[ XXXX : two case reports with different clinical courses - case 3/2011].

@entity1 @entity4 @entity648 @entity843 @entity281 @entity1442 @entity3960 @entity74 @entity158 @entity617 @entity99 @entity1118 @entity130 @entity775
HISTORY AND CLINICAL FINDINGS: Case 1: A 46-year old female @entity1 presented with a recently occurred icterus of unknown origin as well as dark urine and decolored stool. No diseases were found in the @entity1 's medical history. Clinical examination showed no other findings exept from the icterus. Case 2: A 48-year old female @entity1 was admitted to hospital with @entity158 and icterus. Similar symptoms reoccurred regularly since several years. The @entity1 already underwent cholecystectomy and an ERCP (endoscopic retrograde cholangiopancreaticography) that showed no pathological findings. She reported @entity1118 in her finger joints and appearance of haematomas without adequate @entity130 . CLINICAL INVESTIGATIONS: Case 1: We found highly elevated liver enzymes and @entity648 . Ultrasound examination was unremarkable.The laboratory examination showed a negative serology for @entity99 , B and C, marked immunoglobulin G (IgG) elevation and hypergammaglobulinaemia. Liver biopsy and analysis of autoimmune antibodies were performed showing high titers of antinuclear antibodies (ANA) and @entity3960 ( @entity3960 ). Case 2: We found a considerably reduced liver function with low albumin and prothrombin time, as well as a moderate elevation of liver enzymes and a high @entity648 . Ultrasound examination revealed hepatic parenchymal changes, splenomegaly, and @entity843 . Oesophagogastroduodenoscopy showed oesophageal varices I  . Serology for @entity99 , B, and C was negative. Also in this case, a marked IgG elevation and hypergammaglobulinaemia were found. Liver biopsy was performed. Autoimmune antibodies (ANA and @entity3960 ) were detectable with high titers. DIAGNOSIS, TREATMENT AND COURSE: In both cases, we diagnosed an @entity775 by means of laboratory values, histological findings and detection of typical autoantibodies. Immediate therapy with high-dose @entity617 therapy was initiated (case 1: 60 mg/day; case 2: 100 mg/day), resulting in improvement of @entity1 ' condition, clinical findings and laboratory values in both cases. Case 1: The @entity1 showed fast recovery under @entity617 and the further course was without any complications. Continuous therapy with 15 mg /day and clinical monitoring through day hospital was recommended. Case 2: We saw a slower recovery and prolonged reduced liver function with the necessity to substitute coagulation factors. Furthermore, the therapy of subsequent complications, such as surgical drainage of a haematoma, @entity4 , @entity74 and @entity281 under @entity617 was necessary. Liver transplantation is planned if the disease progresses further. CONCLUSION: Elevated liver enzymes should always be further investigated. @entity775 is a rare disease. Rapid response to immunosuppressive therapy, such as @entity617 , is characteristic. Early diagnosis and therapy are essential for the @entity1 prognosis. Liver transplantation is indicated in @entity1442 .
