Pirfenidone in Idiopathic Pulmonary Fibrosis: Real-Life Experience from a German Tertiary Referral Center for Interstitial Lung Diseases.
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Pirfenidone is a novel antifibrotic drug for the treatment of mild-to-moderate idiopathic pulmonary fibrosis (IPF). However, adverse events may offset treatment benefits and compliance.
Objectives: To assess recent course of disease, adverse events and compliance in patients who started pirfenidone.
Methods: In an observational cohort study, 63 patients with mild-to-moderate IPF who started pirfenidone between May 2011 and June 2013 were reviewed. Pulmonary function, adverse events and treatment compliance were recorded at each clinic visit. Disease progression was defined as a reduction of vital capacity ≥10% and/or diffusion capacity (DLCO) ≥15%.
Results: Follow-up time on pirfenidone treatment was 11 (±7) months. Sixty-six percent of the patients continued with pirfenidone monotherapy and 34% of the patients received pirfenidone combined with corticosteroids (CCS) and/or N-acetylcysteine (NAC). There was a nonsignificant reduction in mean decline of percent predicted forced vital capacity after treatment start (0.7 ± 10.9%) compared to the pretreatment period (6.6 ± 6.7%, p = 0.098). Sixty-two percent of the patients had stable disease on pirfenidone treatment. Adverse events affected 85% of the patients, leading to discontinuation of pirfenidone in 20%. Adverse events and treatment discontinuation were seen more frequently in patients with concomitant CCS and/or NAC treatment.
Conclusions: Adverse events affect the majority of patients treated with pirfenidone, but are mostly manageable with supportive measures. In this heterogeneous patient group, a nonsignificant effect of pirfenidone treatment on pulmonary function was seen, underlining the need for more data on patient selection criteria and efficacy of pirfenidone, particularly in patients with coexistent emphysema and concomitant NAC/CCS treatment. © 2014 S. Karger AG, Basel.
Pulmonary manifestations of primary immunodeficiency disorders in children.
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Primary immunodeficiencies (PIDs) are inherited disorders in which one or several components of immune system are decreased, missing, or of non-appropriate function. These diseases affect the development, function, or morphology of the immune system. The group of PID comprises more than 200 different disorders and syndromes and the number of newly recognized and revealed deficiencies is still increasing. Their clinical presentation and complications depend on the type of defects and there is a great variability in the relationship between genotypes and phenotypes. A variation of clinical presentation across various age categories is also presented and children could widely differ from adult patients with PID. Respiratory symptoms and complications present a significant cause of morbidity and also mortality among patients suffering from different forms of PIDs and they are observed both in children and adults. They can affect primarily either upper airways (e.g., sinusitis and otitis media) or lower respiratory tract [e.g., pneumonia, bronchitis, bronchiectasis, and interstitial lung diseases (ILDs)]. The complications from lower respiratory tract are usually considered to be more important and also more specific for PIDs and they determinate patients' prognosis. The spectrum of the causal pathogens usually demonstrates typical pattern characteristic for each PID category. The respiratory signs of PIDs can be divided into infectious (upper and lower respiratory tract infections and complications) and non-infectious (ILDs, bronchial abnormalities - especially bronchiectasis, malignancies, and benign lymphoproliferation). Early diagnosis and appropriate therapy can prevent or at least slow down the development and course of respiratory complications of PIDs.
Brief exposure to cigarette smoke impairs airway epithelial cell innate anti-viral defence.
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Brief exposure to cigarette smoke impairs airway epithelial cell innate anti-viral defence.
Toxicol In Vitro. 2014 Aug 8;
Authors: Logan J, Chen L, Gangell C, Sly PD, Fantino E, Liu K
Abstract
BACKGROUND: Human rhinovirus (hRV) infections commonly cause acute upper respiratory infections and asthma exacerbations. Environmental cigarette smoke exposure is associated with a significant increase in the risk for these infections in children.
OBJECTIVE: To determine the impact of short-term exposure to cigarette smoke on innate immune responses of airway epithelial cells infected with hRV.
METHODS: A human bronchial epithelial cell line (HBEC-3KT) was exposed to cigarette smoke extract (CSE) for 30 minutes and subsequently infected with hRV serotype 1B. Viral-induced cytokine release was measured with alphaLISA and viral replication quantified by shed viral titer and intracellular viral copy 24 hours post-infection.
RESULTS: CSE induced a concentration-dependent decrease in CXCL10 (p<0.001) and IFN-β (p<0.001), with a 79% reduction at the highest dose with an associated 3-fold increase in shed virus. These effects were maintained when infection was delayed up to 24 hours post CSE exposure. Exogenous IFN-β treatment at t=0 after infection blunts the effects of CSE on viral replication (p<0.05).
CONCLUSION: A single exposure of 30 minutes to cigarette smoke results has a lasting impact on epithelial innate defence providing a plausible mechanism for the increase in respiratory infections seen in children exposed to second-hand tobacco smoke.
PMID: 25111775 [PubMed - as supplied by publisher]
Influence of the microbiota on vaccine effectiveness.
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Influence of the microbiota on vaccine effectiveness.
Trends Immunol. 2014 Aug 8;
Authors: Valdez Y, Brown EM, Finlay BB
Abstract
Studies of the relationship between the microbiome and the development and function of the immune system are demonstrating novel concepts that could significantly alter the way we treat disease and promote wellness. Several diseases, including inflammatory bowel disease, allergy/asthma, and diabetes, are associated with changes in composition of the microbiome. Recent findings suggest novel complex mechanisms by which the microbiome impacts immune cell development and differentiation. A major implication of these findings is that the composition of microbiome may ultimately affect vaccine efficacy. We explore here the potential role of the microbiome in vaccine responses in the context of our growing understanding of the relationship between the gastrointestinal microbiota, resident immune cell populations, and systemic immunity.
PMID: 25113637 [PubMed - as supplied by publisher]
Update on house dust mite immunotherapy: are more studies needed?
Update on house dust mite immunotherapy: are more studies needed?
Curr Opin Allergy Clin Immunol. 2014 Aug 11;
Authors: Nelson HS
Abstract
PURPOSE OF REVIEW: Although systematic reviews have confirmed the effectiveness of subcutaneous immunotherapy (SCIT) and sublingual immunotherapy (SLIT) for the treatment of allergic rhinitis and allergic asthma, a review of studies employing extracts of house dust mites (HDMs) for immunotherapy found no consensus on basic treatment parameters.
RECENT FINDINGS: Over the last 2 years nine additional reports on immunotherapy with HDM extract preparations have been published. Six studies were with SLIT, five for allergic rhinitis and one for allergic asthma. The other three studies were all with SCIT in children with allergic asthma.
SUMMARY: The publication of these nine studies supports the efficacy of HDM-SLIT for allergic rhinitis and asthma and for HDM-SCIT for asthma in children. The reported safety data are also reassuring, especially for SLIT.HDM-SLIT tablets under development will have optimal doses established in large, randomized, placebo-controlled trials. The HDM-SCIT trial in children with allergic asthma confirms the efficacy of a commercialized HDM preparation for injection immunotherapy. However, the information that is presented on dosing in the articles on SLIT-liquid is unsatisfactory, as doses are not presented in a form that the clinician can use to guide their practice.
PMID: 25115684 [PubMed - as supplied by publisher]