Association of autoimmune hepatitis and systemic lupus erythematodes: a case series and review of the literature

Abstract

Liver test abnormalities have been described in up to 60% of patients with systemic lupus erythematodes (SLE) at some point during the course of their disease. Prior treatment with potentially hepatotoxic drugs or viral hepatitis is commonly considered to be the main cause of liver disease in SLE patients. However, in rare cases elevated liver enzymes may be due to concurrent autoimmune hepatitis (AIH). To distinguish whether the patient has primary liver disease with associated autoimmune clinical and laboratory features resembling SLE - such as AIH - or the elevation of liver enzymes is a manifestation of SLE remains a difficult challenge for the treating physician. Here, we present six female patients with complex autoimmune disorders and hepatitis. Patient charts were reviewed in order to investigate the complex relationship between SLE and AIH. All patients had coexisting autoimmune disease in their medical history. At the time of diagnosis of AIH, patients presented with arthralgia, abdominal complaints, cutaneous involvement and fatigue as common symptoms. All patients fulfilled the current diagnostic criteria of both, AIH and SLE. Remission of acute hepatitis was achieved in all cases after the initiation of immunosuppressive therapy. In addition to this case study a literature review was conducted.

Keywords: Autoimmune hepatitis; Elevated liver enzymes; Systemic lupus erythematodes.

Figure 1

Typical histopathology of a patient with autoimmune hepatitis (case 1). Cirrhotic changes of the liver parenchyma with interface hepatitis. The portal and periportal inflammatory infiltrate is composed of lymphocytes, monocytes/macrophages and plasma cells (haematoxylin and eosin staining; × 200).