Antimitochondrial Antibodies and Reactivity toN. AromaticivoransProteins in Icelandic Patients with Primary Biliary Cirrhosis and Their Relatives

Olafsson, Sigurdur; Gudjonsson, Hallgrimur; Selmi, Carlo; Amano, Katsushi; Invernizzi, Pietro; Podda, Mauro; Gershwin, M. Eric
November 2004
American Journal of Gastroenterology;Nov2004, Vol. 99 Issue 11, p2143
Academic Journal
OBJECTIVES: Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic disease of unknown etiology characterized by serum antimitochondrial antibodies (AMA) directed against a functionally related family of mitochondrial enzymes. We recently suggested that N. aromaticivorans might be the trigger of autoimmunity in PBC. No data are available on the specificity and crossreactivity of AMA in a genetically homogenous group of patients, such as the Icelandic population. METHODS: To address these issues and to confirm previous findings in a unique population, we obtained sera from 14 PBC patients and 85 first-degree relatives, all of Icelandic descent. We analyzed such sera for AMA specificity using recombinant mitochondrial antigens and for reactivity against N. aromaticivorans proteins. RESULTS: Thirteen of the 14 Icelandic patients with PBC (93%) were found AMA positive. We found that 5/13 AMA positive sera (38%) reacted against PDC-E2 only; 5/13 (or 38%) reacted against BCOADC-E2; and 2/13 (15%) reacted against all three antigens. There was no reactivity against OGDC-E2. Reactivities of patients' sera against N. aromaticivorans were consistent with the AMA status. One serum among the 85 first-degree relatives (1.2%) was found to be AMA-positive, as well as reactive against N. aromaticivorans. CONCLUSIONS: Interestingly, despite the homogenous genetic background, the group of Icelandic patients with PBC was heterogeneous in their AMA reactive patterns and also reacted with N. aromaticivorans proteins.


Related Articles

  • Rheumatoid Arthritis and Primary Biliary Cirrhosis: Cause, Consequence, or Coincidence? Smyk, Daniel S.; Bogdanos, Dimitrios P.; Mytilinaiou, Maria G.; Burroughs, Andrew K.; Rigopoulou, Eirini I. // Arthritis (20901984);2012, p1 

    Primary biliary cirrhosis (PBC) is a progressive cholestatic liver disease characterized serologically by cholestasis and the presence of high-titre antimitochondrial antibodies and histologically by chronic nonsuppurative cholangitis and granulomata. PBC patients often have concomitant...

  • IgG4 Cholangiopathy. Zen, Yoh; Nakanuma, Yasuni // International Journal of Hepatology;2012, p1 

    IgG4 cholangiopathy can involve any level of the biliary tree which exhibits sclerosing cholangitis or pseudotumorous hilar lesions.Most cases are associated with autoimmune pancreatitis, an important diagnostic clue.Without autoimmune pancreatitis, however, the diagnosis of IgG4-cholangiopathy...

  • Balloon Dilation Compared to Stenting of Dominant Strictures in Primary Sclerosing Cholangitis. Kaya, Muhsin; Petersen, Bret T.; Angulo, Paul; Baron, Todd H.; Andrews, James C.; Gostout, Christopher J.; Lindor, Keith D. // American Journal of Gastroenterology;Apr2001, Vol. 96 Issue 4, p1059 

    OBJECTIVE: In some patients with primary sclerosing cholangitis (PSC). a localized, high-grade (dominant) stricture may be the principal cause of symptoms and hyperbilirubinemia. The aim of this retrospective study was to compare the beneficial effects and risk of balloon dilation alone...

  • Analysis of the clinical relevance of antimitochondrial antibodies to the β- and 7gamma;-subunits of the F1F0-ATPase in patients with primary biliary cirrhosis. Nann, Dominik; Berg, Christoph P.; Preuá, Beate E.; Klein, Reinhild // BMC Gastroenterology;2012, Vol. 12 Issue 1, p152 

    Background: In a recent study we showed that in patients with primary biliary cirrhosis (PBC) being positive or negative for anti-M2 antibodies reacting with the 2-oxoacid-dehydrogenase complex (ODC) also antibodies to the beta- and gamma-subunits of F1F0-ATPase (anti-β, anti-γ) occur....

  • Predominance of IgG3 subclass in primary biliary cirrhosis. Riggione, O.; Stokes, R.P.; Thompson, R.A. // British Medical Journal (Clinical Research Edition);3/26/1983, Vol. 286 Issue 6370, p1015 

    Investigates the predominance of immunoglobulin G (IgG) subclass in primary biliary cirrhosis. Distribution of mitochondrial antibodies; Increase in serum concentration of IgG; Estimation of IgG subclasses.

  • Mitochondrial Antibody in Primary Biliary Cirrhosis and Other Diseases. Klatskin, Gerald; Kantor, Fred S. // Annals of Internal Medicine;Oct72, Vol. 77 Issue 4, p533 

    Presents a study that investigated mitochondrial antibody in the serum of patients with primary biliary cirrhosis and other liver diseases. Assessment of the incidence of mitochondrial antibody; Analysis of patients with chronic active hepatitis; Examination of patients with cryptogenic cirrhosis.

  • Human equilibrative nucleoside transporter 1 (hENT1) protein is associated with short survival in resected ampullary cancer. D. Santini; G. Perrone; B. Vincenzi; R. Lai; C. Cass; R. Alloni; C. Rabitti; A. Antinori; F. Vecchio; S. Morini; P. Magistrelli; R. Coppola; J. R. Mackey; G. Tonini // Annals of Oncology;Apr2008, Vol. 19 Issue 4, p724 

    Background: Gemcitabine is an acceptable alternative to best supportive care in the treatment of advanced biliary tract cancers. The human equilibrative nucleoside transporter 1 (hENT1) is a ubiquitous protein and is the major means by which gemcitabine enters human cells. Moreover, recent...

  • Biliary Cystadenoma: An Uncommon Cause of Recurrent Cholestatic Jaundice. Beretta, E.; De Franchis, R.; Staudacher, C.; Faravelli, A.; Primignani, M.; Vecchi, M.; Conti, E.; DiCarlo, V. // American Journal of Gastroenterology;Feb1986, Vol. 81 Issue 2, p138 

    The clinical history of a patient with intermittent jaundice is presented. The diagnostic work up, including ultrasound, laparoscopy, and endoscopic retrograde cholangiography, revealed a multiloculated cystic mass in the left lobe of the liver. At laparotomy, a multicystic lesion invading the...

  • Mechanical jaundice, acute cholangitis, biliary sepsis: pathogenic relationship and principles of differential diagnostics. Datsenko, B. M.; Borisenko, V. B. // Surgery News / Novosti Hirurgii;2013, Vol. 21 Issue 5, p31 

    Objectives. Clinico-morphological research is directed toward the study of pathogenetic relationship between biliary sepsis, mechanical jaundice and acute cholangitis as well as elaboration of the complex program differential diagnostics of these diseases. Methods. The experimental-clinical...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics