Abstract
58歳女性の原発性胆汁性肝硬変(PBC)に慢性膵炎,涙腺,顎下腺腫瘤を伴った1例を報告した.患者は胆石症のため手術をうけこの時IgM,抗ミトコンドリア抗体高値を示した.肝は生検にてCNSDCを認め,同時に施行した膵生検で小円形細胞の浸潤,腺房の萎縮,線維化がみられた.涙腺と顎下腺の腫大が徐々に増大し,各組織の生検にて肝におけるCNSDCと極めて類似した所見を示し,全身腺組織に共通の免疫学的機序によりもたらされた“dry gland” syndromeの概念を支持する症例と考えられた.
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