RESUMO
Autoimmune chronic active hepatitis is traditionally treated with corticosteroids, or corticosteroids with azathioprine. These drug therapies require long treatment periods and are associated with many debilitating drug side effects. Five patients with type I autoimmune chronic active hepatitis, and one with a probable overlap syndrome were treated with cyclosporine. All patients had previously undergone treatment with corticosteroids or corticosteroids with azathioprine and had failed to achieve total remission. In addition, all patients had significant side effects associated with therapy. Five of six patients normalized or near normalized alanine aminotransferase levels within 10 weeks of starting cyclosporine therapy. Biochemical remission was sustained for periods of up to 1 year in all primary cyclosporine responders as long as therapeutic cyclosporine levels were maintained. All responders had symptomatic improvement. Post-treatment liver biopsy was performed in three patients and histologic improvement was demonstrated in all cases. Cyclosporine appears to be a viable alternative to corticosteroid/azathioprine therapy for autoimmune chronic active hepatitis in patients who experience an incomplete response or who cannot tolerate the side effects of standard therapy.
Assuntos
Doenças Autoimunes/tratamento farmacológico , Ciclosporina/uso terapêutico , Hepatite Crônica/tratamento farmacológico , Prednisona/uso terapêutico , Adulto , Doenças Autoimunes/patologia , Azatioprina/efeitos adversos , Azatioprina/uso terapêutico , Biópsia , Criança , Ciclosporina/efeitos adversos , Resistência a Medicamentos , Feminino , Hepatite Crônica/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Prednisona/efeitos adversosRESUMO
The diagnosis of primary sclerosing cholangitis in association with autoimmune hepatitis was made in this case after the discovery of inflammatory bowel disease prompted a cholangiogram. Immunosuppressive therapy with cyclosporine prior to ERCP resulted in a significant decrease in serum aminotransferase. Liver histology and autoimmune serologies favored a diagnosis of AIH, while cholangiographic findings suggested PSC. A review of similar cases describing the simultaneous occurrence of AIH and PSC is presented. The shared autoimmune characteristics of the two diseases, such as serum autoantibodies, peripheral lymphocyte subsets and HLA haplotypes, raises the question of whether similar mechanisms of immune dysfunction can result in both processes. The existence of a PSC-AIH overlap syndrome may help provide the linkage between the two diseases.