Idiopathic pulmonary fibrosis (IPF) is a debilitating condition with life expectancy of two to five years from diagnosis. Treatment strategies for IPF are disappointingly limited and pirfenidone is currently the only licensed drug that has been shown to reduce the decline in forced vital capacity (FVC) at six months. We demonstrate our experience in prescribing pirfenidone in a single centre observational study of forty patients involved in a named patient programme (NPP) from September 2011 to January 2013. We demonstrate that improved adherence and compliance can be achieved by specialist nurse and clinician review, support and education of the patient. Twenty three of 40 (58%) patients experienced predominantly gastrointestinal adverse effects. Importantly we have enhanced patient adherence and compliance from an initial discontinuation rate of six patients (15%) at the beginning of the study to a zero discontinuation rate in the subsequent ten months. This study shows that in the real world pirfenidone is well tolerated and with expert regular specialist review adherence can be optimised and improved.
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http://dx.doi.org/10.1016/j.rmed.2013.11.005 | DOI Listing |
ACS Pharmacol Transl Sci
January 2025
School of Pharmaceutical Sciences, Sun Yat-Sen University, Guangzhou 510006, P. R. China.
Idiopathic pulmonary fibrosis (IPF) is a debilitating, incurable, and life-threatening disease that lacks effective therapy. The overexpression of phosphodiesterase 10A (PDE10A) plays a vital role in pulmonary fibrosis (PF). However, the impact of selective PDE10A inhibitors on the tumor growth factor-β (TGF-β)/small mother against decapentaplegic (Smad) signaling pathway remains unclear.
View Article and Find Full Text PDFCells
December 2024
Biotherapeutics Enabling Biology, Lilly Research Laboratories, Eli Lilly and Company, Indianapolis, IN 46225, USA.
Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease with unknown etiology, characterized by chronic inflammation and tissue scarring. Although, Pirfenidone and Nintedanib slow the disease progression, no currently available drugs or therapeutic interventions address the underlying cause, highlighting the unmet medical need. A matricellular protein, Wnt-1-induced secreted protein 1 (WISP1), also referred to as CCN4 (cellular communication network factor 4), is a secreted multi-modular protein implicated in multi-organ fibrosis.
View Article and Find Full Text PDFNaunyn Schmiedebergs Arch Pharmacol
December 2024
Department of Thyroid and Breast Surgery, The Affiliated Nanhua Hospital, Hengyang Medical School, University of South China, Hengyang, 421000, Hunan, China.
Immun Inflamm Dis
November 2024
Department of Respiratory and Critical Care Medicine, Beijing Luhe Hospital, Capital Medical University, Beijing, China.
Objective: Smoke inhalation lung injury (SILI) is a common complication in fires and wars, characterized by acute onset and severe condition. Pirfenidone (PFD), a new small-molecule drug, has been shown to improve lung function and inhibit pulmonary fibrosis and inflammation. This study aimed to elucidate the effect and underlying mechanism of PFD on SILI in rats.
View Article and Find Full Text PDFWorld J Clin Cases
November 2024
Department of Clinical Pharmacy, Faculty of Pharmacy, Beni-Suef University, Beni-Suef 343433, Egypt.
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