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

Smoking-related interstitial lung disease

Cigarette smoking has a clear epidemiological association with lung diseases, characterised by chronic inflammation of both the bronchiolar and the interstitial lung compartments. There are several different smoking-related interstitial lung diseases, mainly desquamative interstitial pneumonia, respiratory bronchiolitis- associated interstitial lung disease and pulmonary Langerhans’ cell histiocytosis.The epidemiology of such diseases is largely unknown, although the prevalence of cigarette smoking, particularly in low-income developing countries, indicates that smoking-induced interstitial lung disorders represent a high burden of disease worldwide. The role of chest high-resolution computed tomography has become increasingly important in differential diagnosis and follow-up. A new entity, the syndrome of combined pulmonary fibrosis and emphysema, emerged as another important smoking-related lung disorder with a poor prognosis, associated with the high prevalence of pulmonary hypertension. At the moment the role of anti-inflammatory and immunosuppressive treatment remains unclear, although in clinical practice most of these patients will receive at least one course of corticosteroid therapy. It is vital to stress the importance of identifying these patients and helping them quit smoking

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

Management of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a deadly progressive lung disease without an effective standard treatment approach. Because of the complexity and uncertainties of IPF treatment, therapeutic decisions need to be tailored to the individual patient, after discussing the potential benefits and pitfalls. Pirfenidone has been approved for the treatment of IPF in many countries, but is not recommended as a first-choice therapy by current guidelines because of the lack of a definite efficacy. Randomized controlled trials represent a valid choice for patients with IPF, and their completion is important in improving both survival and quality of life

Sarcoidosis: challenging diagnostic aspects of an old disease.

Over the past few years, there have been substantial advances in our understanding of sarcoidosis immunopathogenesis. Conversely, the etiology of the disease remains obscure for a number of reasons, including heterogeneity of clinical manifestations, often overlapping with other disorders, and insensitive and nonspecific diagnostic tests. While no cause has been definitely confirmed, there is increasing evidence that one or more infectious agents may cause the disease, although the organism may no longer be viable. Here we present 2 cases, in which sarcoidosis preceded tuberculosis and non-Hodgkin lymphoma. Development of new lesions in a patient with chronic/remitting sarcoidosis should be looked at with suspicion and promptly investigated in order to rule out an alternative/concomitant diagnosis. In such cases, tissue confirmation from the most accessible site, and bone marrow biopsy-if lymphoma is in the differential diagnosis-should be performed. In conclusion, we strongly advise that physicians be ready to reconsider the diagnosis of sarcoidosis in the presence of atypical manifestations or persistent/progressive disease despite conventional therapy

Lung fibrosis, bone marrow fibrosis and liver cirrhosis: A Short Telomere Syndrome or a casual association?

Background: Telomere-mediated disease has diverse presentations that span the age spectrum. Their type, age of onset, and severity depend on the extent of the telomere length defect. During adult life, telomerase mutations may represent risk factors rather than genetic determinants and need other factors to contribute to disease development. This is case of diseases such as aplastic anemia, pulmonary fibrosis and liver cirrhosis which may occur as single disease or together in a syndromic clustering. Here we report a case of a man most likely affected by a short telomere syndrome. Case report: A 58 years old man, presented for evaluation of pulmonary fibrosis diagnosed few years earlier in a different medical center. He also presented a mild bone marrow fibrosis and a liver cirrhosis, both diagnosed one year prior evaluation with a bone marrow analysis and liver biopsy. The patient was an active smoker, obese, with digital clubbing and inspiratory Velcro crackles at the right lower lobe. Laboratory tests showed thrombocytopenia and liver enzymes alteration. He rapidly developed ascites and progression of the pulmonary fibrosis, the patient became oxygen-dependent in few months. Methods: Sequencing and mutation analysis of hTERT and hTERC genes, Leukocyte Telomere length (LTL) and Telomerase activity (TA) were evaluated. Results: In our patient LTL was shorter and TA reduced compared to the controls. Genes sequencing did not show any hTERT and hTERC mutations. Conclusions: This is a report on a short telomere syndrome involving lung, liver and bone marrow, associated to very short telomere and absent telomerase activity not in the setting of dyskeratosis congenita. The fact that short telomeres mediate inflammation and fibrosis provides a rationale for pursuing translational strategies aimed at preventing telomere shortening or its cellular consequences as a therapeutic approac

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

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)

the bartolomeo ammannati s fountain an artifact in progress

Abstract Artifacts are not only fundamental evidences of our history and culture, but they are even entities having a proper "life". The present research focuses on Bartolomeo Ammannati's Juno Fountain (1555) – a Late-Renaissance masterpiece whose eventful story made it moving around from its planned site, the "Sala Grande" in Florentine Palazzo Vecchio, to Pratolino Park, then to Boboli Garden. Finally, current fragments re-assembling and museography staging under the vaults of the National Museum of Bargello court in Florence has been set up a few years ago on the 5th centenary of Ammannati's birthdate – after careful historical research about the many vicissitudes of the Fountain. Although there isn't any location change expected for this Ammannati's artwork, investigations and researches are going on. Namely, the seismic performance of the reconstructed Fountain is to be checked with reference to the seismic hazard of the site, as provided by the Italian Code classification. To this objective, the previously done laser scanning which allowed a three-dimensional digital modeling to help re-assembling the Fountain, has been now adopted to perform the structural analysis. Consequently, a structural evaluation to check the setting's seismic behavior is currently under process. The research, developed by joining different knowledges and fields, is an example of the importance of a multidisciplinary approach for preserving artifacts and museums' collections