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Luca Richeldi

Martin Kolb, Luca Richeldi, Jürgen Behr, Toby M Maher, Wenbo Tang, Susanne Stowasser, Christoph Hallmann, Roland M du Bois
RATIONALE: There is no consensus as to when treatment for idiopathic pulmonary fibrosis (IPF) should be initiated. Some physicians prefer not to treat patients with preserved lung volume. OBJECTIVE: To investigate whether patients with IPF and preserved lung volume receive the same benefit from nintedanib as patients with more impaired lung volume. METHODS: Post hoc subgroup analyses of pooled data from the two replicate phase III INPULSIS trials by baseline FVC % predicted (≤90%, >90%)...
September 26, 2016: Thorax
Sophie Fletcher, Mark G Jones, Katherine Spinks, Giacomo Sgalla, Ben G Marshall, Rachel Limbrey, Luca Richeldi
The management of idiopathic pulmonary fibrosis (IPF) has been transformed by the recent approval of two anti-fibrotic drugs, nintedanib and pirfenidone. An increasing number of patients with IPF are receiving treatment with these novel therapies, and the risk of adverse events that may be associated with their use must be carefully evaluated. Areas covered: Safety data about nintedanib and pirfenidone is critically evaluated, including data from randomized clinical trials and post-marketing reports. Management strategies to minimize the occurrence of side effects are summarized...
November 2016: Expert Opinion on Drug Safety
L Richeldi
The recent approval of two safe and effective treatments for patients with idiopathic pulmonary fibrosis (IPF) had as a direct consequence the absolute need for an accurate and early diagnosis. The standard approach to IPF diagnosis has proven to be effective and emphasized the importance of clinical and laboratory evaluations to exclude known causes of pulmonary fibrosis. At the same time, chest high-resolution computed tomography (HRCT) has proven to be the crucial initial diagnostic test, by identifying those patients who should undergo surgical lung biopsy to secure a confident diagnosis and an adequate treatment...
September 2016: QJM: Monthly Journal of the Association of Physicians
Stefania Cerri, Giacomo Sgalla, Luca Richeldi, Fabrizio Luppi
BACKGROUND: 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. CASE PRESENTATION: We report the occurrence of idiopathic pulmonary fibrosis in a white man receiving chronic immunosuppressive treatment following a heart transplant. CONCLUSIONS: This case report suggests that the immune mechanisms targeted by azathioprine and cyclosporine do not play a role in the pathogenesis of idiopathic pulmonary fibrosis...
2016: Journal of Medical Case Reports
Ganesh Raghu, Athol U Wells, Andrew G Nicholson, Luca Richeldi, Kevin R Flaherty, Florence Le Maulf, Susanne Stowasser, Rozsa Schlenker-Herceg, David M Hansell
RATIONALE: In the absence of a surgical lung biopsy, patients diagnosed with idiopathic pulmonary fibrosis (IPF) in clinical practice could participate in the INPULSIS® trials of nintedanib if they had honeycombing and/or traction bronchiectasis plus reticulation, without atypical features of usual interstitial pneumonia (UIP), on high-resolution computed tomography (HRCT). Thus the patients in these trials represented patients with definite UIP and a large subgroup of patients with possible UIP...
June 22, 2016: American Journal of Respiratory and Critical Care Medicine
Harold R Collard, Christopher J Ryerson, Tamera J Corte, Gisli Jenkins, Yasuhiro Kondoh, David J Lederer, Joyce S Lee, Toby M Maher, Athol U Wells, Katerina M Antoniou, Juergen Behr, Kevin K Brown, Vincent Cottin, Kevin R Flaherty, Junya Fukuoka, David M Hansell, Takeshi Johkoh, Naftali Kaminski, Dong Soon Kim, Martin Kolb, David A Lynch, Jeffrey L Myers, Ganesh Raghu, Luca Richeldi, Hiroyuki Taniguchi, Fernando J Martinez
Acute exacerbation of idiopathic pulmonary fibrosis has been defined as an acute, clinically significant, respiratory deterioration of unidentifiable cause. The objective of this international working group report on acute exacerbation of idiopathic pulmonary fibrosis was to provide a comprehensive update on the topic. A literature review was conducted to identify all relevant English text publications and abstracts. Evidence-based updates on the epidemiology, etiology, risk factors, prognosis, and management of acute exacerbations of idiopathic pulmonary fibrosis are provided...
August 1, 2016: American Journal of Respiratory and Critical Care Medicine
Mark G Jones, Aurélie Fabre, Philipp Schneider, Francesco Cinetto, Giacomo Sgalla, Mark Mavrogordato, Sanjay Jogai, Aiman Alzetani, Ben G Marshall, Katherine M A O'Reilly, Jane A Warner, Peter M Lackie, Donna E Davies, David M Hansell, Andrew G Nicholson, Ian Sinclair, Kevin K Brown, Luca Richeldi
In idiopathic pulmonary fibrosis (IPF), the fibroblast focus is a key histological feature representing active fibroproliferation. On standard 2D pathologic examination, fibroblast foci are considered small, distinct lesions, although they have been proposed to form a highly interconnected reticulum as the leading edge of a "wave" of fibrosis. Here, we characterized fibroblast focus morphology and interrelationships in 3D using an integrated micro-CT and histological methodology. In 3D, fibroblast foci were morphologically complex structures, with large variations in shape and volume (range, 1...
April 21, 2016: JCI Insight
Mark G Jones, Luca Richeldi
Interstitial lung diseases (ILDs) are a diverse range of conditions affecting the lung interstitium. The prototypic ILD, idiopathic pulmonary fibrosis (IPF), is a chronic progressive fibrotic lung disease with a median survival of only 3 years from the time of diagnosis. Recently significant progress has been made in both our understanding of the pathogenesis and of the therapeutic targeting of IPF. This culminated in the worldwide approval of the first antifibrotic therapies nintedanib and pirfenidone. While an important first step, patients continue to progress and better therapies are urgently required...
June 2016: Seminars in Respiratory and Critical Care Medicine
Jay H Ryu, Luca Richeldi
No abstract text is available yet for this article.
June 2016: Seminars in Respiratory and Critical Care Medicine
Luca Richeldi
No abstract text is available yet for this article.
May 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Martin Kolb, Gisli Jenkins, Luca Richeldi
No abstract text is available yet for this article.
May 2016: Thorax
Mark G Jones, Simon L F Walsh, Kirk D Jones, Luca Richeldi
No abstract text is available yet for this article.
April 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Robert Brownell, Naftali Kaminski, Prescott G Woodruff, Williamson Z Bradford, Luca Richeldi, Fernando J Martinez, Harold R Collard
Precision medicine is defined by the National Institute of Health's Precision Medicine Initiative Working Group as an approach to disease treatment that takes into account individual variability in genes, environment, and lifestyle. There has been increased interest in applying the concept of precision medicine to idiopathic pulmonary fibrosis, in particular to search for genetic and molecular biomarker-based profiles (so called endotypes) that identify mechanistically distinct disease subgroups. The relevance of precision medicine to idiopathic pulmonary fibrosis is yet to be established, but we believe that it holds great promise to provide targeted and highly effective therapies to patients...
June 1, 2016: American Journal of Respiratory and Critical Care Medicine
Luca Richeldi, Vincent Cottin, Roland M du Bois, Moisés Selman, Toshio Kimura, Zelie Bailes, Rozsa Schlenker-Herceg, Susanne Stowasser, Kevin K Brown
BACKGROUND AND PURPOSE: The Phase II TOMORROW trial and two Phase III INPULSIS(®) trials investigated the efficacy and safety of nintedanib versus placebo in patients with idiopathic pulmonary fibrosis (IPF). To obtain an overall estimate of the treatment effect of nintedanib 150 mg twice daily (bid), pooled and meta-analyses of data from these three trials were conducted. METHODS: Pooled and meta-analyses were conducted for annual rate of decline in forced vital capacity (FVC), time to first acute exacerbation, change from baseline in St George's Respiratory Questionnaire (SGRQ) total score and mortality over 52 weeks...
April 2016: Respiratory Medicine
Giacomo Sgalla, Elisabetta Cocconcelli, Roberto Tonelli, Luca Richeldi
Idiopathic Pulmonary Fibrosis (IPF) is a progressive, fatal lung disorder of unknown cause with a highly variable and unpredictable clinical course. The advances made in deciphering IPF pathobiology over the last decades have led to the approval of two anti-fibrotic molecules, pirfenidone and nintedanib, that showed to be effective in significantly reducing the rate of progression of the disease. Such pharmacological breakthroughs represent a dramatic change in the management of these patients and are reflected in updated international guidelines...
February 26, 2016: Expert Review of Respiratory Medicine
Bram Rochwerg, Binod Neupane, Yuan Zhang, Carlos Cuello Garcia, Ganesh Raghu, Luca Richeldi, Jan Brozek, Joseph Beyene, Holger Schünemann
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease associated with high morbidity and mortality. Effective treatments for IPF are limited. Several recent studies have investigated novel therapeutic agents for IPF, but very few have addressed their comparative benefits and harms. METHODS: We performed a Bayesian network meta-analysis (NMA) to assess the effects of different treatments for IPF on mortality and serious adverse events (SAEs)...
2016: BMC Medicine
Nicky Britten, Catherine Pope, Susan Halford, Luca Richeldi
No abstract text is available yet for this article.
January 2016: Lancet Respiratory Medicine
Luca Richeldi
Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), with an annual incidence up to 20% and a short-term mortality around 50%, are the more frequent cause of death in patients with IPF. These unpredictable and clinically relevant events are more frequent in patients with advanced disease, as measured by a lower lung function. In the absence of an evidence-based, approved treatment of AE-IPF, patients with IPF with acute respiratory worsening are usually treated with systemic high-dose corticosteroids according to current guideline recommendations...
November 2015: Annals of the American Thoracic Society
Giacomo Sgalla, Alice Biffi, Luca Richeldi
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing lung disorder of unknown aetiology whose diagnosis involves the careful exclusion of secondary causes for pulmonary fibrosis and the presence of a pattern of usual interstitial pneumonia (UIP) at either high-resolution computed tomography (HRCT) scan or surgical lung biopsy. Despite great efforts made in establishing precise, universally acknowledged diagnostic criteria for IPF, its ascertainment remains a challenge, especially in those individuals presenting with atypical HRCT patterns...
April 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
Sara De Biasi, Stefania Cerri, Elena Bianchini, Lara Gibellini, Elisa Persiani, Gloria Montanari, Fabrizio Luppi, Cristiano Matteo Carbonelli, Luigi Zucchi, Marialuisa Bocchino, Alessandro Sanduzzi Zamparelli, Carlo Vancheri, Giacomo Sgalla, Luca Richeldi, Andrea Cossarizza
BACKGROUND: It has been suggested that circulating fibrocytes and endothelial cells actively participate in the intense remodelling of the pulmonary vasculature in patients with idiopathic pulmonary fibrosis (IPF). Indeed, fibrotic areas exist that have fewer blood vessels, whereas adjacent non-fibrotic tissue is highly vascularized. The number of circulating endothelial cells (CEC) and endothelial progenitor cells (EPC) might reflect the balance between vascular injury and repair. Thus, fibrocytes as well as endothelial cells could potentially be used as biomarkers of disease progression and treatment outcome...
2015: BMC Medicine
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