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A 56-year-old Japanese female simultaneously developed thrombocytopenia, sicca symptoms, and an elevation of transaminase. Antiphospholipid antibodies were detected in her serum. The presence of anti-SS-A antibodies in the serum and sialectasis, disclosed by sialography, suggested the presence of primary Sjogren's syndrome (SjS). The laboratory data and the biopsy of the liver showed compatible findings with autoimmune hepatitis (AIH). Thrombocytopenia and liver dysfunction satisfactorily responded to corticosteroid. To our knowledge, this is the first reported case of SjS with AIH and antiphospholipid antibody syndrome (APAS). Analysis of serum cytokine levels showed a predominance of ThO-Thl response, which is not compatible with AIH, in this complicated autoimmune state. (Internal Medicine 39: 73-76, 2000)

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