxiety and Depression Scale (HADS-A) was used to determine patient anxiety level. Subjective questionnaires concerning main fears and complaints were answered before and after FOB. Results: Mean age was 56.0 ± 14.1 years; 66% male. Most (65%) patients had low score (0.05) and pain (4% vs 12%; p>0.05) were not statistically different. Willingness to repeat the exam was reported in all patients in Group 1 and in 82% in Group 2 (p=0.003). Conclusion: Sedation with midazolam in FOB improved patient's comfort and decreased complaints, without significant haemodynamic changes. It should be offered to the patient on a routine basis. Palavras-chave: Sedação, Midazolam, Broncofibroscopia, Keywords: Sedation, Midazolam, Bronchoscopy