Autoimmune intrahepatic cholangiopathy associated with antiphospholipid antibody syndrome

We report the case of a 26-year-old woman who developed thrombophlebitis in her left leg in 2002, followed by fever, asthenia and headache in 2004. Antinuclear antibodies, antimitochondrial antibodies, anti-liver kidney microsome, anti-Smith, antiphospholipid (aPL) and antineutrophil cytoplasmic antibodies, as well as lupus- anticoagulant activity were positive. Systemic lupus erythematosus (SLE) with aPL syndrome was diagnosed and the patient was treated with azathioprine and heparin. Symptoms persisted and itching arose in the following months. The patient was admitted to our department in January 2005 for jaundice and skin rash. Elevated levels of acute phase proteins and cholestasis and liver necrosis indexes were present. Antinuclear antibodies, aPL and antimitochondrial antibodies (M5) antibodies were positive. Liver histology showed minimal focal hepatocyte necrosis, intrahepatic biliary stasis and intralobular inflammatory cell infiltrate. The absence of clinical signs that are characteristic of SLE as well as the failure to confirm antiSmith antibody positivity led us to rule out a diagnosis of SLE. On the basis of clinical, immunological and histological data, autoimmune intrahepatic cholangiopathy associated with primary aPL syndrome was diagnosed. The patient was treated with intravenous methylprednisolone followed by oral prednisone, warfarin and ursodeoxycholic acid. Liver necrosis and cholestasis indexes rapidly improved within 1 month and progressively reached the normal range. To our knowledge, this is the first description of a patient with an association of intrahepatic cholangiopathy and aPL, thus suggesting that autoimmune liver disease might associate with aPL syndrome.