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Updated: Jul 5, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and
Maryam Fathi1, Jenny Vikgren, Marianne Boijsen
1Karolinska University Hospital, Solna, Sweden. Maryam.Fathi@Karolinska.se
Objective:
To estimate predictors and long-term outcome of interstitial lung disease (ILD) in patients with polymyositis (PM) and dermatomyositis (DM).
Methods:
We conducted a prospective study in which newly diagnosed PM/DM patients, regardless of clinical symptoms of pulmonary disease, were investigated with repeated chest radiography, high-resolution computed tomography (HRCT) of the lungs, and pulmonary function test (PFT). Clinical, radiologic, and lung function outcome was based on the last followup results.
Results:
Twenty-three patients with a mean followup period of 35 months were included. Findings on radiographic examination and/or PFT compatible with ILD were recorded in 18 patients (78%). Patients with ILD had lower lung function, higher radiologic scores, and higher creatine kinase values than those without ILD. All patients were treated with high-dose glucocorticoids and other immunosuppressive agents. Two patients died due to ILD, both with active myositis. During the followup, total lung capacity (TLC) improved in 33%, remained stable in 39%, and deteriorated in 28%. Changes in TLC correlated only partially with HRCT findings, which persisted even after normalizing for lung function.
Conclusion:
ILD associated with PM/DM is in most cases mild, chronic, and has a nonprogressive course during immunosuppressive treatment. PFT can be normalized during treatment with immunosuppressive therapy, even if radiologic signs of ILD persist. The course of ILD could not be predicted on the first examination. Therefore, myositis patients with ILD need careful evaluation of clinical features as well as PFT and radiologic features during followup.
Insights
Interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM) is often mild and chronic. While lung function may improve with immunosuppressive treatment, radiologic signs of ILD can persist.
Area of Science:
- Rheumatology
- Pulmonology
- Radiology
Background:
- Interstitial lung disease (ILD) is a significant complication in polymyositis (PM) and dermatomyositis (DM).
- Predicting the long-term outcome of ILD in these patients is crucial for management.
Purpose of the Study:
- To identify predictors of ILD in PM/DM patients.
- To evaluate the long-term outcome of ILD in PM/DM patients under immunosuppressive treatment.
Main Methods:
- Prospective study of newly diagnosed PM/DM patients.
- Inclusion of chest radiography, high-resolution computed tomography (HRCT), and pulmonary function tests (PFT).
- Assessment of clinical, radiologic, and lung function outcomes at last follow-up.
Main Results:
- 78% of patients showed findings compatible with ILD.
- Patients with ILD had reduced lung function and higher creatine kinase levels.
- Total lung capacity (TLC) showed variable changes, with improvement in 33% and persistence of radiologic findings.
Conclusions:
- ILD associated with PM/DM is typically mild and chronic with a nonprogressive course under treatment.
- Pulmonary function tests (PFT) can normalize despite persistent radiologic signs of ILD.
- Careful longitudinal evaluation of clinical, PFT, and radiologic features is essential for managing myositis-associated ILD.
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