Acute severe Autoimmune Hepatitis with anti-Rods and Rings autoantibodies: Literature first evidence.
Gastroenterology and Hepatology from Bed to Bench,
,
26 January 2021
https://doi.org/10.22037/ghfbb.v14i1.1924
Abstract
Autoimmune hepatitis (AIH) was defined as a progressive, chronic inflammatory autoimmune liver disease (ALD). The diagnosis of AIH requires the presence of characteristic clinical and laboratory features, and the exclusion of other clinical conditions that cause chronic hepatitis and cirrhosis.
AIH can have an acute onset that mimics an acute viral or toxic hepatitis or an acute severe (fulminant, ASF) presentation that satisfies criteria for acute liver. Guidelines from the european Association for the Study of Liver Diseases define ALF with absence of pre-existing liver disease, acute onset of ? 26 weeks, coagulopathy (international normalised ratio (INR) ? 1.5) and presence of hepatic encephalopathy (HE). In recent years, autoantibodies (Aab) targeting subcellular structures described as the rods and rings (R&R) pattern in HEp-2 ANA have been presented as a unique and particular case of Aab generation. These R&R structures are composed of inosine monophosphate dehydrogenase type 2 (IMPDH2), and their formation can be induced in vitro by several small-molecule inhibitors. Aab targeting these relatively unknown structures have been observed in hepatitis C virus (HCV) patients who have undergone treatment with pegylated interferona/ ibavirin (IFN/RBV) therapy. Now We present and characterized a case patient with R&R and SMA Aab in AIH (ASF, fatal, without liver trasnplantation). For our knowledge this is the first evidence described in the Literature.Our early experience showed the R&R circulating Aab in one patient with Primary biliary cholangitis (PBC). This work now demonstrate that R&R Aab can be also present in AIH case.
- Autoimmune liver diseases
- Autoimmune Hepatitits
- Antinuclear antibodies
- R&R autoantibodies
References
EASL Clinical Practice Guidelines: Autoimmune hepatitis European Association for the Study of the Liver Journal of Hepatology 2015 vol. 63, 971–1004.
Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D. The clinical usage and definition of autoantibodies in immune-mediated liver disease: A comprehensive overview. J Autoimmun. 2018 Dec;95:144-158
[EASL Clinical Practical Guidelines on the management of acute (fulminant) liver failure, European Association for the Study of the Liver, Journal of Hepatology 2017 vol. 66 j 1047–1081
EASL Clinical Practical Guidelines on the management of acute (fulminant) liver failure, European Association for the Study of the Liver, Journal of Hepatology 2017 vol. 66 j 1047–1081
Calise SJ, Carcamo WC, Ceribelli A, Dominguez Y, Satoh M, Chan EK. Antibodies to rods and rings. In: Shoenfeld Y, Meroni PL, Gershwin ME, Eds. New York: Elsevier Science; 2014; pp. 161-168.
[Carcamo WC, Ceribelli A, Calise SJ, Krueger C, Liu C, Daves M, et al. Differential reactivity to IMPDH2 by antirods/rings autoantibodies and unresponsiveness to pegylated interferon-alpha/ribavirin therapy in US and Italian HCV patients. J Clin Immunol 2013;33:420-6
Assandri R. Primary biliary cholangitis with contemporary presence of anti-mithocondrial and anti-rods and rings autoantibodies: literature first case Gastroenterol Hepatol Bed Bench 2019;12(1):76-82
Assandri R, Monari M, Montanelli A. Development of systemic sclerosis in patients with autoimmune hepatitis: an emerging overlap syndrome. Gastroenterol Hepatol BedBench 2016;9:211-9.
Valaperta S, Alpini C, Bottone MG, Monari M, Assandri R, Montanelli A. Autoanticorpi anti-Golgi: anche l’occhio vuole la sua parte. Recenti Prog Med 2011;102:11-3
Assandri R, Serana F, Montanelli A. Development of PBC/SSc overlap syndrome in chronic GVHD patient: Immunological implications in the presence of mitochondrial, nucleolar and spindle midzone autoantigens. Gastroenterol Hepatol Bed Bench 2017;10:323-331.
Carcamo WC, Calise SJ, von Mühlen CA, Satoh M, Chan EK. Molecular cell biology and immunobiology of mammalian rod/ring structures. Int Rev Cell Mol Biol.2014;308:35-74
Probst C, Radzimski C, Blöcker IM, Teegen B, Bogdanos DP, Stöcker W, et al. Development of a recombinant cellbased indirect immunofluorescence assay (RC-IFA) for the determination of autoantibodies against "rings and rods"-associated inosine-5'-monophosphate dehydrogenase 2 inviral hepatitis C Clin Chim Acta 2013;418:91-6
Keppeke GD, Nunes E, Ferraz ML, Silva EA, Granato C, Chan EK, et al. Longitudinal study of a human drug-induced model of autoantibody to cytoplasmic rods/rings following HCV therapy with ribavirin and interferon-α. PLoS One 2012;7:e45392.
Carcamo WC, Ceribelli A, Calise SJ, Krueger C, Liu C, Daves M, et al. Differential reactivity to IMPDH2 by antirods/ rings autoantibodies and unresponsiveness to pegylated interferon-alpha/ribavirin therapy in US and Italian HCV patients. J Clin Immunol 2013;33:420-6.
Shaikh Y, Krantz A, El-Farra Y. Anti-rods and rings autoantibodies can occur in the hepatitis c-naïve population. J Prev Med Hyg 2013;54:175-80
Allison AC, Eugui EM. Mycophenolate mofetil and its mechanisms of action. Immunopharmacology 2000;47:85-118.
Climent J, Morandeira F, Castellote J, Xiol J, Niubó J, Calatayud L, et al. Clinical correlates of the "rods and rings" antinuclear antibody pattern. Autoimmunity 2016;49:102-8.
Vergani, D., Choudhuri, K., Bogdanos, D. P. & Mieli Vergani, G. Pathogenesis of autoimmune hepatitis. Clin. Liver Dis. 6, 727–737 (2002)
Mieli-Vergani G, Vergani D, Czaja AJ, Manns MP, Krawitt EL et al, Autoimmune hepatitis Nat Rev Dis Primers. 2018 Apr 12;4:18017
Bonito, A. J. et al. Medullary thymic epithelial cell depletion leads to autoimmune hepatitis. J. Clin. Invest. 123, 3510–3524 (2013)
Calise SJ, Abboud G, Kasahara H, Morel L, Chan EKL Immune Response-Dependent Assembly of IMP Dehydrogenase Filaments. Front Immunol. 2018 Nov 29;9:2789
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