Current opinion in pulmonary medicine
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Despite currently available treatments, many asthma patients remain inadequately controlled, but identifying distinct patient populations (phenotypes/endotypes) may optimize their management. This review discusses some of the controversies and opportunities for improved disease control in severe asthma. ⋯ Appropriate use of biologics in severe asthma should be supported by further understanding of biomarkers predicting response to targeted therapy. Because of their association with significant adverse effects, add-on oral corticosteroids should be considered a last treatment option for patients with uncontrolled severe asthma. Finally, severe asthma in pediatrics poses a unique opportunity for potential prevention strategies.
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Vitamin D supplementation is widespread used in the general population. In sarcoidosis, up to 50% of patients, especially postmenopausal women and those taking corticosteroids, show evidence of increased bone fragility. The purpose of this review is to provide an evidence-based rationale on how to treat sarcoidosis patients with bone health issues. ⋯ Vitamin D supplementation may be withheld in sarcoidosis patients with bone fragility, unless calcitriol levels are below normal limits. A treating scheme is proposed.
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Heritable pulmonary arterial hypertension (PAH) is an autosomal dominant disease with incomplete penetrance because of mutations in bone morphogenetic protein receptor-II (BMPR2), activin A receptor type II-like kinase 1, endoglin, caveolin-1, potassium channel subfamily K, member 3, and T-box gene 4 genes. Heritable pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH) is an autosomal recessive disease because of biallelic mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 gene. The 2015 european society of cardiology (ESC) and european respiratory society (ERS) pulmonary hypertension guidelines recommend genetic counselling and testing to adults and children with PAH or PVOD/PCH as well as in adult relatives at risk of carrying a predisposing mutation. ⋯ Genetic counseling and testing has to be implemented in pulmonary hypertension centers.
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Review
Acute exacerbations of interstitial lung disease: lessons from idiopathic pulmonary fibrosis.
The purpose of this review is to provide an update on acute exacerbation of interstitial lung disease (ILD), with a focus on idiopathic pulmonary fibrosis (IPF), in the light of the recently revised definition of acute exacerbation-IPF. Strengths and limitations of the current definition of acute exacerbation-IPF are also discussed. ⋯ Acute exacerbation-IPF has recently been redefined. A standardized definition, similar to that of other chronic respiratory diseases, will likely facilitate the performance of highly needed studies in acute exacerbation of both IPF and other ILDs.
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Therapeutic advances in the management of idiopathic pulmonary fibrosis (IPF) has led to improved outcomes with the use of the antifibrotic agents pirfenidone and nintedanib, with a number of randomized studies demonstrating benefits in slowing disease progression in IPF. However, treatment of other fibrosing interstitial lung diseases (ILD) remains challenging. ⋯ The review will discuss the rationale for use of established antifibrotic drugs approved for IPF for use in non-IPF ILD, describe supportive data from observational studies and ongoing clinical trials.