Immune response guided treatment of severe lupoid autoimmune hepatitis

• Published in: Zeitschrift für Gastroenterologie
• Main Authors: Weigand, K, Brost, S, Falk, CS, Pflaum, J, Müller, M
• Format: Conference Proceeding
• Language: English
• Published: 11.01.2013
• ISSN: 0044-2771; 1439-7803
• DOI: 10.1055/s-0032-1332188

Some cases of autoimmune hepatitis, especially non-typical cases, can not be treated easily. These cases are therapy refractory and need high doses of prednisolone to be controlled. Others are even non-responders to high dosis of steroids. We here present a rare case of most likely lupoid autoimmune hepatitis accompanied by Sjögren's syndrome in a 21-year old female, diagnosed by ANA titer of 1:10240, increased rheuma factors (172.5U/l) and total immunoglobuline G. In addition positive SS-A antibodies and positive Ro52 antibodies were detected. Histological findings showed minimal lymphocyte infiltration (Desmet I), possibly fitting atypical autoimmune hepatitis. All other reasons for hepatitis were ruled out. Several courses of prednisolone in a dose higher than 60 to 100mg/d were needed, but liver enzymes and bilirubine never normalized but always re-increased when prednisolone was tapered below 60mg/d with bilirubine levels up to 14.6mg/dl. Immune monitoring guided treatment was used to figure out the correct treatment. IFN gamma, IP-10, IL-4, IL-5, IL-7, TNF alpha and CCL2 were found to correlate most appropriate with the course of the disease. By monitoring these factors using immunosuppressive therapy with rituximab, budesonide, azathioprine or mycophenolate showed mycophenolate to be most effective. Liver enzymes and bilirubine improved and prednisolone could be tapered down without relapse. Taken together, we believe that in future therapy of autoimmune diseases will be adapted by cytokine and immune response monitoring. kilian.weigand@ukr.de