PMID- 32034878 OWN - NLM STAT- MEDLINE DCOM- 20210318 LR - 20210323 IS - 1527-6473 (Electronic) IS - 1527-6465 (Linking) VI - 26 IP - 6 DP - 2020 Jun TI - Role of Autoimmunity in Patients Transplanted for Acute Liver Failure of Unknown Origin: A Clinical and Graft Biopsy Analysis. PG - 764-773 LID - 10.1002/lt.25729 [doi] AB - The etiology and prognosis of acute liver failure (ALF) remains unknown in a significant proportion of cases. Signs of autoimmunity may be present, but no consistent pattern has been observed. We aimed to analyze if pretransplant immunological findings, human leukocyte antigen (HLA) haplotypes, and clinical features among patients with an unknown etiology differ from those of autoimmune or other known etiologies. We also analyzed whether such signs impact posttransplant biopsy findings or complications. All adult ALF patients undergoing liver transplantation (LT) in Finland during 1987-2015 were followed to 2016. Data were collected from the LT registry, pathology database, and patient records. A total of 124 patients were included in the analysis. Study subgroups were acute autoimmune hepatitis (AIH; n = 25), known non-AIH etiology (n = 54), and unknown etiology (n = 45). The unknown etiology group differed from the known non-AIH group with regard to the following pretransplant autoimmunity-associated features: positive perinuclear anti-neutrophil cytoplasmic antibodies (36% versus 8%; P = 0.02) and higher mean immunoglobulin A (IgA; 3.2 +/- 1.7 versus 2.1 +/- 1.4, P = 0.006) and immunoglobulin G (IgG; 12.7 +/- 4.3 versus 8.5 +/- 3.6, P = 0.001). AIH-associated HLA haplotypes B8, DR3, and B8DR3 were more common in the AIH group (40%, 44%, and 36%, respectively) and in the unknown group (29%, 33%, and 29%, respectively) than in the known non-AIH group (11%, 17%, and 11%, respectively) or in the Finnish general population (17%, 18%, and 8%, respectively). However, these findings had no association with protocol biopsies, extrahepatic autoimmune diseases, or survival. Patients with >/= 1 rejection episode had higher pretransplant IgA (3.7 +/- 2.3 versus 2.6 +/- 1.2; P = 0.02) and IgG (16.4 +/- 10.2 versus 12.4 +/- 6.8; P = 0.03) than those without rejections. Autoimmunity-associated pretransplant laboratory findings and HLA haplotypes were common in ALF of unknown etiology, but they showed minimal predictive value for posttransplant biopsy findings, clinical complications, or survival. CI - Copyright (c) 2020 by the American Association for the Study of Liver Diseases. FAU - Liukkonen, Ville AU - Liukkonen V AD - Department of Gastroenterology, Helsinki University Hospital, Helsinki, Finland. FAU - Nordin, Arno AU - Nordin A AD - Transplantation and Liver Surgery Clinic, Helsinki University Hospital, Helsinki, Finland. FAU - Arola, Johanna AU - Arola J AD - Department of Pathology, Laboratory of Helsinki University Hospital (HUSLAB), University of Helsinki, Helsinki, Finland. FAU - Farkkila, Martti AU - Farkkila M AD - Department of Gastroenterology, Helsinki University Hospital, Helsinki, Finland. FAU - Aberg, Fredrik AU - Aberg F AUID- ORCID: 0000-0002-3833-0705 AD - Transplantation and Liver Surgery Clinic, Helsinki University Hospital, Helsinki, Finland. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200511 PL - United States TA - Liver Transpl JT - Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society JID - 100909185 SB - IM CIN - Liver Transpl. 2020 Jun;26(6):743-745. PMID: 32279421 CIN - Liver Transpl. 2021 Feb;27(2):309-310. PMID: 33047498 MH - Adult MH - Autoimmunity MH - Biopsy MH - *Hepatitis, Autoimmune/diagnosis MH - Humans MH - *Liver Failure, Acute/diagnosis/etiology/surgery MH - *Liver Transplantation/adverse effects EDAT- 2020/02/09 06:00 MHDA- 2021/03/19 06:00 CRDT- 2020/02/09 06:00 PHST- 2019/09/02 00:00 [received] PHST- 2020/01/28 00:00 [accepted] PHST- 2020/02/09 06:00 [pubmed] PHST- 2021/03/19 06:00 [medline] PHST- 2020/02/09 06:00 [entrez] AID - 10.1002/lt.25729 [doi] PST - ppublish SO - Liver Transpl. 2020 Jun;26(6):764-773. doi: 10.1002/lt.25729. Epub 2020 May 11.