Budd-Chiari syndrome after treatment with budesonide in a cirrhotic patient with autoimmune hepatitis

Autores: Mederacke Ingmar, Helfritz Fabian, Puls Florian, Imeen Ringe Kristina, Klempnauer Juergen, Manns Michael P, Strassburg Christian P

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To the editor Standard treatment of autoimmune hepatitis (AIH) includes steroids such as predniso(lo)ne based upon study data established decades ago. Pilot studies indicate that budesonide may represent an alternative treatment option. Recently, this was confirmed in non-cirrhotic AIH patients showing that budesonide is capable of efficiently inducing remission in comparison to prednisone (both in combination with azathioprine) with the advantage of reduced steroid specific side effects. This is likely to increase the awareness of budesonide in hepatology and its administration in AIH patients. We report a 40-year old female Turkish patient, who underwent orthotopic liver transplantation (OLT) in June 2010 with decompensated liver cirrhosis because of AIH, and hepatocellular carcinoma (HCC). In 2002, a first episode of hepatic decompensation occurred and she was seen in a referring hospital. Following the exclusion of viral or metabolic causes of liver disease, and in view of detectable smooth muscle (SMA) antibodies (titer: 1:320) as well as elevated immunoglobulin G levels (18.6 g/L) a diagnosis of AIH was established. Later, anti-nuclear antibodies (ANA) (titer: 1:320) were also detected. Autoantibodies were subsequently undetectable once prednisolone treatment (5 to 20 mg per day) had begun. In April 2009, a second decompensation episode occurred. Immunosuppression was increased to 70 mg prednisolone per day in order to induce remission. With the intention to prevent steroid specific side effects this was switched to budesonide (3mg t.i.d.). One month later she was urgently referred to our center, again with hepatic decompensation. Diagnostic work-up revealed Budd-Chiari syndrome, which had not been previously observed on CT or MRI scans performed one month prior to admission. Histology confirmed cirrhosis and evidence of centrilobular sinusoidal congestion. Budesonide was discontinued and prednisone therapy resumed, in addition to anticoagulant treatment with low molecular weight heparin. The patient was successfully stabilized. However, 2 months following this episode hepatocellular carcinoma was diagnosed, she was listed for OLT, which was successfully performed one year later.

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2011-11-29   |   1,061 visitas   |   Evalua este artículo 1 valoraciones

Vol. 11 Núm.1. Enero-Febrero 2012 Pags. 143-144 Ann Hepatol 2012; 11(1)