Treatment Of Idiopathic Pulmonary Fibrosis

Publication Date: July 1, 2015
Last Updated: September 2, 2022

Treatment

For patients with IPF, the guideline committee recommends:

Clinicians NOT use imatinib. ( Moderate , S, M)
695
Clinicians NOT use warfarin anticoagulation in patients who do not have a known alternative indication for its use. ( Low , S, L)
695
Clinicians NOT use the combination therapy of N-acetylcysteine, azathioprine, and prednisone. ( Low , S, L)
695
Clinicians NOT use ambrisentan, a selective ER-A endothelin receptor antagonist, regardless of the presence or absence of PH. ( Low , S, L)
695

For patients with IPF, the guideline committee suggests:

Clinicians use nintedanib. ( Moderate , C, M)
695
Clinicians use pirfenidone. ( Moderate , C, M)
695
Clinicians NOT use sildenafil, a phosphodiesterase-5 inhibitor. ( Moderate , C, M)
695
Clinicians NOT use bosentan or macitentan, both dual ER-A and ER-B endothelin receptor antagonists. ( Low , C, L)
695
Clinicians NOT use N-acetylcysteine monotherapy. ( Low , C, L)
695
Clinicians use regular anti-acid treatment. ( Very low , C, VL)
695

Recommendation Grading

Overview

Title

Treatment Of Idiopathic Pulmonary Fibrosis

Authoring Organization

American Thoracic Society

Publication Month/Year

July 1, 2015

Last Updated Month/Year

October 4, 2024

Supplemental Implementation Tools

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Document Objectives

This document updates the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guideline on idiopathic pulmonary fibrosis treatment.

Target Patient Population

Patients with idiopathic pulmonary fibrosis

Inclusion Criteria

Male, Female, Adult, Older adult

Health Care Settings

Ambulatory

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Treatment, Management

Diseases/Conditions (MeSH)

D054990 - Idiopathic Pulmonary Fibrosis

Keywords

interstitial lung disease, Idiopathic pulmonary fibrosis (IPF), IPF, Pulmonary Fibrosis, ILD

Supplemental Methodology Resources

Data Supplement, Data Supplement

Methodology

Number of Source Documents
105
Literature Search Start Date
May 1, 2010
Literature Search End Date
July 1, 2014