title: Recurrent primary sclerosing cholangitis after orthotopic liver transplantation: is chronic rejection part of the disease process?
abstract: BACKGROUND
  The possibility of primary sclerosing cholangitis (PSC) recurrence after liver transplantation has been debated. The aim of this study is to examine whether recurrent PSC and chronic rejection (CR) are different expressions of the same disease process.
  
  
  METHODS
  One hundred consecutive patients receiving 118 grafts for the diagnosis of PSC were reviewed and placed into three groups: group A, recurrent disease, as evidenced by cholangiographic and pathologic findings with radiographic arterial flow to the liver (n=18; 15.7%); group B, those who developed CR (n=15; 13.0%); and group C, all others (n=82; 71.3%). Cholangiograms and histopathologic specimens were examined in a blinded fashion.
  
  
  RESULTS
  Demographic factors were similar, except for age, with a significantly younger age and more episodes of rejection in groups A and B (P<0.03). Group A had a higher incidence of cytomegalovirus hepatitis (P=0.008). Five-year graft survivals for A, B, and C were 64.6%, 33.3%, and 76.1%, respectively (P=0.0001), 5-year patient survivals were 76.2%, 66.7%, and 89.1%, respectively (P=0.0001), and repeat transplantation rates were 27.8%, 46.7%, and 8.5%, respectively (P=0.005). Radiographically, 90% of cholangiograms in patients with recurrent disease showed at least multiple intrahepatic strictures. Histopathologically, patients with recurrent disease and CR shared many features.
  
  
  CONCLUSIONS
  We have described a high incidence of recurrent PSC and CR in patients who received transplants for PSC. Histopathologic analysis suggests that CR and recurrent PSC could represent a spectrum of indistinguishable disease. However, the distinct difference in clinical outcome, as evidenced by an increased repeat transplantation rate and lower graft and patient survival in the CR group, clearly suggests that they are two distinct entities that require very different treatment strategies.
authors.affiliation: Department of Transplantation Surgery, Baylor University Medical Center, Dallas, Texas 75246, USA.
authors.forename: D R
authors.initials: DR
authors.lastname: Jeyarajah
authors.forename: G J
authors.initials: GJ
authors.lastname: Netto
authors.forename: S P
authors.initials: SP
authors.lastname: Lee
authors.forename: G
authors.initials: G
authors.lastname: Testa
authors.forename: O
authors.initials: O
authors.lastname: Abbasoglu
authors.forename: B S
authors.initials: BS
authors.lastname: Husberg
authors.forename: M F
authors.initials: MF
authors.lastname: Levy
authors.forename: R M
authors.initials: RM
authors.lastname: Goldstein
authors.forename: T A
authors.initials: TA
authors.lastname: Gonwa
authors.forename: G W
authors.initials: GW
authors.lastname: Tillery
authors.forename: J S
authors.initials: JS
authors.lastname: Crippin
authors.forename: G B
authors.initials: GB
authors.lastname: Klintmalm
mesh_terms: D000328:Adult; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D002908:Chronic Disease; D003953:Diagnosis-Related Groups; D004351:Drug Resistance; D005260:Female; D006084:Graft Rejection / Q000473:pathology; D006801:Humans; D016031:Liver Transplantation / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012086:Reoperation; D013256:Steroids / Q000494:pharmacology; D016896:Treatment Outcome
chemical_list: D013256:Steroids