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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases
Tomoyuki Fujisawa1, Hironao Hozumi1, Masato Kono1
1Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan.
Background:
Interstitial lung disease (ILD) is strongly associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM). It is also related to mortality. Previous studies have highlighted that the acute form of PM/DM/CADM-associated ILD (PM/DM/CADM-ILD) has a poor short-term prognosis. However, little is known about the long-term clinical features of patients with PM/DM/CADM-ILD. The aim of the present study is to clarify the clinical characteristics and the predictive factors for long-term outcomes in patients with PM/DM/CADM-ILD.
Methods:
Thirty-four patients with PM/DM/CADM-ILD who were followed up for more than 12 months were analyzed retrospectively. The patients were classified as "stable" or "deterioration" according to respiratory symptoms, serial changes in forced vital capacity (FVC) or arterial oxygen pressure, and radiologic findings during the follow-up period.
Results:
Twenty-six patients (76%) were in the stable group and eight patients (24%) were in the deterioration group. Home oxygen therapy was performed in six cases in the deterioration group because of chronic respiratory failure due to progression of ILD. The deterioration group, in comparison to the stable group, had a significantly lower %FVC and a higher positive rate for the anti-PL-7 antibody. Multivariate logistic regression analysis revealed that a positive anti-PL-7 antibody test and a lower %FVC were independently associated with deterioration during long-term follow-up.
Conclusions:
Patients with PM/DM/CADM-ILD are at risk for chronic respiratory failure due to the deterioration of ILD during long-term follow-up. The presence of anti-PL-7 antibody and a lower %FVC at initial diagnosis may predict long-term deterioration in patients with PM/DM/CADM-ILD.
Insights
Interstitial lung disease (ILD) associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM) can lead to chronic respiratory failure. Early detection of anti-PL-7 antibody and low %FVC may predict long-term deterioration in these patients.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Interstitial lung disease (ILD) is a significant complication of polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM).
- Acute PM/DM/CADM-ILD has a poor short-term prognosis, but long-term outcomes remain understudied.
- ILD is associated with increased mortality in patients with PM/DM/CADM.
Purpose of the Study:
- To investigate the long-term clinical features of patients with PM/DM/CADM-ILD.
- To identify predictive factors for long-term outcomes in PM/DM/CADM-ILD.
Main Methods:
- Retrospective analysis of 34 patients with PM/DM/CADM-ILD followed for over 12 months.
- Patients classified as 'stable' or 'deterioration' based on respiratory symptoms, lung function (FVC, arterial oxygen pressure), and imaging.
- Multivariate logistic regression used to identify predictive factors.
Main Results:
- 26 patients (76%) were stable, while 8 (24%) showed deterioration.
- The deterioration group had significantly lower %FVC and a higher rate of positive anti-PL-7 antibody.
- Positive anti-PL-7 antibody and lower %FVC were independently associated with long-term deterioration.
Conclusions:
- Patients with PM/DM/CADM-ILD face a risk of chronic respiratory failure from ILD progression.
- Anti-PL-7 antibody positivity and low %FVC at diagnosis are potential predictors of long-term deterioration.
- Early identification of these factors can aid in managing PM/DM/CADM-ILD.
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