Bronchoscopically-visible massive central airway cancer cavitation is associated with metastatic disease, lack of actionable mutations and poor prognosis: a case series.

Cavitating lung tumors occur in approximately 10-15% of the patients, are more commonly associated with squamous histology, and are typically located in the lung parenchyma. Herein we describe an exceedingly rare series of 5 patients, 4 of whom treatment-naïve, whose tumor caused the disruption of the normal airway anatomy at the level of lobar or segmental bronchi, leading to the formation of an endoscopically-visible cavity which ended up in the lung parenchyma or even into the pleural space. Sex (3 males, 2 females), smoking habit (2 never smokers, 2 former smokers, 1 current smoker), and histology (3 adenocarcinoma, 2 squamous cell carcinoma) were heterogeneous, but the 4 patients treatment-naïve presented with metastatic disease, poor ECOG performance status, similar clinical complaints of long duration, and lack of actionable mutations. The only patient who exhibited a meaningful response to treatment had the lowest symptoms' duration, the smallest size of the cavitated mass, and the best performance status at the time of diagnosis. This series provides the first comprehensive description of a rare presentation of lung cancer characterized by similar clinical complaints, delayed diagnosis and poor prognosis.

Road Toward a New Model of Care for Idiopathic Pulmonary Fibrosis in the Lazio Region.

A timely, confirmed diagnosis of Idiopathic Pulmonary Fibrosis (IPF) has a significant impact on the evolution of the disease. The current model of care in the Lazio region (in Italy) was assessed on the basis of real-world data provided by the four reference centers responsible for diagnosing and treating IPF. The 5-year, population-based, retrospective longitudinal study provided the data that is at the basis of the current proposal for a new clinical and therapeutic pathway (DTCP) and has been shared with regional decision makers. A DTCP must be defined and based on four pillars: GPs, pulmonologists, IPF centers, and telemedicine. Each must play a role within a sort of hub-and-spoke model. IPF centers remain the hubs, while spokes are identified in trained GPs and pulmonologists.

The adoption of nintedanib in systemic sclerosis: the SENSCIS study.

Nintedanib shows a statistically significant effect on lung function decay in patients with ILD secondary to systemic sclerosis, but no effect on skin fibrosis and on health-related quality of life http://bit.ly/2vfJRj7.

Differences in hospital length of stay between neonates exposed to buprenorphine versus methadone in utero: A retrospective chart review.

INTRODUCTION: Neonatal abstinence syndrome is a growing concern in neonatal intensive care units in rural and remote settings. METHODS: A retrospective chart review was conducted of 180 mother-infant dyads born with in utero exposure to buprenorphine (n=60), methadone (n=60) or to other opioids (n=60) to determine neonatal length of stay in hospital, number of days on morphine, day of life of initiation of morphine and the need for phenobarbital. RESULTS: The length of stay in hospital for neonates was 5.8 days shorter (95% confidence interval [CI] 6.1 to 8.5 days) for buprenorphine exposure in utero compared to methadone (P=0.001). For neonates requiring treatment for Neonatal abstinence syndrome, those with in utero exposure to buprenorphine required 6.1 fewer days (95% CI 2.5 to 9.7) of treatment with morphine then those exposed to methadone (P<0.0005). There were no statistically significant differences in day of life of initiation of morphine therapy for each of the study groups. The proportion of neonates requiring adjuvant therapy with phenobarbital was statistically significantly higher in neonates exposed to methadone in utero than either buprenorphine or illicit opioids (P<0.0005). CONCLUSIONS: Retrospective data suggest that neonates with in utero exposure to buprenorphine experience a shorter length of stay in hospital, fewer days of treatment with morphine for neonatal abstinence syndrome, and less use of phenobarbital than neonates exposed in utero to methadone. This suggests that Ontario provincial guidelines should be updated to recommend buprenorphine as first line for replacement therapy in pregnancy.

Nintedanib for the treatment of idiopathic pulmonary fibrosis.

INTRODUCTION: Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of pulmonary function, ultimately leading to respiratory failure and death. Two novel compounds, nintedanib and pirfenidone, have shown efficacy in reducing the rate of decline of lung function in IPF patients. The multiple tyrosine kinase inhibitor nintedanib has extensively being studied as a potential angiogenesis inhibitor in clinical against various neoplastic disorders. Afterwards, this compound was successfully tested in IPF. Areas covered: Herein, the authors review the working mechanisms of nintedanib, its pharmacological profile, and its efficacy and safety for patients with IPF. Expert opinion: Nintedanib has shown to be safe and effective in patients with IPF, with a favorable long-term safety profile. There is a lack of comparative trials of pirfenidone and nintedanib, and the choice of treatment is left to the physicians' judgement. Future directions of nintedanib use are represented by the treatment of progressive fibrosing interstitial lung disease other than IPF, IPF with advanced functional impairment, and lung fibrosis secondary to connective tissue diseases. A promising safety profile for the combinational use of nintedanib and pirfenidone in IPF has also recently emerged.

Idiopathic Pulmonary Fibrosis: Molecular Endotypes of Fibrosis Stratifying Existing and Emerging Therapies.

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown causes. Current diagnostic criteria are based on radiological, clinical, and histopathological features but, unfortunately, still many patients remain undiagnosed. Two currently approved therapies, pirfenidone and nintedanib, slow down disease progression but failed to block or revert it. On the other hand, many of the therapeutic agents tested in several clinical trials have not given satisfactory answers, probably due to the pathological heterogeneity of the disease. A growing number of studies show that IPF phenotype is the common clinical outcome of a variety of different pathophysiological mechanisms that identify disease subgroups characterised by specific genetic and molecular biomarkers (endotypes). The precision medicine approach is identifying and analysing the complex system of genetic, molecular, environmental, and behavioural variables underlying the development of the disease and the response to therapy. These molecular pathways are potential targets for novel agents and useful diagnostic, prognostic, and theragnostic biomarkers. We outline the status of knowledge in this field by discussing the complex pathogenetic pathways underlying different disease subgroups and assessing a stratification approach to novel therapeutic agents based on these endotypes.