Pharmacological management of IPF

被引：35

作者：

Borie, Raphael ^{[1
,2
,3
]}

Justet, Aurelien ^{[1
,2
,3
]}

Beltramo, Guillaume ^{[1
,2
,3
]}

Manali, Effrosyni D. ^{[4
]}

Pradere, Pauline ^{[1
,2
,3
]}

Spagnolo, Paolo ^{[5
,6
]}

Crestani, Bruno ^{[1
,2
,3
]}

机构：

[1] Hop Xavier Bichat, AP HP, Ctr Competence Malad Pulm Rares, Dept Pneumol A,DHU Fire, Paris, France

[2] INSERM, Unit 1152, Paris, France

[3] Paris Diderot Univ, Paris, France

[4] Univ Athens, Athens Med Sch, Attikon Univ Hosp, Resp Med Dept 2, Athens 11528, Greece

[5] Univ Padua, Resp Dis Unit, Padua, Italy

[6] Univ Padua, Dept Cardiac Thorac & Vasc Sci, Padua, Italy

来源：

RESPIROLOGY | 2016年 / 21卷 / 04期

关键词：

cough; exacerbation; nintedanib; pirfenidone; pulmonary hypertension; IDIOPATHIC PULMONARY-FIBROSIS; PLACEBO-CONTROLLED TRIAL; CLINICAL-PRACTICE; N-ACETYLCYSTEINE; RANDOMIZED-TRIAL; GASTROESOPHAGEAL-REFLUX; ACUTE EXACERBATION; PHASE-3; TRIAL; DOUBLE-BLIND; PIRFENIDONE;

D O I：

10.1111/resp.12778

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Idiopathic pulmonary fibrosis (IPF) is a deadly disease with a median survival of approximately three years in historical cohorts. Despite increased knowledge of disease pathophysiology and selection of more targeted therapy, main clinical trials yielded negative results. However, two agents, pirfenidone and nintedanib, were recently shown to be effective in IPF and received marketing authorization worldwide. Both drugs significantly reduce functional decline and disease progression with an acceptable safety profile. Yet, none of these drugs actually improves or even stabilizes the disease or the symptoms perceived by the patient. Several other treatments and combinations are currently tested, and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.

引用

页码：615 / 625

页数：11

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