Sarcoidosis-like reaction secondary to adalimumab treatment in a patient with axial spondyloarthritis.

Anti-TNF agents, namely adalimumab, are safe drugs that represent an important arsenal in the treatment of immune-mediated inflammatory diseases. "Paradoxical effects" have been described with their use. A sarcoidosis "like" reaction induced by these agents is rare and is characterized by a systemic granulomatous reaction indistinguishable from sarcoidosis. We present a 55-year-old male patient, with axial spondyloarthritis, treated with with adalimumab. About 17 months under this therapy, he complained of dry cough and wheezing. Chest CT showed a peri-lymphatic and pericisural micronodular pattern and hilo-mediastinal lymph nodes, suggestive of sarcoidosis. Angiotensin converting enzyme was increased. Assuming the hypothesis of a sarcoidosis-like reaction secondary to adalimumab this therapy was discontinued with progressive improvement in the patient's complaints and in the radiological changes.

The Role of Ultrasonography in the Diagnosis and Decision Algorithm for the Management of Pneumothorax after Transbronchial Lung Cryobiopsy.

BACKGROUND: Pneumothorax is one of the main complications of transbronchial lung cryobiopsy (TBLC). Chest ultrasound (CUS) is a radiation-free alternative method for pneumothorax detection. OBJECTIVE: We tested CUS diagnostic accuracy for pneumothorax and assessed its role in the decision algorithm for pneumothorax management. Secondary objectives were to evaluate the post-procedure pneumothorax occurrence and risk factors. METHODS: Eligible patients underwent TBLC, followed by chest X-ray (CXR) evaluation 2 h after the procedure, as our standard protocol. Bedside CUS was performed within 30 min and 2 h after TBLC. Pneumothorax by CUS was defined by the absence of lung sliding and comet-tail artefacts and confirmed with the stratosphere sign on M-mode. Pneumothorax size was determined through lung point projection on CUS and interpleural distance on CXR and properly managed according to clinical status. RESULTS: Sixty-seven patients were included. Nineteen pneumothoraces were detected at 2 h after the procedure, of which 8 (42.1%) were already present at the first CUS evaluation. All CXR-detected pneumothoraces had a positive CUS detection. There were 3 discordant cases (κ = 0.88, 95% CI: 0.76-1.00, p < 0.001), which were detected by CUS but not by inspiration CXR. We calculated a specificity of 97.5% (95% CI: 86.8-99.9) and a sensitivity of 100% (95% CI: 87.2-100) for CUS. Pneumothorax rate was higher when biopsies were taken in 2 lobes and if histology had pleural representation. Final diagnosis was achieved in 79.1% of patients, with the most frequent diagnosis being hypersensitivity pneumonitis. Regarding patients with large-volume pneumothorax needing drainage, the rate of detection was similar between CUS and CRX. CONCLUSION: CUS can replace CXR in detecting the presence of pneumothorax after TBLC, and the lung point site can reliably indicate its size. This useful method optimizes time spent at the bronchology unit and allows immediate response in symptomatic patients, helping to choose optimal treatment strategies, while preventing ionizing radiation exposure.

Correction: A Prediction Rule to Stratify Mortality Risk of Patients with Pulmonary Tuberculosis.

[This corrects the article DOI: 10.1371/journal.pone.0162797.].

Neural respiratory drive predicts long-term outcome following admission for exacerbation of COPD: a post hoc analysis.

Neural respiratory drive (NRD), as reflected by change in parasternal muscle electromyogram (EMGpara), predicts clinical deterioration and safe discharge in patients admitted to hospital with an acute exacerbation of COPD (AECOPD). The clinical utility of NRD to predict the long-term outcome of patients following hospital admission with an AECOPD is unknown. We undertook a post hoc analysis of a previously published prospective observational cohort study measuring NRD in 120 patients with AECOPD. Sixty-nine (57.5%) patients died during follow-up (median 3.6 years). Respiratory failure was the most common cause of death (n=29; 42%). In multivariate analysis, factors independently associated with an increased mortality included NRD (HR 2.14, 95% CI 1.29 to 3.54, p=0.003), age (HR 2.03, 95% CI 1.23 to 3.34, p=0.006), PaCO2 at admission (HR 1.83, 95% CI 1.06 to 3.06, p=0.022) and long-term oxygen use (HR 2.98, 95% CI 1.47 to 6.03, p=0.002). NRD at hospital discharge could be measured in order to assess efficacy of interventions targeted to optimise COPD and reduce mortality following an AECOPD. Original clinicaltrial.gov number: NCT01361451.

Chronic Bacterial Infection Prevalence, Risk Factors, and Characteristics: A Bronchiectasis Population-Based Prospective Study.

BACKGROUND: Few data are available on chronic bacterial infections (CBI) in bronchiectasis patients. Given that CBI seems to trigger longer hospital stays, worse outcomes, and morbimortality, this study was undertaken to assess CBI prevalence, characteristics, and risk factors in outpatients with bronchiectasis. METHODS: A total of 186 patients followed in a bronchiectasis tertiary referral centre in Portugal were included. Demographic data and information on aetiology, smoking history, mMRC score, Bronchiectasis Severity Index (BSI) score, sputum characteristics, lung function, exacerbations, and radiological involvement degree were collected. RESULTS: Patients included (mean age 54.7 ± 16.2 years; 60.8% females) were followed up for a period of 3.8 ± 1.7 years. The most common cause of bronchiectasis was infection (31.7%) followed by immune deficiencies (11.8%), whereas in 29% of cases, no cause was identified. Haemophilus influenzae (32.3%) and Pseudomonas aeruginosa (30.1%) were the most common CBI-associated possible pathogenic microorganisms. CBI patients presented a higher follow-up time than no-CBI patients (p = 0.003), worse lung function, BSI (p < 0.001), and radiological (p < 0.001) scores, and more prominent daily sputum production (p = 0.002), estimated mean volume (p < 0.001), and purulent sputum (p < 0.001). The number of exacerbations/year (p = 0.001), including those requiring hospital admission (p = 0.009), were also higher in the CBI group. Independent CBI predictors were BSI score (OR 3.577, 95% CI 1.233⁻10.378), sputum characteristics (OR 3.306, 95% CI 1.107⁻9.874), and radiological score (OR 1.052, 95% CI 1.004⁻1.102). CONCLUSION: According to the CBI status, two different sub-groups of patients were found on the basis of several clinical outcomes, emphasizing the importance of routine sputum microbiological monitoring. Further studies are needed to better characterize CBI profiles and to define the individual clinical impact of the most prevalent pathogenic microorganisms.

Alpha-1 Antitrypsin Deficiency Detection in a Portuguese Population.

Alpha-1 antitrypsin deficiency (AATD) is an autosomal co-dominant disease characterised by low serum levels of this molecule. Its epidemiology remains unknown in many countries, mainly due to its underdiagnosed state and lack of patients' registries. We aim to evaluate and characterise a sample of Portuguese individuals tested for AATD, between 2006 and 2015, based on a retrospective analysis from the database of a laboratory offering AATD genetic diagnosis service. 1684 individuals were considered, covering almost every region in Portugal. Genetic diagnosis resulted from requests of clinicians from different areas of expertise, mainly pulmonology (35.5%). Most subjects could be distributed into more common genotypes: MZ (25.4%, n = 427), MS (15.5%, n = 261), SZ (11.2%, n = 188), ZZ (9.4%, n = 158) and SS (5.6%, n = 95). 9.5% of the subjects were found to carry at least one rare deleterious allele, including the recently described PGaia, Q0Oliveira do Douro, Q0Vila Real and a novel SGaia variant. This study comprises 417 subjects (24.7%) with severe to very severe AATD and 761 carriers (45.2%), 22.7% of those identified by familial screening. The present study represents the most complete survey of AATD in Portugal so far and discloses a high rate of severe and very severe deficiency cases, attributed not only to ZZ and SZ genotypes but also to a large number of rare combinations with other null and deficiency alleles. It also uncovers a low awareness to AATD among the medical community, highlighting the need to create a Portuguese national registry and AATD guidelines and increase the awareness about this condition.

Diarrhea as a form of presentation of medullary thyroid carcinoma.

A case is presented of a 57-year-old man consulting for chronic diarrhea. Based on subsequent findings (thyroid nodule and metastases), the possibility of metastatic medullary thyroid carcinoma (MTC) was raised. Thyroidectomy allowed diagnosing a multicentric left lobe MTC. MTC is a rare cause of diarrhea, but should be considered, especially in the presence of signs or symptoms of alarm or nonresponse to empirical therapy.

Kaposi Sarcoma and Lung Transplant: Two Case Reports / Sarcoma de Kaposi y trasplante pulmonar: dos casos clínicos

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A Prediction Rule to Stratify Mortality Risk of Patients with Pulmonary Tuberculosis.

Tuberculosis imposes high human and economic tolls, including in Europe. This study was conducted to develop a severity assessment tool for stratifying mortality risk in pulmonary tuberculosis (PTB) patients. A derivation cohort of 681 PTB cases was retrospectively reviewed to generate a model based on multiple logistic regression analysis of prognostic variables with 6-month mortality as the outcome measure. A clinical scoring system was developed and tested against a validation cohort of 103 patients. Five risk features were selected for the prediction model: hypoxemic respiratory failure (OR 4.7, 95% CI 2.8-7.9), age ≥50 years (OR 2.9, 95% CI 1.7-4.8), bilateral lung involvement (OR 2.5, 95% CI 1.4-4.4), ≥1 significant comorbidity-HIV infection, diabetes mellitus, liver failure or cirrhosis, congestive heart failure and chronic respiratory disease-(OR 2.3, 95% CI 1.3-3.8), and hemoglobin <12 g/dL (OR 1.8, 95% CI 1.1-3.1). A tuberculosis risk assessment tool (TReAT) was developed, stratifying patients with low (score ≤2), moderate (score 3-5) and high (score ≥6) mortality risk. The mortality associated with each group was 2.9%, 22.9% and 53.9%, respectively. The model performed equally well in the validation cohort. We provide a new, easy-to-use clinical scoring system to identify PTB patients with high-mortality risk in settings with good healthcare access, helping clinicians to decide which patients are in need of closer medical care during treatment.

Neumonía eosinofílica como manifestación paraneoplásica de un adenocarcinoma de colon / Eosinophilic pneumonia as a paraneoplastic manifestation of colon adenocarcinoma

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