Acute Exacerbation of Idiopathic Pulmonary Fibrosis: Who to Treat, How to Treat

被引：6

作者：

Kulkarni, Tejaswini ^{[1
]}

Duncan, Steven R. ^{[1
]}

机构：

[1] Univ Alabama Birmingham, Dept Med, Div Pulm & Crit Care Med, THT 541 D,1900 Univ Blvd, Birmingham, AL 35294 USA

来源：

CURRENT PULMONOLOGY REPORTS | 2019年 / 8卷 / 04期

关键词：

Acute exacerbation; Idiopathic pulmonary fibrosis; Management; HUMAN SOLUBLE THROMBOMODULIN; IMMOBILIZED FIBER COLUMN; SURGICAL LUNG-BIOPSY; RESPIRATORY-FAILURE; RISK-FACTORS; PIRFENIDONE; MORTALITY; CYCLOPHOSPHAMIDE; ASSOCIATION; OXYGENATION;

D O I：

10.1007/s13665-019-00238-7

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Purpose of Review Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) are the most frequent cause of death among patients with IPF. Here, we review the revised definition and diagnostic criteria for AE-IPF and discuss management strategies including mechanistically targeted investigational therapies for this complex syndrome. Recent Findings Novel therapies targeting various pathways including inflammation, autoimmunity, and coagulation cascade involved in AE-IPF have recently been reported. Although most of these reports are small and uncontrolled, they have provided evidence to design larger randomized, controlled, multicenter studies to improve outcomes among patients with AE-IPF. AE-IPF has a dismal prognosis and current treatment consists mainly of supportive care and symptom palliation. There is a lack of consensus on current therapies for AE-IPF, including corticosteroids, but current randomized control studies for newer therapeutic strategies may hold promise.

引用

页码：123 / 130

页数：8

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