Newer developments in idiopathic pulmonary fibrosis in the era of anti-fibrotic medications

被引：10

作者：

Nair, Girish B. ^{[1
,2
]}

Matela, Ajsza ^{[1
]}

Kurbanov, Daniel ^{[1
]}

Raghu, Ganesh ^{[3
]}

机构：

[1] Winthrop Univ Hosp, Div Pulm & Crit Care Med, Mineola, NY 11501 USA

[2] SUNY Stony Brook, Sch Med, Dept Med, Stony Brook, NY 11794 USA

[3] Univ Washington, Dept Med & Lab Med Adjunct, Div Pulm & Crit Care Med, Seattle, WA 98195 USA

来源：

EXPERT REVIEW OF RESPIRATORY MEDICINE | 2016年 / 10卷 / 06期

关键词：

Idiopathic pulmonary fibrosis; Pulmonary fibrosis; novel targets; pirfenidone; nintedanib; GROWTH-FACTOR-BETA; MUC5B PROMOTER POLYMORPHISM; MUSCLE ACTIN EXPRESSION; N-ACETYLCYSTEINE; EXTRACELLULAR-MATRIX; GENE-EXPRESSION; TRANSFORMING GROWTH-FACTOR-BETA-1; MYOFIBROBLAST DIFFERENTIATION; GASTROESOPHAGEAL-REFLUX; INTERSTITIAL PNEUMONIA;

D O I：

10.1080/17476348.2016.1177461

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 the most common interstitial lung disease with a fatal prognosis. Over the last decade, the concepts in pathobiology of pulmonary fibrosis have shifted from a model of chronic inflammation to dysregulated fibroproliferative repair in genetically predisposed patients. Although new breakthrough treatments are now available that slow the progression of the disease, several newer anti-inflammatory and anti-fibrotic drugs are under investigation. Patients with IPF often have coexistent conditions; prompt detection and interventions of which may improve the overall outcome of patients with IPF. Here, we summarize the present understanding of pathogenesis of IPF and treatment options for IPF in the current landscape of new anti-fibrotic treatment options.

引用

页码：699 / 711

页数：13

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