Organizing pneumonia revisited: insights and uncertainties from a series of 67 patients.

Background: Organizing pneumonia (OP) is classified as an acute/subacute pneumonia according to the American Thoracic Society/European Respiratory Society statement (2013 update). Although its clinical presentation, radiologic and histologic features are well established, data on the relevance of potential causes, corticosteroid doses and length, or management of relapses are based on heterogeneous series of patients. Objectives: The aims of this study were to describe clinical presentation, diagnosis and treatment of OP, explore potential causes, discuss strategies for managing relapses, and analyze prognostic factors. We also discuss our findings in relation to relevant data in the literature. Methods: We performed a cross-sectional study of all patients diagnosed with OP at a tertiary referral center in northern Portugal between 2008 and 2015. Results: Sixty-seven patients were diagnosed with OP over the 7-year study period. Dyspnea and cough were the most common presenting symptoms and approximately 30% of patients were hospitalized at the time of diagnosis. Approximately half of the patients were receiving drugs described as potential causes of OP. Microorganisms were isolated in approximately one-third of patients. Other potential causes identified were hematologic disorders, neoplasms, connective tissue diseases, myelodysplastic syndromes, immunodeficiencies, radiotherapy, and bird exposure. Cryptogenic OP was diagnosed in just 16 patients (23.8%). Corticosteroids were the most common treatment and 11 patients (16.4%) experienced relapse. Conclusions: The findings for this series of patients confirm the extreme variability of the contexts in which OP can occur and suggest that rather than a distinct, homogeneous clinicopathologic entity, OP is a non-specific reaction whose outcomes are dependent on the cause. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 129-138).

Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence.

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease that up to now has been associated with a poor prognosis. However, the results of the INPULSIS and ASCEND trials and the approval of nintedanib and pirfenidone have marked the beginning of a new era for IPF patients. Questions remain, however. Should these drugs be used earlier? What effect will they have on more severe disease? Will their effects last beyond the trial period? This manuscript is the outcome of a multidisciplinary meeting between pulmonology, radiology, and pathology clinicians on the use of antifibrotic agents in IPF. In our opinion, the existing data show that pirfenidone and nintedanib slow functional decline in early stages of disease. These drugs also appear to result in therapeutic benefits when administered to patients with advanced disease at diagnosis and maintain effective over time. The data also suggest that continuing antifibrotic therapy after disease progression may confer benefits, but more evidence is needed. Early diagnosis and treatment are crucial for reducing functional decline, slowing disease progression, and improving quality of life.

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.

Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica.

Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. Although several potential risk factors have been described, it is a progressive fibrosing disease of unknown cause affecting mainly adults over 50 years and associated with a poor prognosis, reflected in a median survival of 2-3 years after diagnosis. The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an internationally accepted recommendation. In regard to therapy, the situation has changed radically since the publication of the ATS/ERS/JRS/ALAT 2011 guidelines on the diagnosis and management of idiopathic pulmonary fibrosis where it was stressed that no proven therapy exists for this disease. Currently besides non-pharmacological treatment, therapy of complications and comorbidities and palliative care, nintedanib and pirfenidone, two compounds with pleiotropic mechanisms of action, are to date, the two drugs with confirmed efficacy in slowing functional decline and disease progression in idiopathic pulmonary fibrosis patients.

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.

Feasibility of Typha latifolia for high salinity effluent treatment in constructed wetlands for integration in resource management systems.

High salinity wastewaters have limited treatment options due to the occurrence of salt inhibition in conventional biological treatments. Using recirculating marine aquaculture effluents as a case study, this work explored the use of Constructed Wetlands as a treatment option for nutrient and salt loads reduction. Three different substrates were tested for nutrient adsorption, of which expanded clay performed better. This substrate adsorbed 0.31 mg kg(-1) of NH4(+)-N and 5.60 mg kg(-1) of PO4(3-)-P and 6.9 mg kg(-1) dissolved salts after 7 days of contact. Microcosms with Typha latifolia planted in expanded clay and irrigated with aquaculture wastewater (salinity 2.4%, 7 days hydraulic retention time, for 4 weeks), were able to remove 94% NH(4+)-N (inlet 0.25 +/- 0.13 mg L(-1)), 78% NO2(-)-N (inlet 0.78 +/- 0.62 mg L(-1)), 46% NO3(-)-N (inlet 18.83 +/- 8.93 mg L(-1)) whereas PO4(3-)-P was not detected (inlet 1.41 +/- 0.21 mg L(-1)). Maximum salinity reductions of 52% were observed. Despite some growth inhibition, plants remained viable, with 94% survival rate. Daily treatment dynamics studies revealed rapid PO4(3-)-P adsorption, unbalancing the N:P ratio and possibly affecting plant development. An integrated treatment approach, coupled with biomass valorization, is suggested to provide optimal resource management possibilities.

Idiopathic pulmonary fibrosis--clinical presentation, outcome and baseline prognostic factors in a Portuguese cohort.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is the most common disease in the subgroup of idiopathic interstitial pneumonias. It is inevitably associated to a bad prognosis, although assuming a highly variable clinical course. METHODS: Patients with IPF, observed at Interstitial Lung Diseases outpatient clinic of Centro Hospitalar de São João - Porto, Portugal, were identified and clinical, functional, radiological and bronchoalveolar lavage (BAL) parameters were reviewed. Their clinical course and survival were analyzed in order to identify prognostic factors. RESULTS: Eighty-one patients were included, with a mean age at diagnosis of 63.8 years old. At diagnosis, the main functional abnormalities were restrictive physiology, reduced lung diffusion and exercise capacity impairment. Clinical course was mainly slowly progressive (72.3%). Ten patients (13.2%) had a rapid progression and 11 (14.5%) patients had an acute exacerbation during the course of the disease. IPF's rapid progression was associated to a higher functional impairment at diagnosis, namely in what is related with Forced Vital Capacity (FVC) and Total Lung Capacity (TLC). Median survival was 36 months. A significant difference in survival was observed among different types of clinical course - 41 months for slow progressors and 9 months for rapid progressors. Lower levels of FVC, TLC, 6th minute walk test distance and rest PaO2, and higher BAL neutrophil count were associated with poorer survival in univariate analysis. CONCLUSION: The analysis of this group of IPF patients confirms two clearly different phenotypes, slow and rapid progressors. Those phenotypes seem to have different presentations and a remarkably different natural history. These results could mean different physiopathologic pathways, which could implicate different therapeutic approaches.

[Multiple endocrine neoplasia type IIb]. / Neoplasia endócrina múltipla tipo IIb.

We describe the clinical manifestations of a multiple endocrine neoplasia, type IIb, in a 32 year old woman, admitted to a general hospital with intestinal sub-occlusion. The phenotype associated with bilateral pheochromocytomas show us the diagnosis, but the unsolved problem is the high levels of calcitonin, without macroscopic evidence of tumor relapse.

[Adenocarcinoma of the colon disclosed by endocarditis caused by Streptococcus bovis]. / Adenocarcinoma do colon revelado por endocardite por estreptococos bovis.

The association of Streptococcus bovis endocarditis with colonic neoplasms has been well documented. We describe a patient in whom the finding of a Str. bovis endocarditis stimulated investigation which resulted in the diagnosis and surgical treatment of an adenocarcinoma of the colon.