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80 - 90% of patients with CAH develop high titre SMA, usually in association with a positive ANA and raised IgG. Low titres are seen in 10-50% of PBC, 28% of cryptogenic cirrhosis, 10% of RA, some malignancies and 3% of normals. SMA is not predictive of the development of liver disease and is not helpful for the prognosis in patients with autoimmune CAH. SMA is useful in differentiating chronic aggressive hepatitis from SLE with Hepatitis since SMA is rarely seen in SLE.