BACKGROUND/AIMS: The cause of cryptogenic ('seronegative') acute liver failure (ALF) is unknown; the presence of non-organ specific autoantibodies (NOSA) may indicate an autoimmune pathogenesis. We investigated the prevalence of autoimmune features including antibodies to soluble liver antigen (anti-SLA) determined by Radioligand assay (RLA) in ALF patients. METHODS: The prevalence and relationship to the immunoglobulin (Ig) response of NOSA and anti-SLA were determined in 73 ALF patients of different causes. Patients were scored using the revised system for diagnosis of autoimmune hepatitis (AIH). RESULTS: Autoantibodies were present in 23 (32%) patients; 16 had anti-SLA, 6 ANA, 4 ASM and 1 AMA. They were absent in paracetamol-related disease and present in 23 of 53 (43%) of non-paracetamol (NP) cases (p<0.0003). Two of 16 (13%) with NP drug-induced (NPDI), 9 of 21 (43%) viral and 5 of 16 (31%) cryptogenic cases had anti-SLA. NOSA and anti-SLA were not associated with liver injury severity or outcome. IgM was higher in those with viral disease (p<0.00001). AIH scores classified 50% of cryptogenic cases as 'probable autoimmune hepatitis'. CONCLUSIONS: Autoantibodies are common in ALF; cryptogenic cases have features suggestive of an autoimmune pathogenesis. IgM is elevated in viral cases but infrequently in ALF of other causes.
BACKGROUND/AIMS: The cause of cryptogenic ('seronegative') acute liver failure (ALF) is unknown; the presence of non-organ specific autoantibodies (NOSA) may indicate an autoimmune pathogenesis. We investigated the prevalence of autoimmune features including antibodies to soluble liver antigen (anti-SLA) determined by Radioligand assay (RLA) in ALFpatients. METHODS: The prevalence and relationship to the immunoglobulin (Ig) response of NOSA and anti-SLA were determined in 73 ALFpatients of different causes. Patients were scored using the revised system for diagnosis of autoimmune hepatitis (AIH). RESULTS: Autoantibodies were present in 23 (32%) patients; 16 had anti-SLA, 6 ANA, 4 ASM and 1 AMA. They were absent in paracetamol-related disease and present in 23 of 53 (43%) of non-paracetamol (NP) cases (p<0.0003). Two of 16 (13%) with NP drug-induced (NPDI), 9 of 21 (43%) viral and 5 of 16 (31%) cryptogenic cases had anti-SLA. NOSA and anti-SLA were not associated with liver injury severity or outcome. IgM was higher in those with viral disease (p<0.00001). AIH scores classified 50% of cryptogenic cases as 'probable autoimmune hepatitis'. CONCLUSIONS: Autoantibodies are common in ALF; cryptogenic cases have features suggestive of an autoimmune pathogenesis. IgM is elevated in viral cases but infrequently in ALF of other causes.
Authors: R Todd Stravitz; Jay H Lefkowitch; Robert J Fontana; M Eric Gershwin; Patrick S C Leung; Richard K Sterling; Michael P Manns; Gary L Norman; William M Lee Journal: Hepatology Date: 2011-01-05 Impact factor: 17.425
Authors: Michael R Narkewicz; Simon Horslen; Steven H Belle; David A Rudnick; Vicky L Ng; Philip Rosenthal; Rene Romero; Kathleen M Loomes; Song Zhang; Regina M Hardison; Robert H Squires Journal: J Pediatr Gastroenterol Nutr Date: 2017-02 Impact factor: 2.839