Conceição Souto Moura

Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study

BACKGROUND Diffuse parenchymal lung disease represents a diverse and challenging group of pulmonary disorders. A consistent diagnostic approach to diffuse parenchymal lung disease is crucial if clinical trial data are to be applied to individual patients. We aimed to evaluate inter-multidisciplinary team agreement for the diagnosis of diffuse parenchymal lung disease. METHODS We did a multicentre evaluation of clinical data of patients who presented to the interstitial lung disease unit of the Royal Brompton and Harefield NHS Foundation Trust (London, UK; host institution) and required multidisciplinary team meeting (MDTM) characterisation between March 1, 2010, and Aug 31, 2010. Only patients whose baseline clinical, radiological, and, if biopsy was taken, pathological data were undertaken at the host institution were included. Seven MDTMs, consisting of at least one clinician, radiologist, and pathologist, from seven countries (Denmark, France, Italy, Japan, Netherlands, Portugal, and the UK) evaluated cases of diffuse parenchymal lung disease in a two-stage process between Jan 1, and Oct 15, 2015. First, the clinician, radiologist, and pathologist (if lung biopsy was completed) independently evaluated each case, selected up to five differential diagnoses from a choice of diffuse lung diseases, and chose likelihoods (censored at 5% and summing to 100% in each case) for each of their differential diagnoses, without inter-disciplinary consultation. Second, these specialists convened at an MDTM and reviewed all data, selected up to five differential diagnoses, and chose diagnosis likelihoods. We compared inter-observer and inter-MDTM agreements on patient first-choice diagnoses using Cohens kappa coefficient (κ). We then estimated inter-observer and inter-MDTM agreement on the probability of diagnosis using weighted kappa coefficient (κw). We compared inter-observer and inter-MDTM confidence of patient first-choice diagnosis. Finally, we evaluated the prognostic significance of a first-choice diagnosis of idiopathic pulmonary fibrosis (IPF) versus not IPF for MDTMs, clinicians, and radiologists, using univariate Cox regression analysis. FINDINGS 70 patients were included in the final study cohort. Clinicians, radiologists, pathologists, and the MDTMs assigned their patient diagnoses between Jan 1, and Oct 15, 2015. IPF made up 88 (18%) of all 490 MDTM first-choice diagnoses. Inter-MDTM agreement for first-choice diagnoses overall was moderate (κ=0·50). Inter-MDTM agreement on diagnostic likelihoods was good for IPF (κw=0·71 [IQR 0·64-0·77]) and connective tissue disease-related interstitial lung disease (κw=0·73 [0·68-0·78]); moderate for non-specific interstitial pneumonia (NSIP; κw=0·42 [0·37-0·49]); and fair for hypersensitivity pneumonitis (κw=0·29 [0·24-0·40]). High-confidence diagnoses (>65% likelihood) of IPF were given in 68 (77%) of 88 cases by MDTMs, 62 (65%) of 96 cases by clinicians, and in 57 (66%) of 86 cases by radiologists. Greater prognostic separation was shown for an MDTM diagnosis of IPF than compared with individual clinicians diagnosis of this disease in five of seven MDTMs, and radiologists diagnosis of IPF in four of seven MDTMs. INTERPRETATION Agreement between MDTMs for diagnosis in diffuse lung disease is acceptable and good for a diagnosis of IPF, as validated by the non-significant greater prognostic separation of an IPF diagnosis made by MDTMs than the separation of a diagnosis made by individual clinicians or radiologists. Furthermore, MDTMs made the diagnosis of IPF with higher confidence and more frequently than did clinicians or radiologists. This difference is of particular importance, because accurate and consistent diagnoses of IPF are needed if clinical outcomes are to be optimised. Inter-multidisciplinary team agreement for a diagnosis of hypersensitivity pneumonitis is low, highlighting an urgent need for standardised diagnostic guidelines for this disease. FUNDING National Institute of Health Research, Imperial College London.

Well-differentiated papillary mesothelioma: clustering in a Portuguese family with a germline BAP1 mutation

BACKGROUND Well-differentiated papillary mesothelioma (WDPM) is a rare variant of epithelioid mesothelioma and is considered to be associated with good prognosis due to its clinically indolent behavior and long survival. Most reported cases involve the peritoneum of women at reproductive age with no history of exposure to asbestos, with pleural involvement being less common. The optimal management, including the role of chemotherapy in the treatment of WDPM, remains unsettled. PATIENTS AND METHODS The authors describe two cases of WDPM in women of the same family (siblings); the elder with WDPM of the pleura and peritoneum with a 12-year survival period and the younger with a WDPM of the peritoneum diagnosed in 2011 and uveal melanoma diagnosed in 2012. Neither patient had any known exposure to asbestos fibers or any other mineral carcinogens. RESULTS After the concurrent diagnosis of WDPM and uveal melanoma, genetic diagnosis was carried out taking into consideration that these two malignancies were recently associated with hereditary BAP1 gene mutations and it was positive for both the patients. CONCLUSIONS To our knowledge, this is the first description of WDPM in two siblings who also presented with a germline BAP1 mutation. This article provides evidence of the wide clinical spectrum of cancer susceptibility associated with a BAP1 germline mutation.

Pneumonia aguda fibrinosa e organizante

The term Acute Fibrinous and Organizing Pneumonia (AFOP) has been proposed by Beasley et al for cases that not fit into the histopathologic criteria of the recognized entities described as acute or subacute clinical presentations. The presence of intra-alveolar fibrin in the form of fibrin ‘balls’ and organizing pneumonia with patchy distribution are the main histological features of this entity. We describe the case of a male patient with the diagnostic of AFOP made by surgical lung biopsy. He had a subacute presentation of symptoms consisting of productive cough, chest pain and fever. Bilateral infiltrates with patchy and diffuse distribution were the predominant features in his chest HRCT scan. The patient had a good clinical course after a treatment with prednisone and cyclophosphamide. Our hope in reporting this case study is to add some more data to the discussion of this new entity. Rev Port Pneumol 2006; XII (5): 615-620

Multi-usage of microwave Doppler radar in pervasive healthcare systems for elderly

Health assessment requirements for aging population have accentuated the need of ubiquitous and pervasive e-Health environments. Recently, new implemented embedded vital signs sensors were joined to ubiquitous computation in order to materialize Ubi-Health systems with lower price and augmented interoperability in comparison with traditional clinical instrumentation for in house health status monitoring needs. The work reports the utilization of the smart sensor based on 24GHz microwave FMCW (frequency modulated continuous wave) Doppler embedded in smart objects such as wheelchairs, walkers or crutches used by elderly, that perform cardiorespiratory signs and physical activity monitoring. According to the requirements of sensor identification and wireless data communication between the smart sensor and the advanced signal processing, data logging and web publishing unit, a combination between Bluetooth MAC based identification and Virtual Transducer Electronic Data Sheet was implemented. The system allows vital signs and motor activity monitoring, including gait characterization.

Hemangioendotelioma epitelióide - Um tumor pulmonar raro

Resumo Os autores descrevem um caso de uma neoplasia pulmonar rara – hemangioendotelioma epitelioide – numa doente do sexo feminino, de 39 anos, assintomatica ate Dezembro de 2003, altura em que apresentou toracalgia direita de caracteristicas pleuriticas. A doente era portadora de uma radiografia toracica antiga, efectuada ha 13 anos, que revelava multiplos pequenos nodulos pulmonares bilaterais, atribuidos a sequelas de tuberculose pulmonar. O diagnostico histologico definitivo foi efectuado atraves de biopsia pulmonar por toracotomia. Dado a doente ter apresentado agravamento clinico e imagiologico foi iniciada terapeutica com interferao α-2a. Apesar da estabilidade imagiologica das lesoes pulmonares, a doente manteve-se sintomatica e faleceu nove meses depois do diagnostico ter sido estabelecido. Os autores realcam a raridade deste tipo de neoplasia pulmonar e discutem a sua apresentacao clinica, caracteristicas histologicas, tratamento e prognostico. Rev Port Pneumol 2008; XIV (3): 421-425

Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity

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.

Linfangioma cístico do mediastino

Resumo O linfangioma mediastinico e uma neoplasia vascular rara, correspondendo a 0,7 a 4,5% de todos os tumores do mediastino. E um tumor benigno, que ocorre geralmente como uma massa mediastinica de crescimento lento. A maioria dos doentes esta assintomatica, sendo diagnosticado incidentalmente, apos a realizacao de uma radiografia toracica de rotina. O tratamento de eleicao consiste na resseccao cirurgica completa. O prognostico depende do grau de ressecabilidade e, se a lesao for totalmente removida, nao se esperam recidivas. Rev Port Pneumol 2006; XII (6): 731-735

Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy

AbstractObjectivesPleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) with variable clinical and radiological features. Diagnosis is based on histology obtained by surgical lung biopsy, which is associated with significant mortality and morbidity. This study aims to briefly review PPFE and discuss the role of CT-guided transthoracic core lung biopsy (TTB) in its diagnosis.Materials/MethodsFour cases of PPFE diagnosed at our institution with TTB are reported and discussed.ResultsClinical, radiological and histological features are in agreement with the previous literature cases. TTB provided the diagnosis in all cases. Iatrogenic pneumothorax was the main complication in all patients. Placement of a chest tube was needed in three patients. An overlap between PPFE and other interstitial lung diseases (ILD) was documented.ConclusionPPFE is an underdiagnosed IIP, so radiologist awareness of it needs to be widespread in patients with fibrosis with apical-caudal distribution. Coexistence of different lung diseases strengthens the idea of a predisposing factor. TTB proved to be a good diagnostic tool and can be considered the first choice for invasive assessment of these patients. PFFE has a variable course with no established therapeutic options; therefore a multidisciplinary team is crucial in the approach to patients with ILD.Main messages/Teaching Points• PPFE should be considered in the differential diagnosis of fibrosis with apical-caudal distribution. • CT-guided TTB can be considered the first choice for invasive assessment of PPFE. • Site of biopsy has to be chosen carefully in order not to miss PPFE. • Coexistence of different lung diseases strengthens the idea of a predisposing factor. • A multidisciplinary team is crucial in the approach to patients with ILD.

Open lung biopsy in patients on mechanical ventilation with suspected diffuse lung disease.

BACKGROUND While open lung biopsy (OLB) performed in patients on mechanical ventilation (MV) with diffuse lung diseases (DLD) can be extremely important in establishing the diagnosis, the associated risk of this procedure should be taken into account. AIM To determine the diagnostic yield, therapeutic changes, complications and mortality in patients with DLD on MV submitted to OLB. METHODS Retrospective study of 19 patients admitted to S. João Hospital between January 1999 and July 2007 (8.5 years). Data analysed included demographic data, ventilation variables before and after biopsy, diagnostic yield, effect on subsequent treatment changes and complications of OLB. Statistical analysis was performed using SPSS 14.0. RESULTS The mean age of patients was 58+/-16.3 years old and 53% were male. The mean duration of hospital stay in Intensive Care Unit before performing OLB was 13+/-7 days. All biopsies were performed by thoracotomy. The diagnostic yield was 95%. There were no significant differences in partial pressure of arterial oxygen/fraction of inspired oxygen (PaO(2)/ FiO(2)) ratio and the positive end expiratory pressure (PEEP) before and after OLB. Postoperative complications occurred in 4 patients (21%; persistent air leak). Alteration in the diagnosis occurred in 14 patients (74%) and in 8 patients (42%) there was a modification in the treatment regimen. Global mortality was 47% (9 patients) but there were no biopsy-related deaths. CONCLUSION The high diagnostic yield and the low incidence of complications make OLB a useful procedure in patients on MV with DLD of unknown aetiology. However, early OLB may lead to even better results in some patients.

Acute fibrinous and organizing pneumonia: A report of 13 cases in a tertiary university hospital.

IntroductionAcute fibrinous and organizing pneumonia (AFOP) is a rare diffuse pulmonary disease, but it is not yet known whether it is a distinct form of interstitial pneumonia or simply a reflection of a tissue sampling issue. MethodsCross-sectional evaluation of clinical and radiological findings, treatments, and outcomes for patients with histologically confirmed AFOP at a tertiary university hospital between 2002 and 2015. ResultsThirteen patients (7 women, 53.8%) with a mean ± SD age of 53.5 ± 16.1 years were included. The main symptoms were fever (69.2%), cough (46.2%), and chest pain (30.8%). All patients presented a radiological pattern of consolidation and 5 (38.5%) had simultaneous ground-glass areas. Histology was obtained by computed tomography-guided transthoracic biopsy in 61.5% of cases and by surgical lung biopsy in the remaining cases. Several potential etiologic factors were identified. Eight patients (61.5%) had hematologic disorders and 3 had undergone an autologous hematopoietic cell transplant. Two (15.4%) had microbiologic isolates, 5 (38.4%) had drug-induced lung toxicity, and 2 (15.4%) were classified as having idiopathic AFOP. In addition to antibiotics and diuretics used to treat the underlying disease, the main treatment was corticosteroids, combined in some cases with immunosuppressants. Median survival was 78 months and 6 patients (46.2%) were still alive at the time of analysis. ConclusionOur findings for this series of patients confirm that AFOP is a nonspecific reaction to various agents with a heterogeneous clinical presentation and clinical course that seems to be influenced mainly by the severity of the underlying disorder.