Pirfenidone for the treatment of Hermansky–Pudlak syndrome pulmonary fibrosis
نویسندگان
چکیده
منابع مشابه
Pirfenidone treatment of idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) is a discrete clinicopathologic entity defined by the presence of usual interstitial pneumonia on high-resolution CT scan and/or open lung biopsy and the absence of an alternate diagnosis or exposure explaining these findings. There are currently no FDA-approved therapies available to treat this disease, and the 5-year mortality is ∼80%. The pyridone derivati...
متن کاملPirfenidone in idiopathic pulmonary fibrosis.
We read with interest the article of TANIGUCHI et al. [1] entitled ‘‘Pirfenidone in idiopathic pulmonary fibrosis’’. These authors have spearheaded the investigation of this novel antifibrotic agent for idiopathic pulmonary fibrosis, and their efforts are commendable. In an editorial that accompanied their manuscript, COLLARD [2] pointed out a number of shortcomings of the study that raise conc...
متن کاملPirfenidone in idiopathic pulmonary fibrosis.
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease without proven effective therapy. A multicentre, double-blind, placebo-controlled, randomised phase III clinical trial was conducted in Japanese patients with well-defined IPF to determine the efficacy and safety of pirfenidone, a novel antifibrotic oral agent, over 52 weeks. Of 275 patients randomised (high-dose, 1,800 mg x day(...
متن کاملIdiopathic pulmonary fibrosis and pirfenidone.
T he last decade has seen important progress in the clinical investigation of idiopathic pulmonary fibrosis (IPF). Basic insights into mechanisms of fibroproliferation have been translated into novel investigational agents, networks of clinical centres capable of enrolling hundreds of patients in research studies have been developed, and multiple high-quality treatment trials have been successf...
متن کاملPirfenidone treatment in idiopathic pulmonary fibrosis: nationwide Danish results
BACKGROUND Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect to target population, safety, adherence to the treatment and effect analysis in a well-characterised IPF ...
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ژورنال
عنوان ژورنال: Molecular Genetics and Metabolism
سال: 2011
ISSN: 1096-7192
DOI: 10.1016/j.ymgme.2011.02.003