A case of Primary Biliary Cirrhosis Complicated with Inter-sterno-costoclavicular Osteoarthropathy

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  • 胸肋鎖骨間異常骨化症を呈した原発性胆汁性肝硬変症
  • 症例報告 胸肋鎖骨間異常骨化症を呈した原発性胆汁性肝硬変症
  • ショウレイ ホウコク キョウ ロク サコツ カン イジョウ コッカショウ オ テイシタ ゲンパツセイ タンジュウセイ カンコウヘンショウ

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A 64-year-old woman visited our medical center due to skin itching and visual disturbance. She was diagnosed with uveitis based upon inter-sterno-costoclavicular osteoarthropathy (ISCCO). Hematological and serological examination showed an increased erythrocyte sedimentation rate, and serum levels of enzymes, such as alkaline phosphatase, leucine aminopeptidase and gamma glutamyl transpeptidase. Autoantibodies, such as anti-nuclear antibody and anti-mitochondria M2 antibody were also positive. Chest X-ray examination and computed-tomography of the chest showed an abnormal hyperostosis of both the clavicular and upper part of the sternum. Together with these findings and symptoms, she was diagnosed as having a primary biliary cirrhosis (PBC) with an ISCCO. Arthropathies complicated with PBC usually depend on the complications of other autoimmune diseases, such as Sjogren’s syndrome, rheumatoid arthritis and scleroderma. Some reports have shown that arthropathies complicate chronic liver diseases, including PBC, and most of such arthropathies showed hyperplastic changes of the bones and joints. The ISCCO presented here is one of the typical hyperplastic arthropathies, however, the overlapping of PBC and ISCCO has not been reported. PBC of this patient is now well controlled with ursodeoxycholic acid treatment.

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