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Pulmonary complications of inflammatory myopathy
1Division of Rheumatology and Clinical Immunology, University of Pittsburgh, S707 Biomedical Science Tower, 3500 Terrace Street, Pittsburgh, PA 15261, USA. ascher@pitt.edu
Abstract:
Pulmonary manifestations contribute significantly to the morbidity and mortality of the idiopathic inflammatory myopathies, ranging from intrinsic lung disease to secondary complications that include aspiration pneumonia, opportunistic infection, congestive heart failure, and hypoventilation. Newer classification schemes for interstitial lung disease have permitted closer correlation between histologic subtype and clinical outcome, while diagnostic techniques such as bronchoalveolar lavage have begun to define the cellular elements responsible for immune-mediated pulmonary dysfunction. Investigators have identified several serum markers correlating with inflammatory disease activity in the lung that should enhance noninvasive monitoring of therapeutic responses to newer regimens involving agents such as cyclosporine and tacrolimus. Taken together, these advances have contributed to better understanding of the immunopathogenesis of myositis-associated interstitial lung disease that should ultimately translate into more effective treatment.
Insights
Idiopathic inflammatory myopathies can cause serious lung problems. Advances in classification, diagnostics, and serum markers are improving the understanding and treatment of myositis-associated interstitial lung disease.
Area of Science:
- Pulmonology
- Rheumatology
- Immunology
Background:
- Pulmonary manifestations significantly impact morbidity and mortality in idiopathic inflammatory myopathies.
- These manifestations include intrinsic lung disease and secondary complications like pneumonia and heart failure.
Purpose of the Study:
- To review advances in understanding and managing myositis-associated interstitial lung disease.
- To highlight the role of newer classification schemes and diagnostic techniques.
Main Methods:
- Review of newer classification schemes for interstitial lung disease.
- Discussion of diagnostic techniques like bronchoalveolar lavage.
- Identification of serum markers for monitoring disease activity.
Main Results:
- Newer classification schemes correlate histologic subtype with clinical outcome.
- Bronchoalveolar lavage helps define cellular elements in immune-mediated pulmonary dysfunction.
- Serum markers correlate with inflammatory lung disease activity, aiding therapeutic response monitoring.
Conclusions:
- Advances enhance the understanding of myositis-associated interstitial lung disease immunopathogenesis.
- Improved diagnostics and monitoring facilitate more effective treatment strategies.
- Newer therapeutic agents like cyclosporine and tacrolimus show promise.