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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary complications of polymyositis and dermatomyositis
Maryam Fathi1, Ingrid E Lundberg, Göran Tornling
1Department of Medicine, Division of Respiratory Medicine and Allergy, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden.
Abstract:
Polymyositis and dermatomyositis are systemic inflammatory diseases with unknown etiology and prognosis. Pulmonary involvement is increasingly recognized to be a major complication and a common cause of morbidity and mortality in these diseases. Thus a thorough pulmonary evaluation is necessary to permit appropriate management. There are three categories of pulmonary complications in myositis: aspiration pneumonia, hypoventilation, and interstitial lung disease (ILD). ILD is a frequent pulmonary complication in patients with myositis, and respiratory symptoms are not reliable signs for diagnosis. The strongest predictive factor for ILD in patients with myositis is the presence of antihistidyl transfer ribonucleic acid (tRNA) synthetase antibodies (anti-Jo-1), but ILD may also be present in patients without these autoantibodies. Therefore, all patients with polymyositis or dermatomyositis should be investigated with chest radiography, high-resolution computed tomography, and lung function tests.
Insights
Polymyositis and dermatomyositis can cause serious lung issues, including interstitial lung disease (ILD). Early pulmonary evaluation is crucial for managing these inflammatory muscle diseases and their respiratory complications.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Polymyositis and dermatomyositis are systemic inflammatory myopathies with uncertain causes and outcomes.
- Pulmonary complications, particularly interstitial lung disease (ILD), are significant contributors to morbidity and mortality in these conditions.
- Respiratory symptoms are often unreliable indicators of ILD in myositis patients.
Purpose of the Study:
- To highlight the importance of pulmonary evaluation in patients diagnosed with polymyositis or dermatomyositis.
- To outline the spectrum of pulmonary complications associated with inflammatory myopathies.
- To emphasize the diagnostic value of specific autoantibodies and imaging in detecting ILD.
Main Methods:
- Review of literature on pulmonary involvement in polymyositis and dermatomyositis.
- Categorization of pulmonary complications: aspiration pneumonia, hypoventilation, and ILD.
- Identification of diagnostic tools including chest radiography, high-resolution computed tomography (HRCT), and pulmonary function tests (PFTs).
Main Results:
- Interstitial lung disease (ILD) is a common pulmonary complication in myositis.
- Anti-histidyl transfer RNA (tRNA) synthetase antibodies (anti-Jo-1) are the strongest predictors of ILD.
- ILD can occur in myositis patients even in the absence of anti-Jo-1 antibodies.
Conclusions:
- Comprehensive pulmonary assessment is essential for all patients with polymyositis or dermatomyositis.
- Diagnostic workup should include chest imaging and lung function testing regardless of respiratory symptoms.
- Early detection and management of ILD can improve outcomes in inflammatory myopathies.
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