The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology
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Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc. There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. ⋯ There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5-9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1-2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.
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A proportion of patients with fibrosing interstitial lung diseases (ILDs) develop a progressive phenotype characterised by decline in lung function, worsening quality of life and early mortality. Other than idiopathic pulmonary fibrosis (IPF), there are no approved drugs for fibrosing ILDs and a poor evidence base to support current treatments. Fibrosing ILDs with a progressive phenotype show commonalities in clinical behaviour and in the pathogenic mechanisms that drive disease worsening. ⋯ Studies in animal models with features of fibrosing ILDs such as IPF, SSc-ILD, rheumatoid arthritis-ILD, hypersensitivity pneumonitis and silicosis demonstrate that nintedanib has anti-fibrotic activity irrespective of the trigger for the lung pathology. This suggests that nintedanib inhibits fundamental processes in the pathogenesis of fibrosis. A trial of nintedanib in patients with progressive fibrosing ILDs other than IPF (INBUILD) will report results in 2019.
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Although there has been tremendous growth in our understanding of chronic obstructive pulmonary disease (COPD) and its pathophysiology over the past few decades, the pace of therapeutic innovation has been extremely slow. COPD is now widely accepted as a heterogeneous condition with multiple phenotypes and endotypes. ⋯ Precision medicine is enabled by biomarkers that can: 1) accurately identify subgroups of patients who are most likely to benefit from therapeutics and those who will only experience harm (predictive biomarkers); 2) predict therapeutic responses to drugs at an individual level (response biomarkers); and 3) segregate patients who are at risk of poor outcomes from those who have relatively stable disease (prognostic biomarkers). In this essay, we will discuss the current concept of precision medicine and its relevance for COPD and explore ways to implement precision medicine for millions of patients across the world with COPD.