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Significance of connective tissue diseases features in pulmonary fibrosis.

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Significance of connective tissue diseases features in pulmonary fibrosis.

Eur Respir Rev. 2013 Sep 1;22(129):273-80

Authors: Cottin V

Abstract
Interstitial lung disease (ILD) can occur in any of the connective tissue diseases (CTD) with varying frequency and severity, and an overall long-term prognosis that is less severe than that of idiopathic pulmonary fibrosis (IPF). Because ILD may be the presenting manifestation of CTD and/or the dominant manifestation of CTD, clinical extra-thoracic manifestations should be systematically considered in the diagnostic approach of ILD. When present, autoantibodies strongly contribute to the recognition and classification of the CTD. Patients with clinical extrathoracic manifestations of CTD and/or autoantibodies (especially with a high titer and/or the antibody is considered "highly specific" of an autoimmune condition), but who do not fit with established international CTD criteria may be called undifferentiated CTD or "lung-dominant CTD". Although it remains to be determined which combination of symptoms and serologic tests best identify the subset of patients with clinically relevant CTD features, available evidence suggests that such patients may have distinct clinical and imaging presentation and may portend a distinct clinical course. However, autoantibodies alone when present in IPF patients do not seem to impact prognosis or management. Referral to a rheumatologist and multidisciplinary discussion may contribute to management of patients with undifferentiated CTD.

PMID: 23997055 [PubMed - in process]

Pulmonary hypertension in chronic interstitial lung diseases.

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Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis.

The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed "out-of-proportion" PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH.

At present, no specific therapies have been approved for the treatment of PH in patients with ILDs.

Sex-specific lung diseases: effect of oestrogen on cultured cells and in animal models.

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Sex prevalence in lung disease suggests that sex-specific hormones may contribute to the pathogenesis and/or progression of at least some lung diseases, such as lung adenocarcinoma, lymphangioleiomyomatosis (LAM) and benign metastasising leiomyoma (BML). Oestrogen is an important hormone in normal lung development and in the pathogenesis of female predominant pulmonary diseases.

In vivo and in vitro studies have facilitated our understanding of disease pathogenesis and discovery of potential therapeutic targets. Oestrogen promoted disease progression in cell and animal models of lung adenocarcinoma, LAM and BML. Specifically, oestrogen enhanced tumour growth and metastasis in animal models of these diseases. Furthermore, 17β-estradiol (E2), the most abundant form of oestrogen in humans, increased the size and proliferation of cultured cells of lung adenocarcinoma and LAM. Coupled with the known mechanisms of oestrogen metabolism and signalling, these model systems may provide insights into the diverse effects of oestrogen and other hormones on lung diseases.

Anti-oestrogen treatments that target key events of oestrogen synthesis or signalling, such as aromatase activity, oestrogen receptors and signalling pathways, may offer additional opportunities for clinical trials.

The role of infection in the pathogenesis of idiopathic pulmonary fibrosis.

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Idiopathic pulmonary fibrosis (IPF) is a progressive, and invariably fatal, condition that is believed to arise in genetically susceptible individuals as a consequence of an aberrant wound-healing response following repetitive alveolar injury.

The exact triggers, which initiate the fibrotic process, remain unknown. Infectious agents, including both viruses and bacteria, have the capacity to cause alveolar-epithelial cell injury and apoptosis.

Relatively few studies have examined the role of infection in IPF. Those that have, point to viruses playing a key role as cofactors in the initiation and progression of IPF. There is also some evidence to suggest that viral infection may be responsible for a proportion of acute exacerbations of IPF. The role played by bacteria in the pathogenesis of IPF is less clear cut. Studies from other respiratory diseases suggest that alterations in the lung microbiome are associated with disease and that these changes influence disease behaviour. Emerging molecular microbiological techniques are making the study of microbial communities in the lung easier. It is hoped that by combining such techniques with the careful longitudinal phenotyping of patients with IPF, it will be possible to elucidate the role played by bacteria and viruses in the pathogenesis of the disease.

If infection plays a causal role in IPF then it is possible that therapeutic strategies, utilising currently available antiviral or antibiotic drugs, may be effective in modifying the course of this devastating condition.

Mechanistic Insights into the Contribution of Epithelial Damage to Airway Remodeling: Novel Therapeutic Targets for Asthma.

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Mechanistic Insights into the Contribution of Epithelial Damage to Airway Remodeling: Novel Therapeutic Targets for Asthma.

Am J Respir Cell Mol Biol. 2013 Aug 27;

Authors: Royce SG, Li X, Tortorella S, Goodings L, Chow BS, Giraud AS, Tang ML, Samuel CS

Abstract
It has been suggested that an inherent airway epithelial repair defect is the root cause of airway remodeling in asthma. However, the relationship between airway epithelial injury and repair, airway remodeling and airway hyperresponsiveness has not been directly examined. We investigated the contribution of epithelial damage/repair to the development of airway remodeling and airway hyperresponsiveness using a validated naphthalene-induced murine model of airway injury. Additionally, we examined the endogenous vs exogenous role of the epithelial repair peptide, trefoil factor 2 (TFF2) in disease pathogenesis. A single dose of naphthalene (200mg/kg in 10ml/kg body weight corn oil vehicle, i.p.) was administered to mice. Control mice were treated with corn oil (10ml/kg body weight, i.p.). At 12, 24, 48, 72h post-naphthalene or corn oil injection, airway hyperresponsiveness and various measures of airway remodeling were examined by invasive plethysmography and morphometric analyses, respectively. TFF2-deficient mice and intranasal treatment were used to examine the role of the epithelial repair peptide. Naphthalene treatment induced denudation and apoptosis of airway epithelial cells, goblet cell metaplasia, elevated airway hyperresponsiveness as well as increased levels of endogenous TFF2. Airway epithelial changes peaked at 12h following naphthalene treatment, while airway remodeling changes were observed from 48h. TFF2 was protective against epithelial damage and induced remodeling, and was found to mediate organ protection via a platelet-derived growth factor-associated mechanism. Our findings directly demonstrated the contribution of epithelial damage to airway remodeling and airway hyperresponsiveness, and suggest that preventing airway epithelial damage/promoting epithelial repair may have therapeutic implications for asthma treatment.

PMID: 23980699 [PubMed - as supplied by publisher]

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