Respiration; international review of thoracic diseases
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Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia and has a dismal prognosis. Median age at IPF onset is 60-70 years and it is mainly related to cigarette smoke exposure. Its clinical profile is heterogeneous and different clinical phenotypes are now better defined: familial IPF, slow and rapid progressors, combined pulmonary fibrosis and emphysema, anti-neutrophil cytoplasmic antibodies/microscopic polyangiitis and IPF, and IPF associated with lung cancer. ⋯ However, less invasive procedures (transbronchial lung biopsy or even improved transbronchial lung biopsy by cryoprobes) are now under consideration. Prognostic indicators are mainly derived by pulmonary function tests. Recently, staging systems have been proposed.
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Acute respiratory distress syndrome (ARDS) is a pulmonary syndrome with growing prevalence and high mortality and morbidity that increase with age. There is no current therapy able to restore pulmonary function in ARDS patients. ⋯ Recent studies have demonstrated that MSCs can also control oxidative stress, transfer functional mitochondria to the damaged cells, and control bacterial infection by secretion of antibacterial peptides. These characteristics make MSCs promising candidates for ARDS therapy.
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Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and ultimately fatal disease, with a highly variable course in individual patients. Episodes of rapid deterioration are not uncommon, often following a period of stability. In cases of uncertain etiology, with typical clinical and high-resolution computed tomography (HRCT) features, the term 'acute exacerbation of IPF' (AE-IPF) has been coined to describe a combination of diffuse alveolar damage and preexisting usual interstitial pneumonia. ⋯ Acute exacerbations are less prevalent in other fibrotic lung diseases than in IPF and may have a better outcome, with the exception of acute exacerbations of rheumatoid lung. In AE-IPF, the exclusion of alternative causes of rapid deterioration, including heart failure and infection, is the main goal of investigation. Empirical high-dose corticosteroid steroid therapy is generally used in AE-IPF, without proven benefit.
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Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease with increasing incidence; the median survival is only 35 months and as yet no therapy has been proven to prolong survival. Recent unexpected randomised controlled trial (RCT) results and the conflicting evaluations of drug efficacy by regulatory agencies when considering the approval of pirfenidone have emphasised that we remain in the first stages of both our understanding of disease-relevant therapeutic targets and in our ability to investigate these putative targets with well-designed RCT. ⋯ The role of a formal multi-disciplinary team meeting in a specialist centre with expertise in IPF is key to this. New methodological and ethical research challenges will arise as we enter an era of potential combination therapy; standardized, robust RCT design will be central to meeting these challenges if we are to enable ongoing progress in our aim of increasing both the length and quality of life of patients with IPF.