Overview of idiopathic pulmonary fibrosis, evidence-based guidelines, and recent developments in the treatment landscape.

Overview of idiopathic pulmonary fibrosis, evidence-based guidelines, and recent developments in the treatment landscape.

Glassberg, Marilyn K;
The American journal of managed care 2019 Vol. 25 pp. S195-S203
358
glassberg2019overviewthe

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive-fibrosing interstitial lung disease of unknown origin that affects 3 million people worldwide and imparts substantial burdens to patients, their families, and the healthcare system. The IPF disease course is highly variable and presents several diagnostic and management-related challenges. Two therapies, nintedanib and pirfenidone, are FDA approved and are recommended by clinical practice guidelines for the treatment of IPF. Although neither of these treatments is curative, both slow disease progression and impact survival of patients with IPF. To ensure optimal management, this supplement will provide an overview of the epidemiology, pathophysiology, and diagnosis of IPF, along with management-based considerations including evidence-based guideline recommendations, in-depth reviews of nintedanib and pirfenidone, and outcomes from other completed clinical trials.

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