J Korean Neurol Assoc.  1993 Mar;11(1):127-132.

Polymyositis in a Primary Biliary Cirrhotic Patient Treated with D-penicillamine

• Seo DW
• Lee HB
• Lee WY
• Lee KW
• Shin GS
• Chi JG

Abstract

"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.

MeSH Terms

Alanine
Alkaline Phosphatase
Aspartic Acid
Autoimmune Diseases
Biopsy
Creatine
Electromyography
Female
Follow-Up Studies
Humans
Liver Cirrhosis, Biliary
Middle Aged
Myalgia
Myositis
Penicillamine*
Polymyositis*
Prednisolone
Quadriceps Muscle
Quadriplegia
Alanine
Alkaline Phosphatase
Aspartic Acid
Creatine
Penicillamine
Prednisolone