| D-penicillamine으로 치료한 원발성 담도계 경화증 환자에서 발생한 다발성 근염 |
| 서대원, 이한보,이원용,이광우,신건성,지제근 |
| 서울대학교 신경과, 내과, 병리학. |
| Polymyositis in a Primary Biliary Cirrhotic Patient Treated with D-penicillamine |
| D. W. Seo, M.D., H. B. Lee, M.D., W. Y. Lee, M.D., K. W. Lee, M.D., G. S. Shin, M.D., J. G. Chi, M.D. |
| Department of neurology, Internal Medicine and Pathology, College of medicine, Seoul National University |
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| Abstract |
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Polymyositis is occasionally accompanied with other autoimmune diseases. A few cases of polymyositis associated with primary biliary cirrhosis and polymyositis complicating D-penicillamine treatment were recently reported. This case is a 50-year-old female patient who was diagnosed as primary biliary cirrhosis five years ago and treated with D-penicillamine. Gradully she experienced the muscle tenderness and proximal weakness which slowly progressed to quadriparesis Laboratory examination
showed a level of lactic dehydrogenase(LDH) 1108 IU/L, creatine phosphokinase(CPK)
6400 IU/L, aspartate aminotransferase(AST) 480 IU/L, alanine aminotransferase(ALT)
578 IU/L, and serum alkaline phosphatase 1054 IU/L. Electromyography showed
fibrillation potentials, positive sharp waves, complex repetitive discharges and small
amplitude polyphasic MUPs which were compatible with polymyositis. The muscle
biopsy of vastus lateralis revealed multifocal degenerating and regenerating myofibers
with infiltration of inflammatory cells which were consistent with inflammatory
myopathy. After high dose prednisolone therapy, her weakness slowly improved and
muscle tenderness subsided. After fifteen days of prednisolone therapy, the follow-up
laboratory examination revealed a level of LDH 1108 IU/L, CPK 2526 IU/L, AST 367
IU/L, ALT 496 IU/L, serum alkaline phosphatase 939 IU/L. This is an interesting case
of polymyositis associated with a primary biliary cirrhosis treated with D-penicillamine
in view of autoimmune theory in the pathogenesis of polymyositis. |
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