Dataset Information

Prognostic factors for acute exacerbation of idiopathic pulmonary fibrosis: protocol for a systematic review and meta-analysis.

ABSTRACT:

Introduction

Idiopathic pulmonary fibrosis (IPF) is a form of chronic fibrosing interstitial pneumonia with unknown disease aetiology. Acute exacerbation (AE) of IPF is an accelerated disease progression beyond its expected course. A 30-day mortality of AE of IPF is 40%. While death may occur, there is much variation in the clinical progression of this condition. Previous attempts have been made to investigate various possible prognostic factors for AE of IPF; however, they have yet to be confirmed. The aim of this systematic review is to clarify these prognostic factors.

Methods and analysis

In this review, AE of IPF is the condition of interest, which has been defined according to previously established diagnostic criteria. The primary outcomes of interest include short-term all-cause mortality and pulmonary-cause mortality. The secondary outcomes of interest include long-term mortality and hospital separation for the disease. Primary studies investigating prognostic factors for AE of IPF are eligible for inclusion in this review. All study types are permitted except case reports. Two reviewers will search electronic databases, such as Medline and EMBASE, from 2002 to the 1 April 2019 and extract data independently. Risk of bias in individual studies will be assessed using the Quality in Prognostic Studies tool. Meta-analysis will be conducted for univariate data if at least three studies report the effect of a specific prognostic factor using similar statistical methods. Multivariate results will be reported qualitatively. Subgroup analysis and sensitivity analysis will be considered with the aim of generalising findings to the clinical settings and drawing more robust conclusions. The Grades of Recommendation, Assessment, Development and Evaluation (GRADE) method will be applied to evaluate the quality of evidence for each prognostic factor.

Ethics and dissemination

Ethical approval will not be required. Results will be reported in a peer-reviewed scientific journal.

Prospero registration number

CRD42018106172.

SUBMITTER: Kamiya H

PROVIDER: S-EPMC6537967 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:OBJECTIVE:To clarify prognostic factors of acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF). DESIGN:A systematic review and meta-analysis. DATA SOURCES:Medline, Embase and Science Citation Index Expanded were searched from 2002 through 1 March 2019. ELIGIBILITY CRITERIA FOR SELECTING STUDIES:The review included primary studies addressing the association between the outcomes such as all-cause mortality of AE of IPF and its potential prognostic factors, which were designated as any clinical information related to the outcomes. DATA EXTRACTION AND SYNTHESIS:Two reviewers extracted relevant data independently and assessed risk of bias. Univariate results were pooled using a random-effect model if at least three studies were available. Prognostic factors were determined based on significant and consistent results on both univariate and multivariate analyses in the majority of studies. RESULTS:Out of a total of 6763 articles retrieved, 37 were eligible and 31 potential prognostic factors for all-cause mortality were selected. Each study was subject to certain methodological shortcomings. The following five factors were statistically significant by a meta-analysis of univariate results, which was confirmed by multivariate analysis, that is, Acute Physiology and Chronic Health Evaluation (APACHE) II score (HR 1.10, 1.01 to 1.19), partial pressure of arterial oxygen to fraction of inspired oxygen (PaO2/FiO2) ratio (ORs 0.99 in two studies and HRs 0.31 and 0.99 in two studies, respectively), lactate dehydrogenase (LDH) (HRs 1.002, 1.003, 1.01 and 1.02), white blood cell (WBC) count (OR 1.38, 1.04 to 1.83) and oxygen therapy before AE (HRs 3.68, 1.05 to 12.9 and 2.34, 1.04 to 5.28) (multivariate analysis, 95% CI). CONCLUSIONS:APACHE II score, PaO2/FiO2 ratio, LDH, WBC count and oxygen therapy before AE were deemed as prognostic factors of AE of IPF. Although there are some methodological limitations in this study, these findings are reliable due to consistent results by both univariate and multivariate analyses. PROSPERO REGISTRATION NUMBER:CRD42018106172.

Project description:INTRODUCTION:Idiopathic pulmonary fibrosis(IPF) is chronic fibrosing interstitial pneumonia of unknown aetiology. IPF is diagnosed based on the exclusion of known causes such as connective tissue diseases(CTDs). However, some patients fail to meet defined CTD criteria regardless of an implication of immunological involvement and these cases were described in a variety of terms. The classification criteria of this clinical entity consist of a combination of clinical, serological and morphological findings and it is reported to be distinct from IPF. However, the significance of the sole presence of autoantibodies complicated with IPF is still unknown. Therefore, this systematic review aims to clarify the significance of autoantibodies complicated with IPF. METHODS AND ANALYSIS:IPF with any autoantibody associated with CTDs is eligible for the review. Primary outcomes are all-cause mortality and pulmonary-cause mortality, while secondary outcomes include a progression of the disease, a deterioration of health-related quality of life and the development of a defined CTD. Primary studies of any type except a case report are included. Two reviewers search four electronic databases such as Medline, EMBASE, Science Citation Index Expanded and Google Scholar from each inception through 1 February 2018 and extract data independently. A risk of bias in individual studies is assessed by the Quality in Prognostic Studies tool. Meta-analysis is sought to be conducted if three or more studies report an outcome for a specific autoantibody with the same statistics. If it is inappropriate to combine data due to substantial heterogeneity, the result is reported qualitatively. Subgroup and sensitivity analyses are considered to identify the source of heterogeneity. The Grades of Recommendation, Assessment, Development and Evaluation method is applied to evaluate the evidence level of the result. ETHICS AND DISSEMINATION:There is no concerning ethical issue. The result will be sought for publication. PROSPERO REGISTRATION NUMBER:CRD42017077336.