Abstract
Cardiomyopathy and rapid progressive interstitial pneumonia (IP) are potentially fatal complications in polymyositis/dermatomyositis. We experienced a dermatomyositis patient with multiple adverse prognostic factors, complicating rapid progressive IP, macrophage activation syndrome (MAS), and cardiomyopathy. IP and MAS improved with strong immunosuppressive therapy, despite which cardiomyopathy developed. Therefore, we applied intravenous high-dose immunoglobulin therapy (IVIg), and cardiac function improved dramatically. This is the first report to present the effectiveness of IVIg for cardiomyopathy in dermatomyositis.
Keywords:
clinical aspects (myositis and related syndromes); drug treatment (myositis and related syndromes); myositis and related syndromes.
© 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.
MeSH terms
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Cardiomyopathies / diagnostic imaging
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Cardiomyopathies / drug therapy*
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Cardiomyopathies / immunology
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Cardiomyopathies / physiopathology
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Dermatomyositis / diagnosis
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Dermatomyositis / drug therapy*
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Dermatomyositis / immunology
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Disease Progression
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Echocardiography
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Humans
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Immunoglobulins, Intravenous / administration & dosage*
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Immunologic Factors / administration & dosage*
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Immunosuppressive Agents / administration & dosage*
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Lung Diseases, Interstitial / diagnostic imaging
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Lung Diseases, Interstitial / drug therapy*
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Lung Diseases, Interstitial / immunology
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Macrophage Activation Syndrome / diagnosis
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Macrophage Activation Syndrome / drug therapy
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Macrophage Activation Syndrome / immunology
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Male
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Middle Aged
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Recovery of Function
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Tomography, X-Ray Computed
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Treatment Outcome
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Ventricular Function, Left / drug effects
Substances
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Immunoglobulins, Intravenous
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Immunologic Factors
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Immunosuppressive Agents