Medicina personalizzata e fibrosi polmonare idiopatica

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). IPF shows a high variability in the evolution from one patient to another, and between different periods in time in a given individual, showing great clinical heterogeneity. Therefore, predicting the outcome and the response to treatment in IPF is challenging, but potentially very useful, particularly in the single IPF patient. In the last decade, with the common use of proteomic and genomic technologies, our knowledge about the pathogenesis of the disease dramatically improved and it has led to the recognition of various treatment targets and numerous potential biomarkers. Molecular biomarkers are needed in IPF, where they can simplify drug development, facilitate early detection, increase prognostic accuracy and inform treatment recommendations. Although there are not yet validated biomarkers in IPF, some of them are in the proximity to be validated and have demonstrated their potential to improve clinical predictors beyond that of routine clinical practice

Occurrence of idiopathic pulmonary fibrosis during immunosuppressive treatment: a case report

Immunosuppressive therapy has been-until the recent release of new guidelines on diagnosis and management-the recommended treatment for idiopathic pulmonary fibrosis. However, its efficacy in patients with idiopathic pulmonary fibrosis has always been a matter of debate

BPCO e altre malattie polmonari croniche

Introduction: Chronic obstructive pulmonary disease (COPD) is characterized by a relatively irreversible airflow limitation caused by chronic inflammation, in most cases tobacco-related. The impact of COPD on morbidity and mortality at the single-patient level depends upon the severity of COPD symptoms and the existence of other types of systemic and/or pulmonary disease, also known as co-morbid conditions. Materials and methods: This review examines the pulmonary diseases commonly associated with COPD, in terms of their prevalence, clinical features, pathogenic mechanisms, prognoses, and implications for management of COPD. Results: The incidence and prevalence of various pulmonary diseases are significantly increased in patients with COPD. These conditions include symptomatic bronchiectasis, combined pulmonary fibrosis and emphysema, lung cancer, sleep-related respiratory disorders, and pulmonary embolism. Some of these concomitant respiratory diseases have an independent negative impact on the prognosis of COPD patients, and their presence has important implications for treatment of these patients. Conclusions: Physicians treating patients with COPD need to be aware of these coexisting pulmonary diseases. All patients with COPD should be carefully evaluated to identify pulmonary comorbidities, since they not only influence the prognosis but also have an impact on disease management. The treatment of COPD is no longer restricted exclusively to inhaled therapy. The therapeutic approach to this disease is becoming increasingly multidimensional in view of the fact that successful management of comorbidities might positively affect the course of COPD itself. \ua9 2011 Elsevier Srl. All rights reserved

Janus-faced amiodarone-induced pneumopathy

The authors describe a patient showing bilateral, peripheral, predominantly basal ground-glass and reticular opacities consistent with a non-specific interstitial pneumonia (NSIP) radiological pattern. This was followed by the occurrence of two nodules that progressively decreased in size after oral steroids had been given and therefore they were interpreted as an unusual manifestation of amiodarone-related pulmonary toxicity (APT)

Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy

open11siBackground Chronic, progressive respiratory symptoms are associated with great psychological and emotional impact in patients suffering from interstitial lung disease (ILD). This single-centre pilot study evaluated for the first time the safety, feasibility and efficacy of a Mindfulness Based Stress Reduction Program (MBSR) in a group of patients with ILD. Methods Prospective observational study set in a university hospital ILD outpatient clinic. Nineteen patients with different ILDs were recruited 2 months prior to the start of the 8-week MBSR program and followed up for 12 months. Primary outcomes were program safety and feasibility, while secondary outcomes were changes in moods and stress (assessed by Profile Of Mood State (POMS) and Perceived Stress Scale (PSS) questionnaires), symptoms (Shortness Of Breath (SOB) and Cough And Sputum Assessment (CASA-Q) questionnaires), lung function and exercise tolerance at 12 months. Results Two patients (10.5%) dropped out in the observational period before the start of the MBSR intervention because of non-respiratory causes. All 17 patients who entered the 8-week MBSR program managed to complete it with an adherence average of eight sessions of nine. No adverse events related to the mindfulness training were reported. Statistically significant improvements in the POMS total score and in several individual items of POMS and PSS were observed throughout the study. However, respiratory questionnaire scores, lung function and exercise tolerance did not show a significant difference over time. Conclusions An MBSR program appears to be safe and feasible in patients with ILD, and might affect perceived moods and stress producing a positive and lasting improvement in several stress-related negative domains. These findings pave the way to larger (possibly multicentre), randomised, controlled confirmatory trials.openSgalla, Giacomo; Cerri, Stefania; Ferrari, Roberto; Ricchieri, Maria Pia; Poletti, Stefano; Ori, Margherita; Garuti, Martina; Montanari, Gloria; Luppi, Fabrizio; Petropulacos, Kyriakoula; Richeldi, LucaSgalla, Giacomo; Cerri, Stefania; Ferrari, Roberto; Ricchieri, Maria Pia; Poletti, Stefano; Ori, Margherita; Garuti, Martina; Montanari, Gloria; Luppi, Fabrizio; Petropulacos, Kyriakoula; Richeldi, Luc

Bronchiectasis as long-term complication of acute fire smoke inhalation?

In this letter to editor, we discuss the occurrence of radiological and clinical evidence of bronchiectasis syndrome three years after acute exposure to fire smoke in a Caucasian non-smoker asthmatic patient

Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Lights and Shadows

Rheumatoid arthritis (RA) is a chronic and systemic inflammatory disease affecting 0.5-1% of the population worldwide. Interstitial lung disease (ILD) is a serious pulmonary complication of RA and it is responsible for 10-20% of mortality, with a mean survival of 5-8 years. However, nowadays there are no therapeutic recommendations for the treatment of RA-ILD. Therapeutic options for RA-ILD are complicated by the possible pulmonary toxicity of many disease modifying anti-rheumatic drugs (DMARDs) and by their unclear efficacy on pulmonary disease. Therefore, joint and lung involvement should be evaluated independently of each other for treatment purposes. On the other hand, some similarities between RA-ILD and idiopathic pulmonary fibrosis and the results of the recent INBIULD trial suggest a possible future role for antifibrotic agents. From this perspective, we review the current literature describing the pulmonary effects of drugs (immunosuppressants, conventional, biological and target synthetic DMARDs and antifibrotic agents) in patients with RA and ILD. In addition, we suggest a framework for the management of RA-ILD patients and outline a research agenda to fill the gaps in knowledge about this challenging patient cohort

Diagnosis of occult tuberculosis in hematological malignancy by enumeration of antigen-specific T cells.

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Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

There are over 200 interstitial lung diseases (ILDs). In addition to patients with idiopathic pulmonary fibrosis (IPF), a percentage of patients with other ILDs also develop progressive fibrosis of the lung during their disease course. Patients with progressive-fibrosing ILDs may show limited response to immunomodulatory therapy, worsening symptoms and lung function and, ultimately, early mortality. There are few data for ILDs that may present a progressive fibrosing phenotype specifically, but we believe the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF. This review discusses the burden of ILDs that may present a progressive fibrosing phenotype and the factors impacting healthcare utilisation