Abstract
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.
Highlights
Idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases that primarily target the skeleton muscles [1, 2]
We retrospectively reviewed the medical records of 424 patients with DM, PM, and clinically amyopathic dermatomyositis (CADM) who were admitted to our center from February 2011 to February 2019, and performed a case-control analysis to identify potential related risk factors for AE-interstitial lung disease (ILD) among these patients
The updated criteria included previous or concurrent diagnosis of ILD, acute worsening or development of dyspnea typically
Summary
Idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases that primarily target the skeleton muscles [1, 2]. The incidence of DM, PM, and CADM was considerably low in common people, the high mortality rate, the various clinical manifestations, and multiple complications have drawn much attention from clinicians and researchers. Among the multiple extramuscular complications of IIM, interstitial lung disease (ILD) was identified as both the most frequent and severe involvement, leading to a significant elevation in mortality rate [6]. In CTD patients, AE-ILD was reported to occur at a 1-year frequency of 1.25–3.3%, at a lifetime incidence of 7.2% in CTD patients, and contributed to a high mortality rate within these patients [7, 8]. In the past few years, there existed a few reports and small-sample studies of AE-ILD, or rapid progression of ILD, in IIM patients. It is necessary to uncover the enigma by figuring out factors correlated with AE-ILD in patients with DM, PM, or CADM, and factors associated with outcome of patients with AE-ILD
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
