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anti-neuronal nuclear autoantibodies. Neurology 1998;50:652-657 3. Vernino S, Adamski J, Kryzer TJ, et
al: Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related
syndromes. Neurology 1998;50:1806-1813 4. Vernino S, Low PA, Fealey RD, et al: Autoantibodies to
ganglionic acetylcholine receptors in autoimmune autonomic neuropathies. N Engl J Med
2000;343:847-855 5. Pittock SJ, Kryzer TJ, Lennon VA: Paraneoplastic antibodies coexist and predict
cancer, not neurological syndrome. Ann Neurol 2004;56:715-719 6. Dhamija R, Tan KM, Pittock SJ, et
al: Serological profiles aiding the diagnosis of autoimmune gastrointestinal dysmotility. Clin
Gastroenterol Hepatol 2008;6:988-992 7. McKeon A, Lennon VA, Lachance DH, et al: The ganglionic
acetylcholine receptor autoantibody: oncological, neurological and serological accompaniments. Arch
Neurol 66(6):735-741 8. Kraichely RE, Farrugia G, Castell DO, et al: Neural autoantibody profile of
primary achalasia. Dig Dis Sci 2009; June 5 Epub
Autoimmune Liver Disease Evaluation, Serum
Clinical Information: Smooth muscle antibodies (SMA) with specificity for actin are found in
patients with chronic autoimmune hepatitis (CAH) and are often accompanied by the presence of
antinuclear antibodies (ANA). Anti-liver/kidney microsome type 1 antibodies (anti-LKM-1) occur in a
subset of patients with CAH, predominately children. The associated disease is referred to as autoimmune
hepatitis (AIH), type 2. These patients often have other autoantibodies, eg, parietal cell antibodies and
thyroid microsomal antibodies. Anti-mitochondrial antibodies (AMA) is a marker for primary biliary
cirrhosis (PBC), a chronic inflammatory liver disease characterized by the progressive destruction of
interlobular bile ducts with development of cholestasis and eventually cirrhosis.(2)
Useful For: Evaluating patients suspected of having autoimmune congenital adrenal hyperplasia or
primary biliary cirrhosis and evaluation of patients with liver disease of unknown etiology
Interpretation: Positive results for smooth muscle antibodies (SMA) are consistent with the diagnosis
of chronic autoimmune hepatitis. The presence of anti-liver/kidney microsome type 1 antibodies with or
without SMA is consistent with autoimmune hepatitis, type 2. The presence of anti-mitochondrial
antibodies is consistent with primary biliary cirrhosis.
Reference Values:
SMOOTH MUSCLE ANTIBODIES
Negative
If positive, results are titered.
Reference values apply to all ages.
LIVER/KIDNEY MICROSOME TYPE 1 ANTIBODIES
< or =20.0 Units (negative)
20.1-24.9 Units (equivocal)
> or =25.0 Units (positive)
Reference values apply to all ages.
MITOCHONDRIAL ANTIBODIES (M2)
Negative: or =1.0 Units
Reference values apply to all ages.
Clinical References: 1. Doniach D, Roitt IM, Walker JG, Sherlock S: Tissue antibodies in primary
biliary cirrhosis, active chronic (lupoid) hepatitis, cryptogenic cirrhosis, and other liver diseases and their
clinical implications. Clin Exp Immunol 1966 July;1(3):237-262 2. Van Norstrand MD, Malinchoc M,
Lindor KD, et al: Quantitative measurement of autoantibodies to recombinant mitochondrial antigens in
patients with primary biliary cirrhosis: relationship to levels of autoantibodies to disease progression.
Hepatology 1997 Jan;25(1):6-11
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