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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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.
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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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Neurosarcoidosis occurs in 5% of patients with sarcoidosis and can be difficult to diagnose. In this review we discuss the most recent advances in our understanding of the disease, describing clinical characteristics, diagnostic process, treatment, and prognosis. ⋯ Diagnosing and treating patients with neurosarcoidosis remains a challenge. Long-term prospective studies evaluating patients suspected of neurosarcoidosis are needed to assess sensitivity and specificity of ancillary investigations and diagnostic criteria. Furthermore, future studies are needed to evaluate the prognosis and the optimal treatment strategy.
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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.