Carla Nogueira

Diagnosis of sarcoidosis in the Endobronchial Ultrasound-guided Transbronchial Needle Aspiration era

BACKGROUND Sarcoidosis is a multisystemic disorder of unknown etiology. Its diagnosis is based on compatible clinical and radiological features and supported by histological demonstration of epithelioid cell noncaseating granulomas with exclusion of other causes. Endobronchial ultrasound combined with transbronchial needle aspiration (EBUS-TBNA) has been proposed as a valuable tool in obtaining suitable tissue sample. The aim of this study was to evaluate the contribution of EBUS-TBNA to the diagnosis of stages I and II thoracic sarcoidosis in a community-based hospital. METHODS A prospective study was conducted in patients with suspected stages I and II pulmonary sarcoidosis, based on clinical and radiological data, who were being followed in our Interstitial Lung Disorders Outpatient Clinic or sent from other hospitals to our Respiratory Endoscopy Unit for diagnostic procedures. All suitable and fit patients underwent EBUS-TBNA between March 2010 and June 2013. We assessed demographic characteristics, radiological stages, cytological/histological examination and diagnostic techniques performed. RESULTS In the period considered 39 patients underwent EBUS-TBNA for suspected stages I and II thoracic sarcoidosis and adequate samples were obtained in 38 (97.4%). Within this population, 33 (84.6%) patients had a definite diagnosis of sarcoidosis, of which 31 patients (93.9%) were confirmed to have epithelioid noncaseating granulomas by EBUS-TBNA. Four patients were submitted to surgical procedures (three to mediastinoscopy and one to open surgical lung biopsy). Data analysis allowed to calculate a sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy of 93.9%, 100%, 100%, 75.0% and 94.8%, respectively. No complications were observed. CONCLUSIONS EBUS-TBNA is a valuable tool in the diagnostic workup of patients with suspected stages I and II thoracic sarcoidosis providing a substantial number of pathological confirmations and with few complications. Its high diagnostic accuracy precludes the need for more invasive procedures such as surgical biopsy.

Ventilação não invasiva

Resumo A ventilacao nao invasiva (VNI) consiste na aplicacao de um suporte ventilatorio sem recorrer a metodos invasivos, evitando desta forma as complicacoes associadas a entubacao orotraqueal e ventilacao mecânica invasiva. Esta tecnica ja demonstrou ser eficaz em diversos tipos de insuficiencia respiratoria aguda ou cronica agudizada. Neste artigo, os autores fazem uma revisao dos beneficios, vantagens e limitacoes da VNI, interfaces utilizadas e principais indicacoes desta tecnica na insuficiencia respiratoria aguda (IRA) e na insuficiencia respiratoria cronica agudizada.

Bronchoscopic hemostatic tamponade with oxidized regenerated cellulose for major hemoptysis control: two case reports

Hemoptysis is a common and alarming clinical problem. Acute massive hemoptysis is a life threatening condition. Different therapeutic strategies such as surgery, endovascular treatment and/or bronchoscopy have been applied. We report two cases of patients with severe hemoptysis who were treated by bronchoscopy guided topical hemostatic tamponade therapy with oxidized regenerated cellulose.

Tamponamento hemostático por broncoscopia com aplicação de celulose oxidada regenerada no controlo de hemoptises graves — a propósito de dois casos clínicos

Hemoptysis is a common and alarming clinical problem. Acute massive hemoptysis is a life threatening condition. Different therapeutic strategies such as surgery, endovascular treatment and/or bronchoscopy have been applied. We report two cases of patients with severe hemoptysis who were treated by bronchoscopy guided topical hemostatic tamponade therapy with oxidized regenerated cellulose.

Técnicas de dilatação broncoscópica e aplicação tópica de mitomicina C no tratamento da estenose traqueal pós-entubação – A propósito de dois casos clínicos

Resumo A estenose traqueal surge na sequencia de agressoes a mucosa traqueal (isquemicas, traumaticas ou outras). O tratamento das estenoses traqueais continua a representar um desafio em termos de tecnicas endoscopicas e cirurgia, apesar de todos os recentes avancos nesta area. O processo de cicatrizacao e a reestenose sao as principais causas de falencia terapeutica. Os autores apresentam dois casos clinicos de doentes com estenose traqueal como consequencia de entuba cao orotraqueal prolongada/traqueostomia, em que o tratamento foi bem sucedido com dilatacao com broncoscopia rigida e laser , complementada por aplicacao topica de mitomicina C. Rev Port Pneumol 2010; XVI (1): 149-156

Quilotórax: A propósito de um caso clínico

Resumo O quilotorax caracteriza-se pela presenca de linfa no espaco pleural devido a lesao ou obstrucao do ducto toracico. O diagnostico e feito com base no elevado conteudo de trigliceridos e na presenca de quilomicrons. A etiologia do quilotorax pode ser dividida em traumatica e nao traumatica. A ruptura traumatica ocorre apos acidentes ou cirurgias. Das causas nao traumaticas, a mais comum e o linfoma e, na presenca de um quilotorax de etiologia desconhecida, a primeira suspeita diagnostica devera ser dirigida para esta entidade, sendo o tipo mais frequente o nao Hodgkin. Os autores apresentam o caso de um doente com quilotorax, cujo diagnostico etiologico foi de linfoma nao Hodgkin, discutem os aspectos particulares do quilotorax, bem como a investigacao e modalidades de tratamento desta entidade.

Not yet known side effects of pirfenidone in the treatment of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive fibrosing pneumonia of unknown cause, occurring primarily in older adults and limited to the lungs,1,2 with a very poor prognosis. There were no therapeutic options with proven efficacy until recent randomized controlled trials have shown the efficacy of pirfenidone and nintedanib in reducing the decline rate of forced vital capacity (FVC).3,4 Like any other recently introduced drug, the whole spectrum of the side effects of pirfenidone is still unknown. The most frequent side effects reported from the CAPACITY and ASCEND trials,3,5 the follow-up extension study RECAP6 and PASSPORT registry interim results,7 are gastrointestinal (nausea, diarrhea, dyspepsia and vomiting), cutaneous (rash, photosensitivity), headache, dizziness and fatigue. Although cough, upper airway tract infection and worsening of IPF were also reported, these could be attributed to the pathophysiology of IPF per se. We report the cases of 2 patients diagnosed with IPF according to the American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society/ALAT consensus statement on IPF1 and the 2013 ATS/ERS update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias statements,2 who experienced probable toxicity induced by pirfenidone. The first case reports a 62-year-old female non-smoker diagnosed with IPF (Possible UIP HRCT and UIP pattern in surgical lung biopsy) in 03-2011, being started on Nacetylcisteine 600 mg tid. The patient was started on pirfenidone in 05-2014; at that time a mild restriction was observed (FVC: 75%, 1760 mL, TLCO: 76.3%). From the time she was started on pirfenidone, she complained of dyspepsia, increasing dyspnea (modified Medical Research Council dyspnea scale [mMRC] 2--3), cough and wheezing. On phys-

Extracorporeal membrane oxygenator venovenous in treatment of a fulminant varicella pneumonia in an adult

Introduction: Varicella pneumonia is the most frequent and severe complication of varicella infection in adults, associated with a mortality of 30%. Case Report: A 46-year-old female with ulcerative colitis that starts fever, increasing dyspnea and a generalized rash with vesicles two days after admission. Laboratory tests showed renal impairment, acute hepatitis, a highly inflammatory state and chest radiography shows bilateral shadows with a rapid clinical deteriorating despite the anticipated treatment with antibiotic. 24-hours after the first symptoms, she develops an acute respiratory distress syndrome and was admitted to the intensive unit care and initiated protective lung ventilation. Despite acyclovir treatment and ventilator strategy, she maintained severe hypoxemia and need to started extracorporeal membrane oxygenator venovenous (ECMO-vv). The lavage fluid polymerase-chain reaction and vesicular liquid returned highly positive for varicella zoster virus (VZV) and completed treatment for seven days. The patient maintained ECMO-vv during nine days, was extubated after 13 days of mechanical ventilation, with all dysfunctions resolved. Conclusion: We can conclude that systemic varicella zoster virus infection, although rare, can cause serious and fatal complications. Early detection and proper treatment is mandatory for minimizing mortality and controlling infection, but often difficult because typical dermatological findings and the elevation of specific antibodies can be absent in immunosuppressed patients. (This page in not part of the published article.) International Journal of Case Reports and Images, Vol. 8 No. 7, July 2017. ISSN – [0976-3198] Int J Case Rep Images 2017;8(7):466–469. www.ijcasereportsandimages.com Neto et al. 466 CASE REPORT PEER REVIEWED | OPEN ACCESS Extracorporeal membrane oxygenator venovenous in treatment of a fulminant varicella pneumonia in an adult Rita Rei Neto, Sara Ferraz, Petra Monteiro, Margarida Correia, Carla Nogueira, Paula Castelões

Interstitial lung disease in the intensive unit care setting

t w c a c Interstitial lung diseases (ILD) are a heterogeneous group of disorders.1 Although the disease remains stable in some patients, episodes of acute respiratory failure (ARF) requiring invasive mechanical ventilation (IMV) are observed.2 Acute respiratory failure is often the result of disease progression or an acute exacerbation, but occasionally occurs as an inaugural manifestation or as an adverse reaction to treatment.1,3 We conducted a retrospective cohort study including patients admitted into ICU, with previously known ILD diagnosis and as an inaugural event, between January 2004 and May 2015, in order to evaluate the clinical outcome, overall survival and prognostic factors of ILD patients in the ICU setting. Thirty seven patients were included, 27 (73%) were male. Mean age of 65.1 ± 11.1 years (min: 27; max: 83). Thirty (81.1%) patients were admitted with previous ILD diagnosis. The diagnoses of the patients admitted in ICU were: 5 (13.5%) idiopathic pulmonary fibrosis (IPF); 5 (13.5%) silicosis; 5 (13.5%) fibrotic unclassifiable ILD; 5 (13.5%) small vessel vasculitis (ANCA +); 4 (10.8%) chronic hypersensitivity pneumonitis; 3 (8.2%) sarcoidosis; 2 (5.5%) rheumatoid lung (RL); 2 (5.5%) acute interstitial pneumonitis; 2 (5.5%) unclear ILD; 2 (5.5%) cryptogenic organizing pneumonia (COP); 1 (2.7%) scleroderma lung and 1 (2.7%) dermatomyositis. Seventeen had received previous therapy with corticosteroids, 8 immunosuppressant therapy and 10 long term oxygen therapy. The median length of ICU and hospital stay were 10 days (min: 1; max: 64) and 21 days (min:1; max:100) respectively. Mean APACHE II score was 18.3 ± 7 and mean SAPS II was 36.9 ± 11.5. Four lung biopsies were performed: 1 surgical lung biopsy and 3 core needle biopsies guided by CT scan. Two of the biopsies were consistent with acute interstitial pneumonitis, 1 with COP and 1 was unclear. Seventeen (45.9%) patients had acute exacerbation and

Esclerose tuberosa com envolvimento pulmonar

Resumo A esclerose tuberosa (ET) e uma doenca rara, esporadica ou transmitida de forma autossomica dominante. Caracteriza-se pela triade convulsoes, atraso mental e angiofibromas faciais. O envolvimento pulmonar e raro e, quando ocorre, e mais frequente no sexo feminino. Os autores apresentam o caso de uma doente de 52 anos, nao fumadora, com antecedentes conhecidos de epilepsia na infância e angiomiolipomas renais. Assintomatica e sem alteracoes ao exame objectivo. Em tomografia do torax realizada para avaliacao da doenca, foram detectadas formacoes microquisticas dispersas em ambos os campos pulmonares. Exame funcional respiratorio normal. A ressonância magnetica cerebral evidenciou tuberosidades corticais e nodulos subependimarios calcificados. Concluiu-se pelo diagnostico de ET (linfangioleiomiomatose, tuberosidades corticais, nodulos subependimarios e angiomiolipomas renais). Os autores apresentam o caso pela raridade da doenca e do envolvimento pulmonar, ainda que em fase assintomatica.