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Original Article
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Korean J Med. 2006;70(2):220-226.
- 증례 : 급성 호흡곤란 증후군으로 진행한 봉입체 근육염에 동반된 간질성 폐렴 1예
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박경일
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- Case Reports : A case of inclusion body myositis with interstitial lung disease
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Kyung Il Park
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한림의대 한강성심병원 내과
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- Abstract
- Inclusion body myositis (IBM) is a late-onset inflammatory myopathy with a distinct pattern of proximal and distal limb weakness and muscle wasting. Muscle biopsy shows varying degrees of active inflammation with muscle fibers containing rimmed vacuoles. The presence of rimmed vacuoles and filamentous inclusions in myofiber give an important clue in the diagnosis of IBM. The disease is resistant to steroid and other immunosuppressants. Intravenous immunoglobulin therapy seemed to be helpful.
We report a case of inclusion body myositis with interstitial lung disease. The patient presented with progressive muscular weakness and fever. His chest radiograph showed diffuse interstitial infiltration with ground glass opacity. Dyspnea and hypoxemia progressed rapidly, and acute respiratory failure developed. IBM was diagnosed by clinical symptoms, muscle biopsy and electrophysiologic study. We treated with steroids, however, there was no response. We added intravenous immunoglobulin to steroid. Thereafter chest radiograph abnormality, muscle weakness and hypoxemia were improved.(Korean J Med 70:S220-S226, 2006)
Key Words : Inclusion body myositis, Interstitial lung disease, Immunoglobulin
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