Autoimmune hepatitis (AIH) is a liver disease that runs a course of chronic and progressive inflammation. This occurs in young children and adolescents. AIH can also be manifested in an acute and aggressive form. Its etiology is unknown. Two varieties have been reported. These are autoimmune hepatitis type 1 (AIH-1) and type 2 (AIH-2). AIH-1 affects children as well as adults and it is related with the presence of antinuclear (ANA) and anti-smooth muscle (ASM) antibodies. AIH-1 is associated

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... h immunological illnesses such as ulcerative colitis, sclerosing cholangitis, arthritis, and vasculitis. AIH-2 is more frequent in children. It presents with liver and kidney microsome (Anti-LKM-1) antibodies and anti-cytosol antibodies (Anti-LC-1). AIH-2 has also been associated with other immunopathies such as polyendocrinopathy, vitiligo, thyroiditis, alopecia and diabetes mellitus type 1 (DM1). This report describes the clinical course of two patients with AIH: a female treated since five years of age and followed during 18 years. After 11 years, the patient developed DM1 and later anorexia nervosa. This patient died due to complications of the latter. The other patient, who was diagnosed with AIH at the age of 10 years, was treated and followed during 10 years. This patient manifested idiopathic thrombocytopenia purpura (IPT) and Hashimoto encephalopathy during the course of the disease. This patient continues to remain under control. The relationship of AIH-1 with other autoimmune processes, such as Diabetes mellitus type 1 (DM1) until now, has been poorly investigated. Also, ITP followed by Hashimoto encephalopathy was a rare association in our patient. It has been considered that these are a consequence of autoimmune dysregulation.