Lymphangiome kystique médiastinal solitaire (LKM)

Abstract

OBJECTIVE:LKM-positive, or type 2, autoimmune hepatitis is characterized by the presence of antibodies directed against liver–kidney microsomes (LKM1). Although described frequently in southern Europe and the Mediterranean, this subtype of autoimmune liver disease seems to be extremely rare in northern Europe and in the United States. We report here five cases of LKM-positive autoimmune hepatitis that were seen at our center in the period 1989–1999.METHODS:We reviewed the medical records of all patients with the diagnosis of AIH in our institution during the period 1989–1999, and found that five patients had type 2 AIH. All patients were female; four of five were young, and four of five presented with overt cirrhosis.RESULTS:One patient died, one underwent liver transplantation and two are currently awaiting liver transplantation. Response to conventional immunosuppressive therapy was poor and two patients required treatment with cyclosporine and tacrolimus respectively. Four of five patients had at least one associated autoimmune disorder, including IgE-induced IgA deficiency, idiopathic thrombocytopenic purpura (ITP), and arthritis. HLA class II DR4 was present in two patients.CONCLUSIONS:LKM-positive autoimmune hepatitis seems to be a subset of autoimmune hepatitis with distinct clinical features; although rare, it is occasionally encountered in the western United States. Prompt diagnosis and appropriate immunosuppressive treatment are recommended, as well as early referral to transplantation centers. Clinicians should be aware of this condition in the setting of young female patients with unexplained severe liver disease.