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Maria Grazia Clemente [1; 2] and Roberto Antonucci [2] and Claudia Mandato [3; 4] and Lucia Cicotto [1] and Antonella Meloni [1] and Bruno Gridelli [5] and Stefano De Virgiliis [1] and Michael P. Manns [6] and Pietro Vajro [3]
Academic Editor: Haruki Komatsu
1, Department of Biomedical Sciences and Biotechnologies, 2nd Pediatric Clinic, University of Cagliari, Cagliari, Italy, unica.it
2, Pediatric Clinic, Department of Surgical, Microsurgical and Medical Sciences, University of Sassari, Sassari, Italy, uniss.it
3, Pediatrics, Department of Medicine, Surgery, and Dentistry “Schola Medica Salernitana”, University of Salerno, Baronissi, Salerno, Italy, unisa.it
4, Pediatrics Unit, AORN Santobono-Pausilipon, Naples, Italy, santobonopausilipon.it
5, Mediterranean Institute for Transplantation & Advanced Specialized Therapies (ISMETT), Palermo, Italy, ismett.edu
6, Department of Gastroenterology, Hepatology and Endocrinology, Medical School Hannover, Hannover, Germany, mh-hannover.de
Received Sep 11, 2017; Revised Oct 31, 2017; Accepted Nov 6, 2017
This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
1. Introduction
De novo autoimmune hepatitis (AIH) after orthotopic liver transplantation (OLT) should be suspected in any unexplained graft dysfunction, in both children and adults transplanted for an indication different from autoimmune liver diseases [1, 2]. It is still not clear whether the pathogenesis is autoimmune directed against the “self” or alloimmune against the “non-self” after OLT. Passive transfer of autoantibodies from the organ donor, induction by viral infection, drug therapy, and ischemic injury of rejected transplanted organ are the main hypothesized mechanisms [1, 2].
Differential diagnosis with the far more common graft rejection and viral hepatitis is challenging in spite of the diagnostic criteria for de novo AIH proposed in 2006 by the Banff working group [3].
Both typical and atypical circulating autoantibodies have been found to be associated with de novo AIH. Among the typical ones [4, 5], anti-nuclear antibodies (ANA) have been the most frequently detected (42.8%–100%), followed by anti-smooth muscle antibodies (SMA) (25%−50%), while anti-liver/kidney microsomal antibodies (LKM) have been detected more rarely. Atypical LKM antibodies are peculiar for de novo AIH. They have been renamed anti-liver/kidney cytosol (LKC) because they do not react with the liver microsomes but with the cytosol and are specifically directed against the cytosolic enzyme...