(New lab markers for Autoimmune hepatitis (LC-1 and SLA/LP

By | يونيو 10, 2014

Auto-immune hepatitis (AIH) has a clinical and histologic criteria with well established diagnostic scores. It has predilection for females. Liver enzymes show elevation of aminotransferease more than alkaline phosphatase. Increased serum gamma globulin, presence of smooth muscle antibody and LKM-1, (with usually negative anti-mitochondria antibodies) are usually part of diagnostic criteria. AIH patients have usually negative viral markers. It is associated with HLA DR3 and DR 4 or other autoimmune disease. Histologically. It usually shows interface hepatitis with predominant lymphoplasmacytic infiltrate with liver cell resetting with minimal bile duct changes. Auto-immune hepatitis (AIH) has at least 3 types. TYPE I, represents 80% of the cases, has predilection to adult females and known to be positive for Anti-smooth muscle antibodies (ASMA). Most of patients are asymptomatic, but 20% are cirrhotic at presentation. TYPE II, on the other hand, is known to affect young adults and children and represents 10-15% of AIH. It is known to be positive for anti-LKM-1 (antibody against renal proximal tubules and hepatocytes cytoplasm, a marker that is directed against cytochrome P450), and a new marker that is Liver Cytosole (LC-1) a marker that is directed against liver cytoplasmic component (formiminotransferase cycloadeaminase). Anti-LC-1 is detected in 50% of cases with LKM-1 positive sera. Serum immunoglobulins are moderately elevated with reduction of immunoglobulin A. It carries a high risk of progression to cirrhosis. TYPE III has lower prevalence rate than that of Type II, with similar course and outcome to Type I, with 90% of the cases are females. It is positive for Soluble Liver Antigens/ Liver-pancreas (SLA/LP) and directed against UGA repressor t-RNA associated protein. This marker may be the sole marker that is present in 10% of patient with AIH

Summary: Diagnosing AIH is not complete till you do all the serologic markers: Type I: ASMA. Type II: Anti-LKM-1 and LC-1. Type III: SLA/LP. In addition to requesting ANA, ANCA (positive in 60%-90% of sera in patients with AIH) and anti-mitochondria anti-bodies

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American boards of Anatomic, Clinical Pathology and Cytopathology