Susana Guimarães

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.

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.

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.

Inorganic mercury intoxication: A case report

Mercury is a heavy metal with unique physico-chemical properties, and it is well distributed throughout the environment, being present in soil, water and air. This non-essential element is considered by the World Health Organization (WHO) as one of the ten most troublesome chemical to public health. Its toxicity spectrum depends on the chemical form in which it presents: elemental (metallic), organic or inorganic. The known intoxications are mainly occupational (mining, agriculture, incineration) or related to the use of dental amalgams or the consumption of contaminated fish and shellfish. Nowadays, acute exposures to toxic amounts of mercury are increasingly rare, especially those involving inorganic mercury compounds. The rate is even lower if we refer to intentional poisonings. Although there is a growing understanding of the toxicokinetics of mercury, there is still a lack of studies that support the emerging theories about its bioavailability in humans. In this manuscript we describe a rare case of an individual who committed suicide by ingesting mercuric oxide. The aim is to offer a medical contribution to the better understanding of the kinetics of this metal, making a discussion based on published literature and analyzing its distribution, metabolism, internal doses, target and reservoir organs. The whole case - clinical course of the victim and her fatal destiny, the ante- and post-mortem sample concentrations and the necropsy findings - illustrates a situation that meets specific features of acute poisoning by ingestion of inorganic mercury, thus constituting an important support towards a more realistic and a based on evidence understanding of mercury biodistribution in humans.

Analysis of sarcoidosis in the Oporto region (Portugal)

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 ≥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.

Transbronchial cryobiopsy in the diagnosis of desquamative interstitial pneumonia.

Desquamative interstitial pneumonia (DIP) is a rare interstitial pneumonia usually associated with cigarette smoke.1,2 It is characterized by the accumulation of intra-alveolar macrophages, sometimes associated with giant cells.1,3 The diagnosis may be suggested by patchy ground-glass opacification with a predilection for the mid and lower lung lobes on high-resolution computed tomography (HRCT); subpleural involvement is also typical. Irregular lines, traction bronchiectasis, cysts, emphysema, and nodules are other possible findings of DIP.4 Bronchoalveolar lavage fluid nearly always contains an increased number of alveolar macrophages.2,4 Histologically, DIP is characterized by the accumulation of macrophages in the alveolar spaces associated with interstitial inflammation and/or fibrosis. The macrophages usually contain light brown pigment. Lymphoid nodules and a sparse but distinct eosinophilic infiltrate are common.3 Surgical lung biopsy is still required to make a definitive diagnosis.1--3 Transbronchial lung cryobiopsy (TBLC) is a new endoscopic technique that has recently shown superior diagnostic yield to conventional transbronchial biopsy (TBB).5--7 The advantage of the cryoprobe, compared with conventional TBB or TBB using jumbo forceps is that larger pieces of tissue, without crush artifacts, can be extracted during the freeze-thaw cycle, allowing the identification of complex pathologic patterns. The technique permits visualization of peripheral structures of the secondary pulmonary lobule and facilitates immunohistochemical staining. In addition, TBLC can be performed on an outpatient basis and is both an easier and safer procedure for patients with comorbidities, as it reduces the complications and mortality associated with surgical lung biopsy.6 Most of the data available to date is on TBLC overall diagnostic yield and complication rates.5 However, it is also important for clinicians to know the diagnostic accuracy of TBLC in particular diffuse lung diseases, especially in cases in which histologic evaluation is an essential component of

Learning curve for transbronchial lung cryobiopsy in diffuse lung disease

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 5.4mm (IQR, 5-6) and median area was 19.5mm2 (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 (5.0mm [2.5-16] vs 6.0mm [4-12;] p<0.01) and area (17.5mm2 [6-42] vs 21.5mm2 [10-49]; p<0.01). Logarithm regression was applied to median diagnostic yield and sample length and area for groups of 10 consecutive patients to define the learning curve, which plateaued after approximately 70 procedures. CONCLUSIONS Our findings suggest that proficiency in TBLC is achieved at approximately the 70th procedure; however they need to be validated in more series and cohorts.

WITHDRAWN: Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity

This article has been withdrawn for editorial reasons because the journal will be published only in English. In order to avoid duplicated records, this article can be found at http://dx.doi.org/10.1016/j.rppnen.2014.04.008. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.

Intimal sarcoma of the left atrium – A rare form of mitral valve obstruction

A 70-year-old woman was referred to the emergency department due to mild effort dyspnea, weight loss and night sweats. Transthoracic and transesophageal echocardiography revealed dilated left atrium with an extensive multilobulated mass infiltrating the left atrial posterolateral wall. It prolapsed through the mitral valve during diastole, resulting in elevated mean and peak pressure gradients (8 mmHg and 25 mmHg, respectively [Panels A-C]). Coronary angiography revealed a highly vascularized mass (Panel D, Video 1). Cardiac magnetic resonance (CMR) evidenced the full extension of the mass -measuring 10 cm -in relation to the left atrial posterolateral wall. It extended to both inferior pulmonary veins and revealed tissue characteristics in T1(isointense) and T2-weighted (hyperintense) images. No contrast uptake was found during first-pass perfusion, but progressive and heterogeneous uptake was observed in the early and late gadolinium enhancement (LGE) images, with a low signal intensity central area (Panels E-G, Video 2 and 3). Computed tomography (CT) staging was negative for

Interstitial lung disease and pre-capillary pulmonary hypertension in neurofibromatosis type 1

Although previously reported, the existence of a neurofibromatosis (NF)-associated diffuse lung disease (DLD) still lacks solid evidence. We report a case of a 68-year-old non-smoking female with NF1, pre-capillary pulmonary hypertension (PH) and an interstitial lung pattern. Initial findings included progressive dyspnea, hypoxemia and sparse centrilobular ground-glass micronodules on high-resolution computed tomography (HRCT). Further study demonstrated a severe defect in diffusing capacity for carbon monoxide (DLCO), macrophages on bronchoalveolar lavage and pre-capillary PH on right cardiac catheterization. Surgical biopsy revealed macrophage accumulation along bronchovascular bundles and alveolar spaces and type II pneumocytes hyperplasia. Given the absence of environmental exposure or new drugs, a NF-DLD was hypothesized. Pre-capillary PH was disproportionate to interstitial findings, so it was attributed to a NF1-vasculopathy. Treatment with triple sequential combined therapy was unsuccessful culminating in death 18 months later. This case adds HRCT and anatomopathological data suggesting NF-DLD as a distinct manifestation of the disease.