C1-inhibitor Deficiency Induces Myositis-like Symptoms Via the Deposition of the Membrane Attack Complex in the Muscle
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- Beck Goichi
- Department of Neurology, Osaka University Graduate School of Medicine, Japan
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- Yamashita Rika
- Department of Neurology, Osaka University Graduate School of Medicine, Japan
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- Saeki Chizu
- Department of Neurology, Osaka University Graduate School of Medicine, Japan
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- Ogawa Takuya
- Department of Neurology, Osaka University Graduate School of Medicine, Japan
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- Shimizu Mikito
- Department of Neurology, Osaka University Graduate School of Medicine, Japan
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- Mochizuki Hideki
- Department of Neurology, Osaka University Graduate School of Medicine, Japan
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Abstract
<p>We herein report a 56-year-old Japanese woman who had been diagnosed with hereditary angioedema. She experienced progressing muscle weakness and pain in the upper and lower extremities. Blood tests revealed a marked increase in creatine kinase levels; however, myositis-specific autoantibodies were not detected. Serum C1-inhibitor activity and C4 levels were low. A muscle biopsy showed mild muscle fiber necrosis and C5b-9 deposition in the endomysial capillary vessel walls and sarcolemma, mimicking necrotizing myopathy. These results suggest that C1-inhibitor deficiency induces myositis-like symptoms through the activation of the complement pathway and deposition of the membrane attack complex in the muscles. </p>
Journal
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- Internal Medicine
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Internal Medicine 59 (17), 2173-2176, 2020-09-01
The Japanese Society of Internal Medicine