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We report the findings in two sisters with active cirrhosis and an anti-liver-kidney microsomal antibody (anti-LKM) of the autoimmune type. This unusual disease is characterized by the presence of high levels of antibodies that react with the smooth and rough endoplasmic reticulum of the liver and other tissues. Our patients had the usual features of chronic hepatitis associated with presence of antibodies: they were young girls, they had anti-LKM antibodies of autoimmune type persisting at a high titer during the whole course of the disease, but with no smooth muscle antibodies; one had a low level of IgA. The occurrence of two cases in the same family has not yet been reported and is probably not coincidental because of the rare occurrence of this disease.
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