Effect of Recombinant Human Pentraxin 2 vs Placebo on Change in Forced Vital Capacity in Patients With Idiopathic Pulmonary Fibrosis

Raghu, Ganesh; Blink, Bernt van den; Hamblin, Mark J.; Brown, Alison; Golden, Jeffrey A.; Ho, Lawrence; Wijsenbeek, Marlies; Vašáková, Martina; Pesci, Alberto; Antin‐Ozerkis, Danielle; Meyer, Keith C.; Kreuter, Michael; Santin-Janin, Hugues; Mulder, Geert Jan; Bartholmai, Brian J.; Gupta, Renu; Richeldi, Luca

doi:10.1001/jama.2018.6129

Cited by 198 publications

(168 citation statements)

References 24 publications

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“…Studies investigating effectiveness of pirfenidone in AE-IPF and studies investigating its synergistic effect with novel compounds that entered the pipeline of clinical trials (i.e. pamrevlumab and pentraxin) are greatly anticipated [29][30][31] and will hopefully transform the therapeutic management of patients.…”

Section: Discussionmentioning

confidence: 99%

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena

Vietri

Cameli

Perruzza

et al. 2020

Ther Adv Respir Dis

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Background: Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2–5 years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone. Methods: We enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, survival and functional details were collected retrospectively at start of therapy and after 12, 24, 36 and 48 months of treatment. Lung function tests at least 12 months before starting therapy were available for 40 patients and were entered in the database, as well as side effects. Results: During the observation period (922 ± 529 days), 27 patients died, 5 patients underwent lung transplant and 10 patients interrupted therapy due to adverse events or IPF progression. The median survival was 1606 days. There was a significant reduction in disease progression rate, as measured by trend of forced vital capacity, after 1 year of therapy with respect to before treatment ( p = 0.0085). Forced vital capacity reduction rate was progressively higher in the subsequent years of treatment. Treatment-related side effects were reported in 25 patients and were predominantly mild. Overall, four patients discontinued therapy due to severe photosensitivity. Conclusions: Our findings confirm the efficacy of pirfenidone in reducing functional progression of IPF and its excellent safety profile in a real-life setting. This study, designed on a long-term follow up, contributes to the growing evidence on safety, tolerability and efficacy of pirfenidone in IPF. The reviews of this paper are available via the supplemental material section.

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Section: Discussionmentioning

confidence: 99%

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena

Vietri

Cameli

Perruzza

et al. 2020

Ther Adv Respir Dis

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“…We helped form a company called Promedior to pursue this. Promedior found that SAP is an effective therapeutic in 20 different fibrosis models, and SAP showed better efficacy and fewer side effects than current therapeutics in a Phase 1B trial and a 117-patient Phase 2 trial for pulmonary fibrosis, and a Phase 2 trial for myelofibrosis, a bone marrow fibrosis (Dillingh et al, 2013, Raghu et al, 2018. If SAP continues to be effective in further clinical trials, it would be very helpful for fibrosis patients and an example of taking Dictyostelium secreted factor research into the clinic.…”

Section: Development Of Potential Therapeutics Based On Dictyosteliummentioning

confidence: 99%

Extracellular signaling in Dictyostelium

Consalvo

Rijal²,

Tang³

et al. 2019

Int. J. Dev. Biol.

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In the last few decades, we have learned a considerable amount about how eukaryotic cells communicate with each other, and what it is the cells are telling each other. The simplicity of Dictyostelium discoideum, and the wide variety of available tools to study this organism, makes it the equivalent of a hydrogen atom for cell and developmental biology. Studies using Dictyostelium have pioneered a good deal of our understanding of eukaryotic cell communication. In this review, we will present a brief overview of how Dictyostelium cells use extracellular signals to attract each other, repel each other, sense their local cell density, sense whether the nearby cells are starving or stressed, count themselves to organize the formation of structures containing a regulated number of cells, sense the volume they are in, and organize their multicellular development. Although we are probably just beginning to learn what the cells are telling each other, the elucidation of Dictyostelium extracellular signals has already led to the development of possible therapeutics for human diseases.

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“…These brilliant results raised the hope for finding a definitive cure for IPF and paved the way for newer studies exploring the effectiveness of different molecules. Some of them, such as human recombinant pentraxin 2, PBI‐4050 and GLPG1690, showed promising results in phase 2 studies. Currently, the research landscape is highly flourishing.…”

Section: Disclosure Statementmentioning

confidence: 99%