Dataset Information

Predicting Survival Across Acute Exacerbation of Interstitial Lung Disease in Patients with Idiopathic Inflammatory Myositis: The GAP-ILD Model.

ABSTRACT:

Introduction

Risk prediction is challenging in patients with idiopathic inflammatory myopathies (IIM) and acute exacerbation of interstitial lung disease (AE-ILD) because of heterogeneity and patient-specific variables. Our objective was to assess whether mortality is accurately predicted in patients with IIM and AE-ILD by using the gender age physiology ILD (GAP-ILD) model, a clinical prediction model that was previously validated in patients with idiopathic pulmonary fibrosis.

Methods

A retrospective cohort study was conducted in the First Affiliated Hospital, Zhejiang University, wherein 60 consecutive patients with IIM and AE-ILD admitted between February 2011 and April 2019. The GAP-ILD was assessed retrospectively on the basis of gender, age and pulmonary function test.

Results

Patients with AE-ILD (n = 60) were identified and collected, 26 deaths occurred during follow-up, and the non-survivors group presented a higher level of GAP-ILD index (P = 0.005), bacterial infection (P = 0.013), and myositis disease activity assessment (MYOACT) (P = 0.031). The subsequent multivariate logistic regression analysis of overall mortality in AE-ILD revealed that bacterial infection (OR 5.275, P = 0.037) and GAP-ILD index (OR 2.292, P = 0.011) conferred significant risk of mortality. The GAP-ILD index was able to separate patients with AE-ILD into two groups with a statistically significant difference in survival rate (log rank P = 0.002). Satisfactory mortality estimation was maintained in the corresponding GAP-ILD index across the AE-ILD group.

Conclusion

The GAP-ILD model preforms well in risk prediction of mortality among patients with IIM and AE-ILD. Pulmonary bacterial infection can also be taken as an initial predictor of poor prognosis in patients with IIM and AE-ILD that must be taken seriously.

SUBMITTER: Cao H

PROVIDER: S-EPMC7695794 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Publications

Predicting Survival Across Acute Exacerbation of Interstitial Lung Disease in Patients with Idiopathic Inflammatory Myositis: The GAP-ILD Model.

Cao Heng H Huan Caijuan C Wang Qin Q Xu Guanhua G Lin Jin J Zhou Jianying J

Rheumatology and therapy 20201026 4

<h4>Introduction</h4>Risk prediction is challenging in patients with idiopathic inflammatory myopathies (IIM) and acute exacerbation of interstitial lung disease (AE-ILD) because of heterogeneity and patient-specific variables. Our objective was to assess whether mortality is accurately predicted in patients with IIM and AE-ILD by using the gender age physiology ILD (GAP-ILD) model, a clinical prediction model that was previously validated in patients with idiopathic pulmonary fibrosis.<h4>Metho ...[more]

PMID: 33106937

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Project description:Objective: This study aimed at clarifying the prevalence, risk factors, outcome, and outcome-related factors of acute exacerbation of interstitial lung disease (AE-ILD) in patients with idiopathic inflammatory myopathy (IIM). Methods: Data of IIM patients who were admitted to the First Affiliated Hospital of Zhejiang University (FAHZJU) from September 2007 to September 2019 were retrospectively collected. And the IIM patients with AE-ILD formed the case group. In addition, age and sex matched IIM patients without AE-ILD were randomly selected to constitute the control group. A 1:2 case-control study and intragroup analysis were performed to identify risk factors for development of AE-ILD in IIM patients and unfavorable short-term outcome in AE-ILD patients through comparison, univariate and multivariate logistic regression analysis. Results: AE-ILD occurred in 64 out of 665 IIM patients (9.6%) with a short-term mortality rate of 39.1%. And the 64 IIM patients with AE-ILD formed the case group. Besides, 128 age and sex matched IIM patients without AE-ILD were randomly selected to constitute the control group. The retrospective case-control study revealed that elevated on-admission disease activity (P < 0.001), lower percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%, P = 0.013) and diagnosis of clinically amyopathic dermatomyositis (CADM, P = 0.007) were risk factors for development of AE-ILD in IIM patients. The following intragroup analysis indicated that elevated on-admission disease activity (P = 0.008) and bacterial infection (P = 0.003) were significantly correlated with the unfavorable short-term outcome of patients complicated with AE-ILD. In addition, combined use of steroid and disease modifying antirheumatic drugs (DMARDs, P = 0.006) was found to significantly reduce the short-term mortality in IIM patients with AE-ILD. Conclusion: AE-ILD is a less frequent but fatal complication in IIM patients with elevated on-admission disease activity, lower DLCO% and diagnosis of CADM working as risk factors, indicating the potential roles of autoimmune abnormality and hypoxia in development of AE-ILD. Elevated on-admission disease activity and bacterial infection could predict unfavorable short-term outcome of IIM patients with AE-ILD. A therapeutic regimen of steroid and DMARDs was found to reduce short-term death in these patients.

Project description:BackgroundAcute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is well known as a life-threatening condition during its clinical course. However, the clinical features and prognosis in AE of unclassifiable idiopathic interstitial pneumonia (AE-UCIIP) remain to be elucidated. The aim of this study was to clarify the clinical features and prognosis of AE-UCIIP compared with those of AE-IPF.MethodsIn 187 patients with UCIIP or IPF, 64 patients with AE-UCIIP or AE-IPF, who were diagnosed and treated at our hospital, were retrospectively evaluated.ResultsA total of 24 patients with AE-UCIIP were significantly older (p = 0.011), included more women (p < 0.001) and never-smokers (p < 0.001), and showed fewer lung lesions on high-resolution computed tomography (p = 0.006) than 40 patients with AE-IPF. Incidence of AE-UCIIP was 10.29%/year and was significantly higher than in AE-IPF (Gray's test, p = 0.008). Prognosis of AE-UCIIP was as poor as that of AE-IPF (log-rank, p = 0.681). Percent-predicted forced vital capacity (%FVC) [hazard ratio (HR) 0.934, p = 0.045], and GAP stage within 12 months before AE (HR 3.530, p = 0.023), and partial pressure arterial oxygen/fraction of inspired oxygen (PaO2/FiO2) ratio at AE (HR 0.998, p = 0.016) were significant prognostic factors. Finally, commencement of long-duration (⩾12 h) direct hemoperfusion with a polymyxin B-immobilised fibre column (PMX-DHP) within 2 days after admission significantly improved survival (log-rank, p = 0.038) and was a significant prognostic factor (HR 0.175, p = 0.0039) in AE-UCIIP. Long-duration PMX-DHP showed favourable treatment effects even in the combined group of patients with AE-UCIIP or AE-IPF (log-rank p = 0.002; HR 0.328, p = 0.006).ConclusionsPatients with AE-UCIIP were older and included more women and never-smokers than those with AE-IPF. Prognosis of AE-UCIIP was as poor as that of AE-IPF. The reviews of this paper are available via the supplemental material section.

Dataset Information

Predicting Survival Across Acute Exacerbation of Interstitial Lung Disease in Patients with Idiopathic Inflammatory Myositis: The GAP-ILD Model.

Introduction

Methods

Results

Conclusion

Publications

Predicting Survival Across Acute Exacerbation of Interstitial Lung Disease in Patients with Idiopathic Inflammatory Myositis: The GAP-ILD Model.

Similar Datasets

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