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2008年10月17日，日本厚生劳动省批准Shionogi公司关于吡非尼酮（pirfenidone；pirespa ）用于治疗特发性肺纤维化（IPF）在日本上市申请。本品制剂规格为200mg/片，由日本Shionogi 公司从美国Marnac公司获得许可，用于日本IPF市场的开发，其许可的权利范围还包括台湾和南韩。吡非尼酮为胶原合成抑制剂具有抗炎、抗氧化和抗纤维化作用。
pharmscope.com---InterMune公司5月9日宣称，治疗肺纤维化的药物吡非尼酮的II期临床结果显示，药物对于特发性肺纤维化（IPF）患者的疗效要优于泼尼松。这项临床试验的研究结果与以前所报道的在美国进行的开放标签及在日本进行的临床试验数据一致。随机双盲，多中心的II期临床试验入组了53名中重度IPF患者（n=46）或次级肺纤维化患者（n=7）。病人每日分三次口服40mg/kg的吡非尼酮或是0.33mg/kg的泼尼松。在几个疗效终点指标中，吡非尼酮对于IPF患者均显示出比泼尼松更好的治疗效果。尽管由于本次研究属于探索性的，并没有一个预先定义的主要疗效终点指标。比较基线状态至治疗12个月后，最小氧饱和度（6分钟步行测验中，对病人维持血中氧浓度能力的一个测试指标）的变化可以看见。吡非尼酮组的结果要显著优于泼尼松组（p=0.014）。在12个月时，与基线状态比较，其他几种疗效结果也显示出吡非尼酮治疗组要优于泼尼松组。吡非尼酮是一种口服具有活性的小分子药物。它能够抑制胶原的合成，减少多种细胞因子的产生，抑制成纤维细胞的繁殖及其响应细胞因子的兴奋作用。这种已被证明对多发性纤维化症状有活性的吡非尼酮目前正处于治疗肺、肾脏、肝脏及心脏纤维化疾病的II期临床试验中。除日本，韩，台湾地区以外，InterMune公司在全球拥有开发及上市吡非尼酮治疗所有的纤维化疾病的权限。 Pirfenidone is an orally active small molecule drug that may inhibit collagen synthesis, down regulate production of multiple cytokines and block fibroblast proliferation and stimulation in response to cytokines. Pirfenidone has demonstrated activity in multiple fibrotic conditions, including those of the lung, kidney and liver. Investigational Activity Pirfenidone for idiopathic pulmonary fibrosis (IPF) has been studied in multiple Phase 2 clinical trials. 以下是转载美国肺纤维化基金会（Pulmonary Fibrosis Foundation）关于肺纤维化疾病症状及治疗的一篇科普性介绍 What are the symptoms?
What is the prevalence of Pulmonary Fibrosis? There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same as die from Breast Cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group. What are the causes? Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following: · Inhaled environmental and occupational pollutants · Cigarette smoking · Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis · Certain medications · Therapeutic radiation How is it treated? There are currently no effective treatments or a cure for Pulmonary Fibrosis (目前还没有治愈肺纤维化有效的方法).The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress. Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly. There are a number of new trials testing drugs to treat Pulmonary Fibrosis. 特発性肺線維症は、治癒が困難な慢性の進行性疾患です。 症状の改善にはいたらないまでも、悪化を阻止することが治療の目標となります。
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