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Acute exacerbation of idiopathic pulmonary fibrosis: who to treat, how to treat.

ABSTRACT:

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.

Summary

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.

SUBMITTER: Kulkarni T

PROVIDER: S-EPMC7732144 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Acute exacerbation of idiopathic pulmonary fibrosis: who to treat, how to treat.

Kulkarni Tejaswini T Duncan Steven R SR

Current pulmonology reports 20191126 4

<h4>Purpose of review</h4>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.<h4>Recent findings</h4>Novel therapies targeting various pathways including inflammation, autoimmunity and coagulation cascade involved in AE-IPF have rec ...[more]

PMID: 33312849

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