1,721,092 research outputs found
Medicina personalizzata e fibrosi polmonare idiopatica
No full textIdiopathic 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
Long-term management of IPF with pirfenidone - A clinical case study with 5 years follow-up
No full textIdiopathic pulmonary fibrosis (IPF) is a progressively fibrotic interstitial lung disease that is associated with a median survival of 2-5 years from initial diagnosis.To date, the search for an effective treatment has involved numerous clinical trials of investigational agents but without significant success. Nevertheless, research over the past 10 years has provided us with a wealth of information on its histopathology, diagnostic work-up, and a greater understanding of its pathophysiology. Specifically, IPF is no longer thought to be a predominantly pro-inflammatory disorder. Rather, the fibrosis in IPF is increasingly understood to be the result of a fibroproliferative and aberrant wound healing cascade. The development of therapeutic targets has therefore shifted in accordance with this paradigm change. Emerging clinical data from recently published and ongoing trials investigating new potential pharmacological agents should be considered in the routine clinical management of these patients. Based upon encouraging results from randomised-controlled trials showing a positive effect in slowing decline in pulmonary function and reducing disease progression, pirfenidone was approved in 2011 as the first treatment in patients with IPF. This case study describes the clinical course of a patient enrolled into the Phase III and open-label extension studies of pirfenidone
Genetic commonality between inflammatory bowel disease and sarcoidosis: the beginning of the end or the end of the beginning?
Full text linkCrohn’s disease (CD) and ulcerative colitis, collectively
known as inflammatory bowel disease (IBD), and
sarcoidosis are multifactorial disorders thought to
result from complex interactions between environmental
stimuli (e.g. infectious agents), susceptibility genes (which
may predispose to the development of granulomatous inflammation) and modifier genes (which may affect disease
phenotype in people already susceptible). Neither IBD nor
sarcoidosis is the result of defects in a single major gene or
chemical pathway; instead, multiple genes, each contributing a relatively minor effect, are likely to be involved. In addition
to the granulomatous histopathology, both diseases share a
number of similarities in terms of ocular, dermatological and
joint manifestations, although sarcoidosis rarely involves the
gastrointestinal tract and IBD rarely involves the lung.
Immunological, bacteriological and genetic data support a link
between CD and sarcoidosis. Both disorders share a similar,
yet distinct, immune response, histologically defined by
non-caseating granulomas. Up to 50% of patients with CD
have been reported to test positive for Kveim antigens,
although these data are not replicated in all studies
Tubercolosi
No full textL'infezione da micobatteri tubercolari ha accompagnato la storia dell'uomo sin dall'antichità. Nei secoli la malattia tubercolare ha rappresentato una piaga sociale, principale responsabile di morbilità e mortalità nella popolazione giovane adulta, e ancora oggi è tra le prime 10 cause di morte al mondo con circa 1,8 milioni di decessi all'anno
Tubercolosi
No full textL’infezione da micobatteri tubercolari ha accompagnato la storia dell’uomo sin dall’antichità. Nei secoli la malattia tubercolare ha rappresentato una piaga sociale, principale responsabile di morbidità e mortalità nella popolazione giovane adulta, e ancora oggi costituisce la prima causa di morte per singola malattia infettiva nel mondo, con circa 1,7 milioni di decessi all’anno (WHO, 2011). I progressi nello sviluppo di test diagnostici, nell’utilizzo di regimi terapeutici standardizzati efficaci e nell’adozione di programmi di controllo dell’infezione, hanno prodotto negli ultimi decenni un’inversione di tendenza degli indici di incidenza e prevalenza della malattia (WHO, 2011). Tuttavia, alcuni fattori, tra i quali la co-infezione da virus dell’HIV, il crescente problema dell’emergere di ceppi farmaco-resistenti, i flussi migratori da Paesi ad alta endemia e le condizioni di
immunodepressione (incluse quelle di natura iatrogena, in una popolazione generale che diventa sempre più anziana), rendono ragione della persistenza dell’infezione a livello globale (Dye et al., 2010)
Malattie infiltrative diffuse del polmone
No full textLe pneumopatie infiltrative diffuse (PID), comunemente ed erroneamente note come “polmoniti interstiziali” o “fibrosi polmonari”, sono tradizionalmente fonte di confusione, sia perché il termine “interstiziale” è fuorviante (infatti, queste patologie spesso non sono limitate allo spazio alveolo-capillare), sia perché non tutte le PID esitano in fibrosi. Ad oggi
sono state descritte oltre duecento entità nosologiche distinte, alcune ad esclusivo interessamento polmonare, altre in cui il polmone è solo uno degli organi colpiti. Singoli stimoli possono causare l’insorgenza di episodi acuti, ma è in seguito a un’esposizione ripetuta e protratta a uno o più di questi stimoli che la malattia assume un carattere cronico e persistente evolvendo in fibrosi nei casi più severi. Con riferimento all’eziopatogenesi, le PID possono essere classificate in quattro gruppi: da agenti irritanti, da cause immunologiche, idiopatiche e mimi di PID. Tra le PID sono incluse frequentemente, ma erroneamente, anche le polmoniti eosinofile e le vasculiti a prevalente interessamento
polmonare
Steroid and/or other immunosuppressive therapies in idiopathic interstitial pneumonias: have they still a role?
No full textThe idiopathic interstitial pneumonias (IIPs) comprise a number of clinico-pathological entities of unknown origin, which are sufficiently different from one another to be designated as separate disease entities. The aim of this review is to describe the level of evidence available on the efficacy of corticosteroids and immunosuppressive, antifibrotic, or immunomodulatory therapies in the treatment of different IIP
Rifabutin for treating pulmonary tuberculosis
No full textBackground: rifamycins are an essential component of modern short-course regimens for treating tuberculosis. Rifabutin has favourable pharmacokinetic and pharmacodynamic properties and is less prone to drug?drug interactions than rifampicin. It could contribute to shortening of therapy or simplify treatment in HIV-positive people who also need antiretroviral drugs.Objectives: to compare combination drug regimens containing rifabutin with those containing rifampicin for treating pulmonary tuberculosisSearch methods: we searched Cochrane Infectious Diseases Group Specialized Register (July 2009), CENTRAL (The Cochrane Library 2009, Issue 3), MEDLINE (1966 to July 2009), EMBASE (1974 to July 2009), and LILACS (1982 to July 2009). We also searched the Indian Journal of Tuberculosis (1983 to 2006), conference abstracts, reference lists, and unpublished data on file at Pfizer Inc.Selection criteria: randomized and quasi-randomized trials including participants with sputum smear and/or culture-confirmed tuberculosis that compared a rifabutin-containing with an otherwise identical rifampicin-containing regimen.Data collection and analysis: two authors independently assessed study eligibility and methodological quality, and extracted data. Dichotomous data were analysed and combined using relative risks (RR) with 95% confidence intervals (CI) using a fixed-effect model. Subgroup analyses were carried out according to rifabutin dose.Main results: five trials with a total of 924 participants met the inclusion criteria; 5% of participants were HIV positive. Only one small trial was methodologically adequate. The two largest trials (818 participants) had unclear allocation concealment and included < 90% of randomized participants in the analysis. There was no statistically significant difference in between the regimens for cure (RR 1.00, 95% CI 0.96 to 1.04; 553 participants, 2 trials) or relapse (RR 1.23, 95% CI 0.45 to 3.35; 448 participants, 2 trials). The number of adverse events was not significantly different (RR 1.42, 95% CI 0.88 to 2.31; 714 participants, 3 trials), though the RR increased with rifabutin dose: 150 mg (RR 0.98, 95% CI 0.45 to 2.12; 264 participants, 2 trials); and 300 mg (RR 1.78, 95% CI 0.94 to 3.34; 450 participants, 2 trials). However, lack of dose adjustment by weight in the relevant trials complicates interpretation of this relationship.Authors' conclusions: the replacement of rifampicin by rifabutin for first-line treatment of tuberculosis is not supported by the current evidence. HIV-positive people with tuberculosis, the group most likely to benefit from the rifabutin use, are under-represented in trials to date, and further trials in this group would be usefu
Challenges in idiopathic interstitial lung disease
No full textIdiopathic interstitial pneumonias (IIPs) are a group of pulmonary disorders with distinct histologic and radiologic appearances and no identiiable cause. The new classiication of IIPs published in 2013 distinguishes six distinct major entities, including chronic, usually progressive ibrosing diseases, such as idiopathic pulmonary ibrosis (IPF) and idiopathic nonspeciic interstitial pneumonia. IPF, an invariably progressive and ultimately fatal lung disease that occurs in older adults, is the most frequent among the IIPs. Recent evidence and international guidelines advocate the importance of chest high-resolution computed tomography and multidisciplinary discussion (MDD) in the initial diagnostic assessment of patients with suspected IPF. MDD is currently considered the gold standard because improves the accuracy of IIPs diagnosis, avoiding unnecessary testing, and optimizing patient management, particularly nowadays that two drugs have been approved by regulatory agencies for the treatment of IPF. In this review, we focus on the revised diagnostic criteria for IIPs and IPF and provide an overview of the most recent clinical trials. Finally, we stress the fact that NSIP, one of the most frequent differential diagnosis in cases presenting with suspected IPF, is not anymore considered a provisional entity, but a deinite clinical-pathological entity
Management of idiopathic pulmonary fibrosis
No full textIdiopathic 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
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