Autoimmune liver serology: current diagnostic and clinical challenges - PubMed (original) (raw)

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Autoimmune liver serology: current diagnostic and clinical challenges

Dimitrios-P Bogdanos et al. World J Gastroenterol. 2008.

Abstract

Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseases (AiLD), namely autoimmune hepatitis types 1 and 2 (AIH-1 and 2), primary biliary cirrhosis (PBC), and the sclerosing cholangitis variants in adults and children. AIH-1 is specified by anti-nuclear antibody (ANA) and smooth muscle antibody (SMA). AIH-2 is specified by antibody to liver kidney microsomal antigen type-1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1). SMA, ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation. PBC is specified by antimitochondrial antibodies (AMA) reacting with enzymes of the 2-oxo-acid dehydrogenase complexes (chiefly pyruvate dehydrogenase complex E2 subunit) and disease-specific ANA mainly reacting with nuclear pore gp210 and nuclear body sp100. Sclerosing cholangitis presents as at least two variants, first the classical primary sclerosing cholangitis (PSC) mostly affecting adult men wherein the only (and non-specific) reactivity is an atypical perinuclear antineutrophil cytoplasmic antibody (p-ANCA), also termed perinuclear anti-neutrophil nuclear antibodies (p-ANNA) and second the childhood disease called autoimmune sclerosing cholangitis (ASC) with serological features resembling those of type 1 AIH. Liver diagnostic serology is a fast-expanding area of investigation as new purified and recombinant autoantigens, and automated technologies such as ELISAs and bead assays, become available to complement (or even compete with) traditional immunofluorescence procedures. We survey for the first time global trends in quality assurance impacting as it does on (1) manufacturers/purveyors of kits and reagents, (2) diagnostic service laboratories that fulfill clinicians' requirements, and (3) the end-user, the physician providing patient care, who must properly interpret test results in the overall clinical context.

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Figure 1

Figure 1

Immunofluorescence of anti-mitochondrial (A and B), and anti-liver kidney microsomal antibody (anti-LKM1) (C and D). AMA stain (A) stronger the smaller, distal tubules while anti-LKM1 the proximal tubules of the rat kidney (C). These specificities are frequently misdiagnosed, especially when only the kidney substrate is used and the sections do not contain both proximal and distal tubules. Thus, the use of rat stomach (B) and liver (D) is strongly recommended to prevent misinterpretation; AMA characteristically stain the gastric parietal cells while anti-LKM1 stain the rat liver but not the stomach.

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