title: Could post-liver transplantation course be helpful for the diagnosis of so called cryptogenic cirrhosis?
abstract: Cryptogenic cirrhosis (CC) is diagnosed in 5-30% of cirrhotic patients overall and 7% of patients who undergo liver transplantation for cirrhosis. In our series of patients transplanted for CC, pre-transplant clinical and histological data and the post-transplant course were reexamined in an attempt to identify the aetiology. Among the 881 patients transplanted in our centre between 1987 and 2000, 28 patients with a median age of 46 yr (range: 18-69) at transplantation were initially classified as having CC. Two patients were excluded because of intense ischaemic lesions caused by chemoembolization prevented histological analysis of the native liver (n = 1) and because of cryptic HBV infection (n = 1). Among the remaining 26 patients, four groups were individualized: (i) patients with chronic inflammatory liver disease with autoimmune features (n = 14, 54%); (ii) patients with features suggestive of non-alcoholic fatty liver disease (n = 3, 11.5%); (iii); patients with incomplete septal cirrhosis (ISC) and vascular liver disease (n = 3), and (iv) patients with unresolved CC (n = 6, 23%). In the autoimmune liver disease group, the median International Autoimmune Hepatitis score was 12.5 (range: 11-19) after reevaluation and review of the post-transplantation course was helpful to confirm the diagnosis with the occurrence of active graft hepatitis in nine patients, with autoantibodies in five patients. The vascular group was characterized by lesions of obliterative portal venopathy and ISC in all native livers. Diagnosis of NAFLD was based on the clinical background of obesity and/or type 2 diabetes and the presence of steatosis or steatohepatitis in native livers and graft biopsies. A definite aetiological diagnosis can be achieved in the majority of patients initially diagnosed with CC. Autoimmune liver disease emerged as the main aetiology (14 of 26 patients, 54%) and frequently recurred on the grafted liver (nine cases). In all cases a precise diagnosis is obviously of practical interest for better management of post-transplant survey and treatment.
authors.affiliation: Centre Hépato-Biliaire, Hôpital Paul-Brousse, Assistance Publique-Hôpitaux de Paris, Villejuif, France. jean-charles.duclos-vallee@pbr.ap-hopparis.fr
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Duclos-Vallée
authors.forename: Funda
authors.initials: F
authors.lastname: Yilmaz
authors.forename: Catherine
authors.initials: C
authors.lastname: Johanet
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: Roque-Afonso
authors.forename: Michelle
authors.initials: M
authors.lastname: Gigou
authors.forename: Catherine
authors.initials: C
authors.lastname: Trichet
authors.forename: Cyrille
authors.initials: C
authors.lastname: Féray
authors.forename: Eric
authors.initials: E
authors.lastname: Ballot
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Dussaix
authors.forename: Denis
authors.initials: D
authors.lastname: Castaing
authors.forename: Henri
authors.initials: H
authors.lastname: Bismuth
authors.forename: Didier
authors.initials: D
authors.lastname: Samuel
authors.forename: Catherine
authors.initials: C
authors.lastname: Guettier
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D003937:Diagnosis, Differential; D005234:Fatty Liver / Q000150:complications / Q000473:pathology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000473:pathology / Q000601:surgery; D006801:Humans; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies